Neuro Flashcards
UMN vs LMN lesion features
LMN shows PERIPHERAL NS symptoms:
muscle wasting, fasciculations, reduced tendon reflexes and tone,
forehead involved if CN VII involved in lesion,
signs are on the same side as the lesion, hypotonia,
FOCAL pattern of weakness (only specific muscles innervated by nerve)
UMN shows CENTRAL NS symptoms:
increased tone and reflexes, upgoing plantars (Babinski +ve),
if facial weakness then forehead is spared,
signs are on OPPOSITE side of lesion,
No muscle wasting,
Extensor planter response present
PYRAMIDAL pattern of weakness seen: extensor muscles affected but flexors are not etc. eg in STROKE
UMNs mainly form sensory and intermediate neurons with inhibitory effect on LMNs reflexes, therefore damage to an UMN or its connections with LMN will EXAGGERATE the reflexes
ACh, function and examples of malfunction
Noradrenaline function and examples of malfunction
ACh:
Enables muscle action, learning and memory
In Alzheimer’s, ACh producing neurons deteriorate
NA:
Helps control alertness and arousal
Undersupply is linked to depressed mood
Dopamine functions, examples of malfunctions
Influences movement, learning, attention and emotion
Excess dopamine receptor activity is linked to schizophrenia
Starved of dopamine, the brain produces the tremors and decreased mobility of Parkinson’s
Serotonin (5HT) function and examples of malfunction
Affects mood, hunger, sleep and arousal
Undersupply is linked to depression
GABA and glutamate function and examples of malfunction
GABA:
Major inhibitory neurotransmitter
Undersupply is linked to seizures, tremors and insomnia
Glutamate:
Major excitatory neurotransmitter involved in memory
Oversupply can overstimulate brain producing migraines or seizures (which is why some people avoid MSG monosodium glutamate in food)
Meningitis
Inflammation of the inner meninges (mostly pia and arachnoid) of the brain and spinal cord
Route of transmission: direct (aerosol, droplet secretion, direct contact with secretions, ear infections, nasopharynx, cranial injury), via the blood (septicemia) or viral meningitis (most common)
Bacterial meningitis is less common but high mortality especially high risk in infants
Cause varies with age, vaccination status (less than 4 years and unvaccinated) and other medical conditions
Meningococcus is most common cause of bacterial meningitis in UK (B, C subtypes)
In UK, syphilis, TB and fungal infections are rare causes
Klebsiella P account for most causes of hospital acquired meningitis (nosocomial infection)
Common causes:
Meningococcus (neisseria meningitidis)
Pneumococcus (streptococcus pneumonia)
Haemophilus influenzae
Types of meningitis
in addition to common classification according to microorganism cause
Aseptic meningitis - CSF has cells but is gram -ve and no bacteria can be cultured include viral and fungal causes
Non-infective meningitis (systemic disease) - inflammation due to infiltration of the meninges, such as malignant cells (leukemia, lymphoma) or rheumatological conditions (sarcoidosis or SLE)
Meningitis presentation
Very general in children: headache, fever, vomiting, malaise
Headache
Neck stiffness (nuchal rigidity)
Fever
Muscle ache/joint pain
Vomiting
Kernig’s sign: inability to fully extend knee when hip is flexed 90 degrees
Brudzinski’s sign: passive flexion of neck causes flexion of both legs and thighs
Papilloedema - swollen optic disc due to increased intracranial pressure
In meningococcal septicaemia - non-blanching petechiae and purpuric skin rash and signs of shock (this is a LATE sign due to septicaemia being present usually day 5 onwards)
Meningitis investigations
Diagnostic: lumbar puncture and laboratory CSF analysis
Bloods: FBC, CRP, blood cultures, blood glucose, ABG/VBG
KFT
Urine cultures
CXR (lung infections or abscesses)
CT (sometimes before LP) to exclude high intracranial pressure however NICE recommends clinical assessment NOT CT for this
Lumbar puncture complications and CIs
LP complications:
Coning (cerebral herniation)
Introduction of infection into CSF
CIs:
Local skin infection
Bleeding problems or anticoagulant therapy
Signs of spinal cord compression/pathology
Papilloedema or other signs of raised intracranial pressure
Suspicion of intracranial or cord mass
Congenital neurological lesions in lumbosacral region
Must have consultant present for LP with any CIs:
Raised ICP or reduced fluctuating loss of consciousness
Altered consciousness (GCS <9)
Focal neurological signs
Relative bradycardia and HTN
Abnormal posture
Unequal, dilated or poorly responsive pupil
Papilloedema
Shock
Coagulation abnormalities or therapy or platelet count <100x109/litre
Local superficial infection at LP site
Respiratory insufficiency in children
Normal CSF in adults
White and colourless
WBC: 0-5 cells/uL
No neutrophils, primarily lymphocytes
RBCs: 0-10/mm3
Protein: 0.15-0.45g/L (or <1% serum protein concentration)
Glucose: 2.8-4.2mmol/L (or >60% plasma glucose concentration)
Opening pressure: 10-20cm H2O
CSF overview in meningitis
Viral: clear, white cells high 5-1000, predominantly lymphocytes, normal RBCs <10, normal/high protein 0.4-1, normal glucose, normal/slightly high opening CSF pressure
Bacterial: cloudy and turbid look, very high white cells 100-50,000, neutrophils (PMN) predominately (some can cause leukocytes), high protein >1, decreased glucose <40%, high opening CSF pressure
TB meningitis: cloudy and viscous, increased white cells <500, mostly lymphocytes, very high protein 1-5, decreased BG <30%, high opening CSF pressure
FUNGAL MENINGITIS: clear/cloudy Elevated opening pressure Elevated WBC (10-500) Low glucose Elevated protein Gram stain positive Most likely causative organisms: cryptococcus neoformans and candida
Meningitis management
Urgent: all suspected meningitis cases are medical emergencies needing immediate hospitalisation
Suspected with rash - empirical antibiotics IM/IV benzylpenicillin (CI in severe allergy, give cefotaxime or ceftriaxone if allergy) blind therapy 1st line - targets meningococcal strain most common in UK
2nd choice antibiotics given when blood/CSF culture results are back (adult <50 - cefotaxime or ceftriaxone for 7-10 days, adult >50 cefotaxime or ceftriaxone + amoxicillin or ampicillin for 7-10 days)
Suspected without rash - urgent hospitalisation NO ANTIBIOTICS unless urgent transfer not possible
Suppurative treatment - analgesia, antipyretics, VI fluid (viral cause)
Identify signs of shock and sepsis an treat as needed
Dexamethasone (add on treatment before or with antibiotics within 12 hours)
Close contact prophylaxis - ciprofloxacin or rifampicin or IM ceftriaxone. People who have had prolonged close contact with case in household setting during 7 days before onset of illness (healthcare workers don’t need unless direct exposure of mouth/nose to infectious droplets with patient with <24 hours antibacterial treatment)
Notification of health protection unit (notifiable disease)
Meningitis complications
Much higher in meningococcal septicaemia (up to 57%)
Cerebral infarction in 1 in 4 people with bacterial meningitis
Neurological complications: hearing loss (higher in infants and children), seizures, cognitive impairment, motor deficits, visual impairments
Hydrocephalus
Learning difficulties
Encephalitis
Inflammation of the brain
Mostly caused by viral or post-infectious due to autoimmune disease
Causes:
Viral: HSV-1, CMV, EBV, VZV, HIV, measles, mumps, rabies, enterovirus
Bacterial: all causes of bacterial meningitis including TB, mycoplasma and listeria
Fungal and parasitic infections
Encephalitis presentation
Fever Headache Neck stiffness Vomiting Altered consciousness Convulsions Focal neurological signs Signs of raised intracranial pressure Psychiatric symptoms
Encephalitis investigations
CSF analysis for HSV-1 or 2, VZV and enteroviruses: usually shows moderate protein increase, raised lymphocytes, CSF/glucose ratio normal
FBC, blood cultures, renal function, U+Es, LFTs, glucose, ESR, CRP
CT scan for lesion/tumour, raised ICP (DOES NOT RULE OUT DIAGNOSIS)
MRI scan to detect demyelination and oedematous changes
Encephalitis management and complications
Urgent hospital admission
Parenteral antibiotics if suspected meningitis (IV/IM benzylpenicillin)
Immediate treatment with IV acyclovir (improves prognosis) +/- antibiotics to treat secondary bacterial infection
Careful IV fluid administration as this can worsen cerebral oedema
Complications: Mortality 70% in 7-10 days if untreated SIADH Disseminated Intravascular Coagulation Epilepsy Complications are usually linked to affected area of brain
Tuberculosis meningitis
Pathology:
Haematogenous spread eg miliary TB, foci of infection in brain and spinal cord
Foci can enlarge forming tuberculomas - foci rupture leads to meningitis
Active TB:
Containment by immune system by T cells and macrophages is inadequate
TB contagious
Either primary infection or reactivation of latent TB
Latent TB:
Granuloma formation prevents bacterial growth and spread - persistent immune system contaminant
Skin or blood testing (TBT, IGRAS) are positive - infection exists but sputum/CXR are normal - patient is asymptomatic and non-infectious
TB meningitis risk factors and presentation
Immunosuppression
HIV/AIDS
Alcoholism
Age <3 YO
Presentation: Headache Meningism Confusion FND Seizures Systemic symptoms: fever, night sweats, anorexia, weight loss and malaise
TB meningitis investigations
LP and CSF analysis: leukocytosis, raised protein, CSF plasma glucose <50%,
NAAT in CSF
AFB stain
PCR and culture
Serologic HIV test
CT/MRI may show hydrocephalus or Tuberculomas
general testing - tuberculin skin testing or IGRAS testing (more sensitive for BCG vaccination)
Management of TB meningitis
Rifampicin 2 months intensive 4 months continuation - p450 inducer: warfarin, calcineurin inhibitors, oestrogen etc. body secretion can coloured orange or red, altered liver function
Isoniazid 2mths intensive 4mths continued - risk of peripheral neuropathy and hepatitis, give with prophylactic pyridoxine,
Pyrazinamide 2 mths intensive - hepatotoxicity risk, measure eGFR before
Ethambutol 2mths intensive - may cause colour blindness and poor visual acuity must check visual acuity before starting and monitor monthly and eGFR
Shingles
VZV varicella zoster virus, form of herpes virus
In children: respiratory droplets containing virus enter lungs and incubate for 14-21 days. Invasion to respiratory mucosa, disseminates via mononuclear cells to skin. Virus containing vesicles causes CHICKEN POX, remains dormant in sensory nerve roots for years
In adults: reactivation of VZV but this time is limited to a dermatome of the skin = SHINGLES
Shingles is less contagious than varicella - household transmission rate is 15%
Blood test for antibodies can confirm is ever had chickenpox before
Those exposed to VZV if never had chickenpox before will get chicken pox no matter what age, but those who have HAD chickenpox will get shingles if reexposed
Presentation of shingles
Painful, burning, numbness in area in prodromal period
Few days later shows macular vesicular rash in SINGLE DERMATOME
Most common sites: lower thoracic dermatomes and ophthalmic division of trigeminal nerve V1 (ocular shingles)
Rash is still infectious until lesions dry and become crusted
Complications and management of Shingles
Post-herpetic neuralgia can be severe for years: most common complications, 10-20% cases of HZ (up to 30% in elderly). Pain lasting for >30 days from onset of lesions. Symptoms include constant or intermittent stabbing or burning pain, allodynia (pain induced by non-painful stimulus), hyperalgesia (severe pain from mildly painful stimulus) and intense itching. Amitriptyline is very effective at managing pain
Ramsay hunt syndrome (facial palsy with auditory involvement)
Immunosuppression can show skin involvement affecting multiple dermatomes
Management:
IV acyclovir for immunosuppressed, severe/disseminated disease (including ocular)
Pregnant women - oral acyclovir (if >20 weeks gestation) can use IV in severe or progressive maternal or neonatal infection as is not embryotoxic and can improve outcome significantly
Prevention: vaccination 9Zostavax) for those >50 YO to prevent recurrence of shingles or zoster immune globulin (VZIG) for pregnancy if VZ antibody negative or in neonates - does not prevent infection but attenuates the disease if given within 10 days
Cranial nerve VII
Facial nerve
Origin from Pons and medulla
Innervation anterior 2/3rds of tongue, facial muscles, scalp, ear and neck
Function to control facial muscles, motor limb of blink and corneal reflex, secretion of salivary and lacrimal glands, sensation of taste, anterior 2/3rd of tongue
LMN palsy and evaluation
Acute LMN palsy can present at any age but mostly seen at ages 15-60 years affecting both sexes equally
Rapid onset of unilateral facial paralysis
Clinical evaluation:
Motor function: observe facial symmetry, flattening of nasolabial fold, ask patient to wrinkle forehead, puff cheeks and smile. Show teeth, close eyes against resistance and whistle
Wrinkle forehead - frontalis
Close eye - orbicularis oculi
Purse lips - buccinator muscle
Show teeth - orbicularis oris
May have aching pain below ear or in mastoid area which may suggest middle ear or herpetic cause
May be hyperacusis (heightened sensitivity to perceived sounds)
Bells palsy
LMN lesion indicative of damage to motor nucleus or peripheral component
Patient can’t wrinkle forehead as final common pathway to muscles is destroyed
Lesion must be either in pons or outside brainstem
Sudden onset, ipsilateral weakness or paralysis on one side
Almost always unilateral and can be recurrent
Most common cause of acute LMN facial palsy
As a result of inflammation of facial nerve in canal due to viral infection (HSV1 or VZV) is the most common cause
Bells palsy features
Eyebrow droops, frowning or raising eyebrows is impossible
Eye cannot be closed; when asked to close eyes and show teeth, eyeball rotates upwards and outwards (Bell’s phenomenon)
Lower lid is everted. Tear production (lacrimation) is decreased
Mouth droop and patient dribbles
Bells palsy managament and prognosis
Reassurance - the majority of cases resolve spontaneously within 3 weeks
Prednisolone given to those over 16 YO presenting within 72 hours onset for 10 days
Antivirals NOT recommended in UK
Eye care (lubricating drops +/- ointment
Referral to ophthalmologist if further functions affected for Botulinum toxins or surgery if needed
Prognosis and complications:
70% fully recover
16% significant complications
5% develop severe facial asymmetry, gustatory lacrimation, inadequate lid closure, brow ptosis, drooling or hemifacial spasms
Focal neurological deficits
FND is a focal problem with the nerve, spinal cord or brain function
Affects a specific location/function such as the left side of the face, right arm or even small area such as the tongue. Speech, vision and hearing problems are also considered focal neurological deficits
Frontal lobe FND signs
Usually involve motor system and may include special deficit types depending on which part of the frontal lobe is affected
Unsteady walking
Muscular rigidity, resistance to limb movement (hypertonia)
Paralysis of limb (monoparesis) or larger area on one side of body (hemiparesis)
Paralysis of head and eye movements
Inability to express oneself linguistically, expressive aphasia (Broca’s aphasia)
Focal seizures which can spread adjacent areas (Jacksonian seizure)
Grand mal/tonic-clonic seizures
Changes in personality - disinhibition, inappropriate jocularity, rage, loss of initiative and concern, apathy etc
Frontal release signs: reappearance of primitive reflexes such as snout reflex, grasp reflex and palmomental reflex
Unilateral loss of smell (anosmia)
Parietal lobe FND signs
Usually involve somatic sensation and can include
Impaired tactile sensation
Impaired proprioception (postural and passive movement sensation)
Sensory and visual neglect syndromes - inability to pay attention to things in certain parts of sensory or spatial environment even as extreme as denial of a limb
Loss of ability to read, write or calculate (dyslexia, dysgraphia or dyscalculia)
Loss of ability to identify objects based on touch (astereognosis)
Temporal lobe FND signs
Usually involve auditory sensation and memory
Deafness without damage to structural (cortical deafness)
Tinnitus, auditory hallucinations
Loss of ability to comprehend music or language (sensory aphasia - Wernicke’s aphasia)
Amnesia, memory loss (long or short term)
Other memory disturbances (deja vu)
Complex multimodal hallucinations (these display complex forms and fill the entire visual field unlike occipital signs)
Complex partial seizures (temporal lobe epilepsy)
Occipital lobe FND signs
Usually involve visual sensation
Total vision loss (cortical blindness)
Loss of vision with denial (Anton’s syndrome)
Loss of vision on one side of visual field of both eyes (homonymous hemianopsia)
Visual agnosias ie inability to recognise familiar objects, colour or faces
Visual illusions such as micropsia (objects appear smaller) and macropsia
Visual hallucinations, displaying elementary forms such as zig zags and flashes in one half of visual field only for each eye
Cerebellar FND signs
Usually involve balance and coordination
Unsteady and clumsy motion of limbs or torso (ataxia)
Inability to coordinate fine motor activities (intention tremor) eg past pointing (pointing beyond the finger in finger nose test)
Inability to perform rapid alternating movements (dysdiadochokinesis) eg rapidly flip the hands
Involuntary left to right eye movements (nystagmus)
Brainstem and spinal cord FND signs
Brainstem signs:
Involve host of specific paralysis with contralateral loss of pain sensation
Spinal cord signs:
Generally involve unilateral paralysis with contralateral loss of pain sensation
Headaches history taking
Location Severity Character Associated symptoms Autonomic symptoms Relieving and exacerbating factors Number of episodes Duration of episodes Frequency of episodes Age of onset Triggers Analgesic use Family history Red flags
Red flags for headaches
Fever, photophobia or neck stiffness (meningitis or encephalitis)
New neurological symptoms (haemorrhage, malignancy or stroke)
Dizziness (stroke)
Visual disturbance (temporal arteritis, glaucoma, pituitary adenoma)
Sudden onset occipital headache (subarachnoid haemorrhage)
Worse on coughing or straining (raised intracranial pressure)
Postural, worse on standing, lying or bending (raised intracranial pressure)
Severe enough to wake from sleep
Vomiting (raised ICP or carbon monoxide poisoning)
History of trauma (intracranial haemorrhage)
Pregnancy (pre-eclampsia)
Primary and secondary causes of headaches
Primary: No structural or metabolic abnormalities: Tension headaches Migraines Cluster headaches Other primary headaches
Secondary:
Structural or metabolic abnormality:
Extracranial: sinusitis, otitis media, glaucoma, TMJ ds
Intracranial: SAH, vasculitis, dissection, CVT, tumour, abscess, meningitis
Metabolic disorders: CO2 retention, CO poisoning
Tension headaches
Very common and often suffer from both migraines and tension type heades (TTH)
Classically produce mild headache across forehead and in band-like pattern around the head with pressure behind the eyes and pericranial tenderness also often a symptom
Can last few mins to few days; often bilateral
Generally not activated by activity and not ‘severe’
Exact mechanism unknown
May be due to frontalis, temporalis and occipitalis muscles or neck muscles
Come on gradually and resolve gradually
Do NOT produce visual changes
Classification:
Infrequent: (<1 month)
Frequently (>10/month for 3 months)
Chronically (>15/month for 3 months)
Pathophysiology:
Trigeminal nerve supplying pericranial muscles, vessels and dura mater
Tension headaches are caused by abnormality in this nerve
Headaches associations and diagnosis
Associations: Stress Depression Alcohol Skipping meals Dehydration
Diagnosis: must have 2 of the following
No nausea or vomiting
Either photophobia or phonophobia NOT both
Not aggravated by movement
Management of headaches
Exclude serious causes of headache
Reassurance
Basic analgesia (rule out medication overuse)
Relaxation techniques and exercise, massage and ice packs
Hot towels to local area
Headaches occurring more than 2 days/week NICE recommends course of acupuncture first line or low dose amitriptyline 25-150mg ON 2nd line (4-6 months then withdraw slowly)
Secondary headaches
Similar presentation to tension headache but with clear cause
Produce a non-specific headache secondary to:
Underlying medical condition like infection, obstructive sleep apnoea, pre-eclampsia
Alcohol
Head injury
Carbon monoxide poisoning
analgesic headache
Caused by long term analgesia use
Gives similar non-specific features to tension headache
Secondary to continuous or excessive analgesia use
Withdrawal of analgesia important in treated the headache, although this can be challenging in patients with long term pain and those that believe the analgesia is needed to treat the headache
Hormonal headache
Related to oestrogen
Produce non-specific tension like headaches, tend to be related to low oestrogen
Often two days before and first three days of menstrual period
Around menopause
Pregnancy: worse in the first few weeks and improves in the last 6 months. Headaches in second half of pregnancy should prompt investigation for preeclampsia
Oral contraceptive pill can improve hormonal headaches
Cervical spondylosis
Common condition caused by degenerative changes in cervical spine
Causes neck pain usually made worse by movement but often presents with headache
Important to exclude other causes of neck pain such as inflammation, malignancy and infection as well as spinal cord or nerve root lesions
Cluster headaches
Rare type of headache caused by trigeminal autonomic cephalalgias (TACs): causes unilateral headache and autonomic features are present on same side as headache
Come as cluster of attacks and then disappear for a while (3-4 attacks a day for weeks/months followed by pain free period of 1-2 years)
Attacks can last between 15 minutes and 3 hours
Typically affects those aged 30-50
More common in smokers and adult males
Attacks can be triggered by alcohol, strong smells and exercise
Cluster headache presentation
Often described as most severe intolerable pain
Sometimes referred to as suicide headaches due to severity
Clusters last 1-2 months, remission for months or years
Lasting few mins to few hours and can occur several times a day
Typically unilateral located to one eye
Red, swollen and watering eye
Pupil constriction (miosis)
Eyelid drooping (ptosis) and oedema
Nasal discharge/rhinorrhoea
Facial sweating
Gets worse when lying down, often they pace the floor
Cluster headache management
Acute:
Check for red flags for secondary headache
Referral for neurology discussion
Triptans (sumatriptan 6mg subcut injection)
High flow oxygen for 15-20 minutes (can be given at home)
Prophylaxis: managed by neurology
Verapamil
Lithium
Prednisolone (short course 2-3 weeks to break cycle during clusters)
Can give injections into scalp of steroid and anaesthetic lasting few weeks to break cycle
Epilepsy
Umbrella term for seizure disorder when neurons are synchronously active
Recurring and unpredictable
Paroxysmal electrical discharges of neurons - temporarily impaired and send out sudden excitatory signals
Thought to be due to too much excitation or too little inhibition
Glutamate is main excitatory NT in brain and NMDA is main receptor
Some patients with epilepsy seem to have fast or longer acting activation of NMDA
Some patients have too little GABA inhibition due to dysfunctional GABA receptors leading to lack of inhibition
Can also be affected by brain tumours, brain injury or infection
Seizures and causes
Occur when there is abnormal electrical discharge in brain
Causes include: Sodium or glucose disturbances Infection (meningitis etc) Brain injury Electric shocks Brain tumours Drugs overdose Febrile seizures
Types of seizure
Generalised: both hemispheres affected Tonic-clonic Absent Myoclonic Tonic Infantile spasms/west syndrome Atonic
Partial/focal seizures: part of hemisphere affected, usually limited to part of cortex temporal lobe frontal lobe parietal lobe occipital lobe
Generalised seizures
both hemispheres of the brain affected
Patient will lose consciousness or awareness and may not remember the seizure
No warning of occurrence
Can begin as partial and spread to general.
Subcategories: tonic (stiff/fixed). Atonic (relaxed). Clonic (convulsions). Myoclonic (short muscles twitching only). Absence (lose and regain consciousness - appear ‘spaced out’)
Tonic-clonic is most common form (stiff then convulsions follow)
Tonic clonic seizures
(grand mal):
Initial tonic stiffening, may cry out then fall
Followed by clonic phase of synchronous jerking of limbs, should reduce in frequency over about 2 minutes
Eyes remain open
Patient may bite tongue
Urinary and/or faecal incontinence can occur
Patient may remain unconscious after awareness may return gradually
Postictal phase: drowsy and confused, irritable or depressed
Management:
First line sodium valproate
Second line lamotrigine or carbamazepine
Absence seizures
(petit mal):
Commonly start in childhood
Typically lasting only 10-20 seconds
Loss of awareness, show vacant expression, may see eyelids fluttering
Patient then returns to normal as though nothing happened
May happen several time a day and can be very short
Almost always a manifestation of generalised epilepsy
May start to experience other types of generalised seizures
Management:
Sodium valproate or ethosuximide
Myoclonic seizures
Abrupt involuntary jerks of the whole or part of the body
Can be very short
Typically occur in various forms of epilepsy but often in children as part of juvenile myoclonic epilepsy
Management:
First line sodium valproate
Lamotrigine, levetiracetam or topiramate
Tonic seizures and infantile spasms
Sudden increase in muscle tone
Patient becomes rigid and falls
infantile spasms:
West syndrome
Rare disorder starting in infancy at around 6 months old
Characterised by full body spasms
Poor prognosis - 1/3rd die by age 25 however 1/3rd are seizure free
First line management prednisolone or vigabatrin
Atonic seizure
‘drop attacks’:
Sudden loss of muscle tone
Causes patient to collapse and become very floppy
Don’t usually last more than 3 minutes
Typically begin in childhood
May be indicative of Lennox-Gastaut syndrome
Management:
Sodium valproate
lamotrigine
Partial of focal seizures
Only one hemisphere or lobe is affected, usually limited to part of the cortex in on hemisphere
Manifestation depends on part of the brain involved
Auras can accompany focal seizures
Subdivided into simple partial seizure where patient remains conscious, or complex partial with impaired consciousness
Important to rule out structural lesions
Usually rather brief
Simple partial: Remains conscious Small area of the brain affected Causes strange sensations for patient depending on lobe affected Doing strange things on autopilot Or jerking movements Usually aware of something happening to them Often remembers event Jacksonian march
Complex partial:
Impaired or loss of consciousness and responsiveness
Often do not remember the even
management:
- carbamazepine or lamotrigine
- sodium valproate or levetracetam
Temporal lobe seizures
Aura: epigastric sensation, olfactory or gustatory hallucinations, autonomic symptoms (change in BP, facial flushing), affective symptoms (fear, depersonalisation), deja vu
Seizure: motor arrest and absence, automatisms (lip smacking, chewing, fidgeting, walking), automatic speech, contralateral dystonia
Duration: slow evolution over 1-2 minutes
postictal: confusion, dysphasia
Frontal lobe seizures
Aura: typically abrupt onset with variable often indescribable aura, forced thinking or emotional manifests
Seizure: vocalisation, violent and bizarre automatisms, cycling legs, ictal posturing and tonic spasms, version of head and eyes contralateral to side of seizure focus, fencing posture (extending and abducting one arm with head rotation), sexual automatisms with pelvic thrusting, obscene gestures and genital manipulation
Duration: typically very brief around 30 seconds
Postictal: confusion brief with rapid recovery
Parietal lobe seizure
Somatosensory symptoms eg tingling, pain, prickling, vertigo, distortions or space
automatisms and secondary generalisation may occur
Occipital lobe seizures
Visual hallucinations - flashing lights, geometrical figures, sometimes complex hallucinations
eyelids might flutter, occasional eye turning or rapid blinking
automatisms and secondary generalisation might occur
Status epilepticus
If the seizure lasts >5 minutes
Is ongoing or without returning to normal
Usually caused by tonic clonic seizure but can occur from any type
Is a medical emergency - needs hospitalisation
Often treated with benzodiazepines to enhance GABA firing
Common after effects of seizures and investigations
After effects:
Postictal confusion
Paralysis (unilateral) of arms or legs Todd’s paralysis can last 15 mins subsides within 2 days. Thought to be caused by severe but temporary arrest from area of brain affected by seizure
Investigations:
MRI/CT brain
EEG electroencephalogram to detect electrical signals in brain
History to determine types; are they frequent, short, do they recall having them, third party info to describe features (absent, convulsions etc), any auras or symptoms before or after (postictal state), tongue biting and postictal confusion, urinary incontinence and pallor are NOT helpful as can occur with syncope. Any possible previous seizures
Full physical and neurological exam
Full mental state exam
Bloods: glucose, U+Es, Ca, eGFR, LFTs, urine biochemistry
Urgent neuro referral
Management of seizures
Specialist input within 2 weeks
EEGs to support diagnosis and MRI to identify structural abnormalities
Advice to cease driving, swimming or potentially dangerous work or leisure activities
Mainly anticonvulsants based on patients age and needs; usually not recommended until after second seizure, implemented once specialist diagnosis confirmed depending on risk of subsequent seizures occurring. Choice of drug based on type of seizure
Surgery to remove what is thought to be causing eg tumours of specific area of brain
Nerve stimulation
Ketogenic diet
Medication for seizures overview
Sodium valproate:
Increases GABA activity which relaxes the brain
Side effects: teratogenic (must be careful with contraception), liver damage and hepatitis, hair loss, tremor
Carbamazepine:
First line for focal seizure
Side effects: agranulocytosis, aplastic anaemia, induces the P450 system so has many drug interactions
Phenytoin:
Side effects: folate and vitamin D deficiency, megaloblastic anaemia (folate deficiency), osteomalacia (vit D deficiency)
Ethosuximide:
Side effects: night terrors and rashes
Lamotrigine:
Side effects: SJS or DRESS syndromes and leukopenia
Emergency management of seizures
Protect patient from injury, refrain from restraining, put cushioning under their head and clear area of dangerous objects
Don’t put anything in their mouth and TIME the seizures
When seizures stops: check airway, place in recovery position and check CV and neuro states
Prolonged seizures: (status epilepticus)
ABCDE approach:
Secure the airway
Give high-concentration oxygen
Assess cardiac and respiratory function
Check blood glucose levels
Gain intravenous access (insert a cannula)
IV lorazepam 4mg, repeated after 10 minutes if the seizure continues
If seizures persist: IV phenobarbital or phenytoin
Medical options in the community:
Buccal midazolam
Rectal diazepam
Pseudoseizures overview
Psychogenic nonepileptic seizures (PNES) occur as result of psychological cause such as severe mental stress rather than from neuronal dysfunction
Often result of other mental health conditions - treating underlying cause is vital
Aware of what’s happening and remember event
Response to touch unlike with actual seizure
Often have eyes closed unlike tonic clonic seizure so may also react to having eyes forced open
Show involuntary stiffening, convulsions and jerking, loss of attention, consciousness, confusion, absence or lack of awareness of surroundings
Conditions that may cause include: Generalised anxiety disorder Panic attacks OCD ADHD Substance abuse, traumatic injuries, emotional disturbance PTSD
In general, some treatments that may be effective for treating PNES include: (no prevention)
individual or family counseling
cognitive behavioral therapy (CBT)
relaxation techniques
behavioral therapy
eye movement desensitization and reprocessing (EMDR) therapy, for traumatic memories
antidepressants
medications for underlying psychological issues
general management principals
Headache diary: record frequency, duration and severity and monitor how well interventions are working
Give written and oral advice on medical disorder and information about support organisations
Warn of risks of medication overuse
Migraines
Complex neurological condition that causes headaches and other associated symptoms
Occurs in attacks often following a particular pattern
Most common cause of episodic headaches; affects up to 20% women and 10% men
Recognised cause of disability in those under age 50
May be a combination of structural, functional, chemical, vascular and inflammatory factors (thought the hypothalamus is involved especially in premonitory phase)
Types of migraines
Migraine without aura: characterised by headache with specific features and associated symptoms
Migraine with aura: primarily characterised by transient focal neurological symptoms before or during headache Silent migraine (with aura but without headache)
Hemiplegic migraine: aura includes motor weakness which can last for weeks, often have relatives with migraines with motor symptoms, gene mutations have been identified
Migraines with brainstem aura: dysarthria, vertigo, tinnitus, hearing impairment, diplopia, ataxia and decreased level of consciousness
Retinal migraines: repeated attacks of monocular visual disturbance - flashes of light (scintillations), scotomata, blindness
Chronic migraines: headaches occur on more than 14 days a month for more than 3 months. Migrainous headaches on at least 8 days a month. Check for medication overuse!!
Migraine without aura criteria
5+ headaches with typical characteristics of:
Unilateral pain
Pulsating
Moderate to severe intensity
Made worse by physical activity such as walking or climbing stairs
Associated with nausea or vomiting
Associated with photophobia and phonophobia
Lasting 4-72 hours
Normal examination and no other cause found
Migraines with aura
Around 25% migraine sufferers
Neurological symptoms immediately before or during headache
Some or all migraine attacks
Usually last for less than an hour (motor symptoms often lasting longer)
Usually precede headache
Neurological symptoms are fully reversible and may take several forms:
Visual - most common, shimmering, zigzags, fragmentation of image, patches of visual loss (scotomas, hemianopia and tunnel vision)
Sensory - tingling or pins and needles over part of the body, face or tongue. Numbness may follow or occur alone
Speech - dysphasia
Loss of motor function
Migraine triggers
Stress Hormones Alcohol Sleep change Bright lights Loud sounds Changes in weather Physical exertion
migraine presentation
Lasting between 4-72 hours Moderate to severe intensity Pounding or throbbing in nature Usually unilateral but can be bilateral Discomfort with lights - photophobia Discomfort with loud noises - phonophobia With or without aura Nausea and vomiting Severity varies between attacks and patients Frequently feel ‘washed out’ Hypersensitivity to touch
Hemiplegic migraine, presentation and triggers
Can mimic strokes
Essential to act fast and exclude stroke in patients presenting with these symptoms before considering hemiplegic migraine
Presentation: Typical migraine symptoms Sudden or gradual onset Hemiplegia (unilateral weakness of limbs) Ataxia Changes in consciousness
Triggers: often not possible to identify triggers Stress Bright lights Strong smells Certain foods Dehydration Menstruation Abnormal sleep patterns Trauma
5 stages of migraines and pathology
not typical in everyone and vary between patients, can experience only one or two stages. Prodromal stage can involve several days of subtle symptoms such as yawning, fatigue, food cravings, sore muscles or mood changes prior to onset of the migraine
Premonitory or prodromal stage - can begin 3 days before headache. Thought to be alternating body homeostasis (hypothalamic involvement).
Aura lasting up to 60 mins. Only 1/3rd patients experience this. Fully reversible neurological symptoms. Most common is visual: spotted or blurred vision, sensory aura, language aura or motor aura (weakness). Thought to be due to cortical spreading depression - neuronal depolarisation lasting longer than usual spreading across the cortex
Headache stage lasting 4-2 hours. Most commonly unilateral, pulsatile nature and may be accompanied by associated symptoms. Can experience changes in sensitivity to light, smell or touch (cutaneous allodynia).
Trigeminal vascular system thought to be activated by premonitory/aura phases causing trigeminal nerve to increase sensitivity in nociceptors in dura mater, referred pain then causing pain around the eye, occipital area etc due to conversion of nerves near the roots
Resolution stage - headache can fade away or be relieved completely by vomiting or sleeping
Post dromal or recovery phase. Lingering symptoms can be triggered by sneezing, moving etc. due to sensitisation in nociceptors in tissues which goes away in few days
acute management of migraines
Often go to dark room and sleep
Paracetamol 1g, high dose aspirin (900mg)
Triptans (sumatriptan 50mg as migraine starts; 5HT receptor agonists act on smooth muscle causing vasoconstriction, peripheral pain receptors inhibiting pain, reduce neuronal activity in CNS) try at least 3 migraines before moving on to alternative due to variability of effectiveness between patients must be taken at the start of headache phase (not useful in aura phase) CI in CVD, PVD, CVA or TIA. subcutaneous or nasal administration also available can repeat 50mg dose after 2 hours PRN but ONLY 2 doses <24 hours if migraines include vomiting then can give subcut injection (self administer)
NSAIDs - ibuprofen 400-600g or naproxen 250-500g
Antiemetics if vomiting occurs (metoclopramide 10mg, prochlorperazine 3-6mg buccal tablets)
Migraine prophylaxis
Avoiding known triggers; headache diary can help this
Physical activity as long as not a known migraine trigger and stop COCs in women whose migraines started or are worsened when taking them (increased cerebral thrombosis risk)
Propranolol 40mg BD-80mg up to 160mg - beta blocker first line
Anticonvulsants: Topiramate 25mg ON for 1 week then increase in 25mg steps to max 200mg - teratogenic and can cause cleft lip/palate so patients should not get pregnant
Amitriptyline 10mg increases 10mg weekly up to 60mg. Decreases neurotransmission. Side effects: can’t see, can’t pee, can’t spit, can’t shit
Sodium valproate initial 200mg BD up to 800mg
Botox for chronic migraines but inconsistent effectiveness
Acupuncture
Vitamin B12 supplements (riboflavin) may reduce frequency and severity
If migraine is specifically triggered around menstruation then prophylaxis with NSAIDs (mefenamic acid) or triptans (frovatriptan or zolmitriptan) can be used around that time as preventative measure
Can go into remission and tend to get better over time
Complications of migraines
Status migrainosus - debilitating attack lasting for than 72 hours
Persistent aura lasting over a week without evidence of infarction on imaging
Infarctions and seizures occurring during aura
GCA
Systemic immune related vasculitis affecting medium and large sized arteries, particularly carotid and its extracranial branches
Unknown pathogenesis but involves chronic inflammatory process, predominantly of large arteries
Highest incidences in northern EU countries
Most present 60-80 years
Cause is poorly understood: potentially autoimmune as often occurs in people with polymyalgia rheumatica
No well established triggers or risk factors
GCA presentation
Temporal headache and scalp tenderness Myalgia Malaise or fever Acute severe sight impairment (20% patients) Jaw claudication Diplopia Abnormal palpation of temporal artery - bruits may be heard over the carotid, axillary or brachial artery Elevated ESR Anaemia (normochromic normocytic anaemia) Features of polymyalgia rheumatica
GCA investigations and complications
Clinical
Temporal artery biopsy
Bloods: elevated ESR, CRP, LFTs (alkaline phosphatase can be elevated)
Colour duplex USS
complications
Loss of vision; permanent, partial or total
Aneurysms, dissections and stenotic lesions of aorta and major branches
CNS diseases: seizures, cerebral vascular accidents etc
Steroid related complications: osteoporosis, bruising, insomnia, hypomania, depression, HTN, diabetes, elevated cholesterol and fluid retention
GCA management
Fast Track referral to rheumatology (<3 days) for no ocular symptoms
Same day referral to ophthalmology if visual symptoms
40mg prednisolone daily
60mg prednisolone if claudication symptoms (jaw, tongue claudication or visual symptoms)
Once symptoms resolve can begin to reduce dose in 10mg steps biweekly, then in 2.5mg steps
Flares treated with increased corticosteroids to level at which symptoms were previously controlled
Low dose aspirin 75mg daily unless CI to decrease rate of visual loss and strokes
With high dose aspirin start PPI to decrease risk
Osteoporosis prophylaxis for long term steroid therapies
Subarachnoid haemorrhage, risk factors and association
Bleeding into the subarachnoid space where the CSF is located
Usually the result of a ruptured cerebral aneurysm
Very high mortality and morbidity
Accounts for 5% strokes
mean age of patient is 50 YO
Risk factors: HTN Smoking Excessive alcohol consumption Cocaine use Family history Black family origin Females Age 45-70
Associated with:
Sickle cell anaemia
Connective tissues disorders (Marfan syndrome, Ehlers-Danlos)
Neurofibromatosis
Autosomal dominant polycystic kidney disease
Subarachnoid haemorrhage presentation
Typical history is sudden onset occipital headache, occurs during strenuous activity. Known as the thunderclap headache due to suddenness and severity
Neck stiffness
Photophobia
Vision changes
Neurological symptoms such as speech changes, weakness, seizures and loss of consciousness
Subarachnoid haemorrhage investigations
CT head first line - blood causes hyperattenuation in the subarachnoid space
Lumbar puncture CSF analysis : RBC raised and xanthochromia (yellow colour caused by bilirubin)
Angiography (CT/MRI) once haemorrhage confirmed to locate source of bleed
Subarachnoid haemorrhage management
Referral to specialist neurosurgical unit
Reduced consciousness may need intubation and ventilation
Supportive care and MDT approach: nursing, nutrition, physio, occupational therapy
Surgical intervention to repair vessel and prevent rebleeding, can be done by coiling (inserting catheter into arterial system, placing platinum coils into aneurysm and sealing it off from artery) or clipping (cranial surgery clipping aneurysm to seal it)
Nimodipine is CCB used to prevent vasospasm. Vasospasm is a common complication that can result in brain ischaemia following haemorrhage
Lumbar puncture or inserting shunt to treat hydrocephalus
Antiepileptic medications to help seizures
Sinusitis
Causes headache with inflammation in ethmoidal, maxillary, frontal or sphenoid sinuses (PARANASAL SINUSES)
Acute: infection lasting 7-30 days. Tends to arise from viral infection with secondary bacterial infection. Commonly strep pneumoniae, H. influenzae
Subacute: infection lasting 4-12 weeks
Recurring: >3 significant acute episodes in a year lasting >10 days with no intervening symptoms
Chronic: symptoms persisting >90 days with or without acute exacerbations (can be caused by irreversible changes in mucosal lining of sinuses). Is multifactorial including infectious, inflammatory or structural
Sinusitis risk factors
Allergies Asthma Smoking Hormonal status (pregnancy etc) Nasal dryness Foreign body Irritant inhalation (cocaine) Dental problems - trauma or infection Swimming, divind, high altitude sports Mechanical obstruction (nasal polyps) Immunocompromised
Sinusitis presentations
This usually produces facial pain behind the nose, forehead and eyes
Often tenderness over affected sinus helping establish diagnosis
Usually resolves within 2-3 weeks
Most sinusitis is nasal. Nasal irrigation with saline can be helpful
Prolonged symptoms can be treated with steroid nasal spray, antibiotics sometimes needed
Acute cases present with non-resolving cold >1 week or worsening symptoms over 4-5 days. Initial viral infection rhinitis appears to settle followed by further malaise relating to the sinusitis
Headache
Cough
Dental pain
Pressure or fullness feeling in ears
Sinusitis investigations
History
Clinical diagnosis - palpate frontal (press up from beneath medial supraorbital ridge), maxillary (press against anterior wall below inferior orbital margin) and ethmoidal sinuses (press medially against medial wall of orbit) for tenderness
percussion and transillumination of sinuses
trigeminal neuralgia
Chronic debilitating condition resulting in intense and extreme episodes of facial pain
Episodes are sporadic and sudden often like electric shocks lasting several seconds to minutes
It is a result of neuropathic disorder of the 5th cranial nerve (trigeminal)
Trigeminal nerve supplies sensation, nociception, thermoception and motor supply to muscles of mastication
Most commonly the maxillary and/or mandibular branch are involved too
Uncommon in those younger than 40
May be genetic predisposition but exact transmission method is unclear
Triggers: Vibration Skin contact eg shaving or washing Brushing teeth Oral intake Exposure to wind
Trigeminal neuralgia presentation
Unilateral usually on the cheek or face but can involve the eyes, lips, nose and scalp
Sudden onset, brief duration
Stabbing, severe shock like pains
Recurrent pain occurring in paroxysms lasting from few seconds to minutes
May have preceding symptoms such as tingling or numbness
Can have triggers
Episodes are intermittent but can last days, weeks, months on end then not return for months or years etc.
Atypical presentations have restless underlying pain like a migraine associated with superimposed stabbing pains and maybe intense burning sensation. This is more difficult to treat
Red flags trigeminal neuralgia
Sensory changes, deafness or other ear problems
Difficulty achieving pain control, poor response to carbamazepine
History of skin lesions or oral lesions that could lead to perineural spread
Ophthalmic division only or bilateral suggestive of malignant lesions or MS
Age onset under 40 years
Optic neuritis
Family history of MS
trigeminal neuralgia investigations and complications and prognosis
Clinical diagnosis
MRI for uncertainty
Complications:
Poor quality of life due to severe pain
Poor mental and physical capacity
Prognosis:
50% experience remissions of at least 6 months duration
65% newly diagnosed will have second episode within 5 years
Periods of remission tend to get shorter with time and attacks of pain tend to get longer
Trigeminal neuralgia management
No definitive cure at present, but newer surgical procedures are promising
Offer carbamazepine first line
If ineffective or severe pain limiting daily activities or atypical clinical features consider expert advice or early referral to specialist pain service
Reassure patient it is not life threatening, education on condition and management options, avoiding triggers, support groups etc
Gabapentin combined with ropivacaine injections into trigger sites can improve pain control
Botox injection to reduced frequency and pain intensity
Surgery - relieving pressure on nerve (microvascular decompression) or damaging it to prevent pain transmission (Rhizotomy)
Chronic pain commonly impacts and risk factors:
Difficulty sleeping most commonly Lack of energy Drowsiness Concentration difficulties Depression Anxiety Poor appetite Can lead to mental health problems - anxiety, depression etc
Risk factors:
Older age
Female
Lower income and social deprivation
Geographical and cultural background: South asian more common
History of abuse or interpersonal violence
Employment and occupation: heavy lifting etc.
Classifications of pain
Acute (<3 months)
Chronic (>3 months, or beyond expected healing time)
Underlying mechanism - nociceptive (ability to detect painful stimulus, protects against injury) vs neuropathic (arising from nerve damage, serves no protective purpose)
Physical origin - visceral (organs) vs somatic (external body- soft tissues, bones, muscles) referred pain
Underlying cause - cancers, inflammatory (often worse at night or early day), post-operative (predictable likely acute nociceptive), mechanical pain (related to movements)
Acute and chronic pain pathology
Acute: Usually resolves in less than 3 months Burns Broken bones Sprained joints Acute pain is commonly nociceptive but can be neuropathic (eg herpes zoster virus)
Chronic:
Acute pain can sometimes persist becoming chronic; this is because repeated exposure to painful stimuli can cause alterations in the nervous system making them more sensitive to painful stimuli. For example, inflammation associated with pain can cause peripheral sensitisation which increases pain intensity and the area the pain is felt. Repeated painful stimuli causes a ‘wind up’ leading to more intense pain felt and more pain signals transmitted leading to central sensitisation leading to people feeling pain when they should not eg those with severe cases can feel pain with touch
Chronic mechanical pain is nociceptive pain
Nociceptive vs neuropathic pain
Nociceptive pain:
Typically sharp pain or dull ache
Degree of pain usually proportional to cause
Can easily describe pain usually
Likely to respond to simple analgesic (NSAID, paracetamol, opioids)
Neuropathic pain:
Often difficult to describe
Burning, shooting, crawling, electric shocks, stabbing pains
May appear out of proportion to cause
Likely to best respond to antineuropathic medications (tricyclic antidepressants like amitriptyline, gabapentinoids like pregabalin or gabapentin)
Pain pathways
Several pathways transmit pain signals
Most importantly the spinothalamic tract which transmits touch and temperature
Other pathways: spinoreticular tract and spinomesencephalic tract
Pain signals pass from periphery to spinal cord then to midbrain, then travels out to several other areas (ascending pathway)
Brain influences pain perception via descending pathway blocking ascending transmission. Descending pathway NTs include endorphins (natural opioids), NA and serotonin. Effectiveness of descending pathway varies with situation and brain state eg when in state of threat/danger may not feel pain until conflict is resolved, but person with low mood may feel pain more than normally expected from injury
Spinothalamic:
Consists of medial and lateral tracts
Lateral carries sensory discriminative part of signal and enables sufferer to localise pain
Medical tract terminates deeper in brain causing general arousal and aversion
Spinoreticular tracts:
Terminated in reticular formation of medulla and pons contributes to general arousal associated with pain
Spinomesencephalic:
Synapses in area of midbrain called periaqueductal grey
Stimulation causes release of endogenous opioids inhibiting pain signals in spinal cord causing natural analgesia
Pain red flags
Possible fractures
Major trauma
Minor trauma in elderly or degenerative bone diseases
Age <20 or >50
History of cancer
Weight loss
Recent bacterial infection
Previous IV drug use
Immunosuppression
Severe worsening pain, especially at night or lying supine
Severe or progressive sensory alteration or weakness
Bladder or bowel dysfunction
Evidence of neurological deficit on physical exam
Visual disturbance
Unexplained seizures
Pain yellow flags
flags indicating increased likelihood of developing chronic pain:
Anxiety
Beliefs that pain is always harmful and activity can cause more damage
Catastrophizing
Depression
Expectation that only passive treatments will help and reluctance to be involved in active participation
Fear avoidance behaviour
Severe pain intensity
Adopting ‘illness’ type behaviours
Mood disruption, avoidance of social contact
Requesting treatment that does not fit best practice or is against clinical judgement
Ongoing litigation
Social and financial problems
History of extended sick leave or other absence
Problems at work or poor job satisfaction
Overprotective family or lack of support
Signs of neuropathic pain
Allodynia, by gently stroking the affected area with a brush, cotton wool or a feather, which causes an exaggerated pain response from the patient
Hyperalgesia, by a normal painful stimulus using a pin, which causes a greatly increased pain response from the patient
Quantifying pain
various assessment scales and questionnaires, such as: Visual analogue scale (VAS) Numerical rating scale (NRS) Verbal rating scale (VRS) Visual signs of pain Physical signs
Pain assessment tools are divided into the following three types:
Unidimensional scales (mainly used for acute pain)
Multidimensional scales
Diagnostic questionnaires The Leeds assessment of neuropathic symptoms and signs (LANSS)
The Douleur Neuropathique 4 (DN4)
PainDETECT
conditions to be aware of if taking NSAIDs
Asthma (may worsen)
Renal impairment increased risk of dehydration
Liver impairment cautioned in liver disease
Upper GI bleeding or ulceration
HF or previous heart attack - increased risk of this and stroke
Pregnant
Opioid overview
acts on multiple opioid receptors in the body (distributed throughout nervous system (CNS and PNS). OP3 thought to mediate most analgesic properties. Activate receptor and signalling pathways reducing transmission of nociceptive impulses
Codeine is an opioid PROdrug meaning needs to be converted to active form in liver, as is tramadol, dihydrocodeine and oxycodone
drug interactions: alcohol enhance drowsiness, SSRIs increase risk of serotonin syndrome
IV morphine should only be given in a place where oxygen and resuscitation facilities are available. Patient observations including the respiratory rate should be monitored until at least 15 minutes after the last dose.
Morphine requires an effective renal system to clear its metabolites. Patients suffering from renal failure may require a reduction in the dose of the drug.
Naloxone can be used for opioid overdose antidote
PCA
Patient-controlled analgesia (PCA) enables a patient to control a pump, on demand, to administer small bolus doses of analgesic drugs. The most common drug used is morphine, but other analgesic agents, such as fentanyl, oxycodone and ketamine, are also used.
Safety features of a PCA pump include:
Limited size of bolus
Lockout interval
Limited total dose administered
Most hospitals have a PCA protocol. A typical adult PCA protocol administers 1 mg of morphine with a 5 minute lockout.
In children, and patients who are not able to use PCA, the responsibility is given to the nurse looking after the patient. This is called nurse‑controlled analgesia (NCA). It is important that only the nurse administers a dose. The pump usually has a longer lockout period to prevent serious adverse side-effects.
If the patient is still requiring the PCA after a few days and is able to eat and drink, it may be appropriate to change the PCA to oral medication.
Opioid common side effects
constricted pupils
drowsiness, confusion and hallucinations
respiratory depression
reduced HR and low BP, palpitations and oedema
anaemia and low platelets
nausea, vomiting and constipation
bone mineral density decreased, joint pain and weakness
urinary retention
skin itching
Opioid drugs doses and equivalent dose to oral morphine
Codeine - 60mg - 3-9mg equivalent
tramadol - 100mg - 10mg equivalent
morphine IV/IM 10mg - 20-40mg equivalent
fentanyl - 100micrograms - 10mg
Neuropathic pain management
Gabapentin - effective and also enhances analgesic effect of morphine if given alongside.
Pregabalin - second line produces similar effects. both stimulates production and release of GABA
side effects: dizziness, headache, tremor, nausea and vomiting, diplopia, nystagmus, ataxia, oedema and drowsiness
Antidepressants - amitripylline (tricyclic) at LOW doses is effective analgesic and evidence to suggest it reduced chronic herpes zoster pain
BEST TAKEN 60-90 minutes before bed (less hangover effect) and increase in slow increments if experiencing bad side effects
Duloxetine is an SNRI been shown to reduce pain of diabetic neuropathy
opioids can be helpful (tramadol and oxycodone)
Linodcane patches and capsaicin creams useful for post herpetic neuralgia
Simple analgesics along with non-pharmacological therapies - TENS
Non-pharmacological pain relief methods
Neuromodulation is considered for certain kinds of pain:
- acupuncture: effective for peripheral joint osteoarthritis and can be appropriate to try 6 week course in most types of pain
- TENS has no evidence of benefit despite many women claiming it helps in during labour
- spinal cord stimulators SCSs: shown to help failed back surgery syndrome and complex regional pain syndrome, also recommended for chronic neuropathic pain
other methods:
- CBT for effective pain management techniques in chronic pain
- structured exercise program eg physiotherapy recommended for lower back pain
- radiation therapy very effective at palliating symptoms for bony metastases along with surgical neuroablative techniques
Common pain relief drugs method of action
Ibuprofen - inhibits cycloxygenase
Morphine - acts on cell membrane opioid receptors
ketamine - blocks NMDA receptors
Gabapentin - stimulates GABA production
How to discuss medications with patients BRAN
benefits of treatment,
Risks,
Alternatives,
Nothing - what might happen if the patient does nothing
Subarachnoid haemorrhage complications and prognosis
Complications:
Rebleeding (most common cause of death; 20% in first few days)
Vasospasm causing cerebral ischaemia - most common cause of permanent CNS deficit (peaks at days 4-12)
Hydrocephalus (first 24 hours) due to blockage of arachnoid granulations needing ventricular or lumbar drainage
SIADH causing hyponatremia
Prognosis:
Not good; 50% die suddenly soon after bleeding
10-20% die in early weeks in hospital from rebleeding
May have long term FND
Subarachnoid haemorrhage pathology
Rupture (70% Berry aneurysms) mainly branching points of circle of willis
10% congenital arteriovenous malformations
20% no lesions found
Main cerebral arteries in circle of willis common for berry aneurysms are:
anterior communicating artery
origin of posterior communicating artery
trifurcation of middle cerebral artery
termination of internal carotid artery
basilar artery
Subarachnoid haemorrhage complications and prognosis
Complications:
Rebleeding (most common cause of death; 20% in first few days)
Vasospasm causing cerebral ischaemia - most common cause of permanent CNS deficit (peaks at days 4-12)
Hydrocephalus (first 24 hours) due to blockage of arachnoid granulations needing ventricular or lumbar drainage
SIADH causing hyponatremia
Prognosis:
Not good; 50% die suddenly soon after bleeding
10-20% die in early weeks in hospital from rebleeding
May have long term FND
Subarachnoid haemorrhage pathology
Rupture (70% Berry aneurysms) mainly branching points of circle of willis
10% congenital arteriovenous malformations
20% no lesions found
Main cerebral arteries in circle of willis common for berry aneurysms are:
anterior communicating artery
origin of posterior communicating artery
trifurcation of middle cerebral artery
termination of internal carotid artery
basilar artery
Raised ICP
Syndrome, often a complication rather than stand alone presentation
Can be acute (trauma) or chronic (eg SOL: brain tumour, abscess, haematoma)
Pathophysiology:
Skull is incompressible and volume inside is fixed
80% volume brain; 10% blood and 10% CSF
Therefore if one component increases then the other must decrease to compensate eg hematoma increases blood so CSF may be diverted down into spinal column
If these changes are too much for compensatory mechanisms then this leads to increased ICP
Cerebral perfusion pressure = MABP - ICP
Main mechanisms:
Oedema caused by stroke, CVA, trauma, hyponatremia
SOL caused by brain tumours, brain abscess, ICH
CSF or venous system blockage caused by hydrocephalus, CVT/IVT etc
causes of increased ICP
CVA
Brain tumours
Trauma (Intracerebral haemorrhage, traumatic cerebral oedema)
Infection (abscesses)
Cerebral oedema (eg post hypoxia, electrolyte imbalances - hyponatremia)
Idiopathic
Presentation of increased ICP
Headache: worse in the morning, coughing or bending over
Vomiting
Tired
Visual problems
Bilateral dilated pupils
Seizures
Decreased GCS
Irregular/altered RR
Arms extended down hands twisting away from body: ominous sign
Focal neurological deficit (unilateral ptosis, 3rd or 6th CN palsy, anisocoria etc)
Cushing’s reflex/triad - decreased HR, increased BP and papilloedema opposite of shock
Complications and investigations of increase ICP
Complications:
Herniation of brain through foramen magnum (CONING - brain hernia)
Investigations:
Urgent CT to assess cause and severity
Management of increased ICP
Early involvement of neurosurgeon or neurologist
Elevate head of bed to 30 degrees
Correct hypotension with fluid resus (3% hypertonic saline to increase blood osmolality encouraging water movement into blood away from intracranial compartments)
Hyperventilation inducing to lower ICP (induces hypocapnic vasoconstriction; causes PaCO2 decrease, leading to arterial vasoconstriction thus lowering cerebral blood flow, blood volume and ICP)
Mannitol (might induce hypovolaemia; monitor serum osmolarity) or dexamethasone to reduce ICP (with tumours must discuss with senior before giving these)
Avoid pyrexia, manage seizures (increases ICP)
CSF drainage with intraventricular catheter and monitor ICP
Analgesia and sedation - usually IV propofol, etomidate r midazolam with morphine/alfentanil
Neuromuscular blockade to avoid further ICP increase from intrathoracic pressure increases
Cushing reflex
Cerebral perfusion pressure = MABP - ICP
During increase ICP both sympathetic and parasympathetic NS are activated
When ICP is greater than MABP the arterioles in brain’s cerebrum become compressed causing cerebral ischaemia
First sympathetic NS causes constriction of arteries inducing HTN to restore blood flow to ischemic brain tissue
Then the baroreceptor reflex in aortic arch notice increased BP and trigger PNS response via vagus nerve to induce bradycardia
Bradycardia may also be due to direct mechanical distortion of vagus nerve and subsequent PNS response
Raised ICP also increases pressure on the brainstem (respiratory centre) causing slow irregular respiration and/or apnea
Coning
Downward cerebellar herniation
Cerebellar tonsils move down through the foramen magnum, compressing lower brainstem and upper cervical spine
Causes dysfunction of respiratory and cardiac centres
Leads to death
Cerebral herniation
Deadly consequence of increased ICP
Divided into supratentorial and infratentorial
Supratentorial types:
Cingulate (subfalsing) herniation when pressure pushes cingulate gyrus under fault cerebri
Central - brain pushed down toward cerebelli
Uncal - medial temporal lobe moves toward tentorium often compresses midbrain
Transcalvarial only occurs with skull defect and brain pushed through defect
Infratentorial when cerebellum pushes up or down (transtentorial = pushed upwards, tonsillar (coning) = pushed downwards)
Papilloedema pathology
Optic nerve (CN I) surrounded by meninges and CSF Subarachnoid space has increased CSF pressure due to raised ICP Compresses blood supply to eye and retina causing papilloedema
Idiopathic intracranial HTN (IIH)/pseudotumor cerebri and presentation
Excess CSF compressing the brain and optic nerves and increased ICP
Most common in obese females in 3rd decade of life
F:M ratio = 8:1
Associated with obesity, endocrine abnormalities (Cushing’s, hypoparathyroidism) systemic conditions like SLE, CKD and drugs like tetracycline, steroids, oral contraceptives etc
Presentation: Mass signs (headache, increased ICP and papilloedema) Gradual visual field defects: blurred vision, narrowed visual fields, increased blind spots +/= diplopia due to CN VI nerve palsy NO conscious or cognition changes
Idiopathic intracranial HTN (IIH)/pseudotumor cerebri investigations and management and prognosis
Investigation:
To rule out differentials: MRI/CT scans
Neurological examinations
Management:
Weight loss
Treat underlying conditions
Acute headache and papilloedema: prednisolone
Mild chronic disease: acetazolamide, topiramate, loop diuretics to lower ICP
Serial LPs to reduce ICP PRN
If fails and visual loss worsens - optic nerve sheath fenestration (decompression) or lumbar peritoneal shunt (Divert CSF into abdomen)
Prognosis:
Often self limiting condition but 10% have permanent significant visual loss
Acetazolamide appears to be most effective agent for lowering ICP
Intracranial haemorrhage and major risk factors
Types of primary ICH: ICH Subarachnoid haemorrhage Subdural hematoma Extradural haemorrhage
Pathology:
Small vessel disease: AVM, microaneurysms eventually leading to rupture and bleed
Bleeding within brain tissue and/or ventricles is ICH
Major risk factors: HTN Alcohol Increasing age Anticoagulants Cocaine, methamphetamine
Intracranial haemorrhage presentation and diagnosis
Presentation: Severe headache Sudden LOC Haemorrhagic stroke (FND) One sided weakness Vomiting Seizures Neck stiffness Fever
Diagnosis:
CT/MRI of brain
Management of intracranial haemorrhage
Admit to ICU start treatment within 3 hours onset of symptoms improves outcome
Controlling high BP
Anticoagulants should be stopped for 7-10 days, case by case decision to restart
Prothrombin complex concentrate should be started
Analgesia for headaches
Surgery - immediate referral to neuro for:
Large ICH: deepening coma or brainstem compression
Cerebellar bleed: obstruction of drainage pathways of CSF causes hydrocephalus
Epidural hematoma
Collection of blood between dura mater and skull.
Caused usually by damage to meningeal arteries associated with head trauma of temporal or parietal bone
Epidural hematoma presentation
Symptoms are lucid intervals pattern of LOC after head injury, concussion, drowsiness, recovery (lucid interval) then rapid deterioration of GCS with FND, increasingly severe headaches, vomiting, confusion, seizures and hemiparesis, brisk reflexes (UMN) and upgoing plantar (Babinski +ve)
Prolonged bleeding causes ipsilateral pupil dilation (mydriasis), coma deepens, bilateral limb weakness, deep and irregular breathing (brainstem compression), respiratory arrest, increased BP and bradycardia are late signs
Epidural hematoma investigations and diagnosis
Ix:
On head CT shows biconvex lens shape, will not cross suture lines due to dura tight attachment to bone, XR or LP for CSF analysis.
Prompt diagnosis and neurosurgical intervention often needed to avoid death
Subdural hematoma
Accumulates between brain and dura mater
Typically from bridging veins
Risk factors include: head trauma and patients on anticoagulation
Most caused by trauma but trauma often forgotten as it was so minor or long ago (up to 9 months) - especially in elderly who veins are already dilated
Subdural hematoma presentation
fluctuating level of consciousness, physical or intellectual slowing, sleepiness, headache, personality changes and unsteadiness, increased ICP, seizures, FNDs
Often associated with trauma
Presents as gradually worsening headache, confusion and increased ICP
Often seen in elderly
Subdural hematoma investigations and management
Ix
Crescent shaped and may cross suture lines on CT
Mx
Often seen in elderly and can often be managed without surgical intervention
Managed by correcting clotting abnormalities urgently, craniotomy or Burr hole to evacuate blood and address cause of trauma (falls/abuse etc)
epidural hematoma summary
bleed between skull and dura mater, source often arterial (middle meningeal) caused often by head trauma/skull fractures presentation: altered state of consciousness headache vomiting confusion/seizure Aphagia
subdural hematoma summary
bleed below dura mater and above arachnoid membrane
source of blood often venous (bridging veins)
caused often by trauma (accidents on motorbikes etc)
presentation:
coma
lucid intervals - progressively worsens neurological decline
present similar to epidural
Subarachnoid haemorrhage summary
in arachnoid space, source often cerebral artery (usually aneurysm) not always traumatic cause presents as altered conscious levels starts suddenly and severe headache thunderclap headache meningisms
Intracerebral haemorrhage summary
bleeding in Lobar, thalamic, pontine cerebellar haemorrhage second most common cause of stroke caused by HTN, embolism, brain tumour, bleeding disorders and drug use presents: neurological signs area dependent headache nausea and vomiting decreased loss of consciousness
Intraventricular haemorrhage
bleeding confined to ventricular system of brain
caused often secondary to ICH or SAH extending to ventricles
presents with similar symptoms to ICH and SAH
Management and prognosis of brain hematomas
Stabilise with intubation, reducing high ICP
Transfer urgently to neurosurgical unit for clot evaluation +/- ligation of bleeding vessel
Prognosis is excellent if diagnosed and operated on early
Poor if coma, pupil abnormalities or decerebrate rigidity are present pre-op
Stroke and causes
Acute neurological deficit lasting more than 24 hours caused by cerebrovascular aetiology
Subdivided into ischaemic stroke (caused by vascular occlusion or stenosis) and haemorrhagic (caused by vascular rupture, resulting in intra-parenchymal and/or subarachnoid haemorrhage)
Ischaemic stroke accounts for 87%
Haemorrhagic stroke for 10%
Subarachnoid haemorrhage for 3%
Small vessel occlusion/cerebral microangiopathy or throbum in situ
Cardiac emboli; AF, endocarditis, MI
Atherothromboembolism eg from carotids
CNS bleed (ICH): high BP, trauma, aneurysm rupture, anticoagulation, thrombolysis
Hypovolemia
Carotid artery dissection
Vasculitis
Pathophysiology of stroke and TIA
Severity of neurological impairment after arterial occlusion depends in degree and duration of obstruction, area and function of tissue, and ability of collateral circulation to provide perfusion to the area
Normal cerebral blood flow is tightly autoregulated to maintain flow of >50mL/100g/minutes across wide range of perfusion pressures
If this falls between 20-50 the brain can compensate by increasing oxygen extraction, but below this threshold, neuronal quiescence occurs with neurological deficits
Below 15 neuronal death occurs therefore with complete loss of blood flow necrosis happens rapidly
With partial blood flow neuronal function is impaired but cell death delayed from minutes to hours
Restoration of blood flow can arrest this progression
Ischaemic stroke
When blood supply in cerebral vascular territory is critically reduced due to occlusion or critical stenosis of cerebral artery
Minority are caused by cerebral sinus or cortical vein thrombosis
Risk factors strongly associated with increasing age, family history, HTN, smoking, diabetes, AF, comorbid cardiac conditions, carotid artery stenosis, sickle cell disease and dyslipidemia
Haemorrhage stroke
Intracerebral haemorrhage caused by rupture of vessel with bleeding into brain parenchyma
¾ are caused by intracerebral haemorrhage while the rest are caused by subarachnoid haemorrhage
Strong risk factors include family history, increasing age, haemophilia, cerebral amyloid angiopathy, HTN, anticoagulation, use of elicit sympathomimetic drugs, vascular malformations, moyamoya syndrome
Cerebral aneurysm
Typically asymptomatic until ruptured resulting in subarachnoid haemorrhage
Screening with non-invasive neuroangiography is recommended for at risk populations
Strong risk factors include smoking, moderate-high level alcohol consumption, family history, previous subarachnoid haemorrhage and heritable connective tissue disease
risk factors for stroke and TIA
AF Valvular disease Carotid stenosis CHF HTN Diabetes Smoking Alcohol abuse Increasing age COC
Stroke presentation
(apoplexy) is the sudden onset of weakness, numbness, paralysis, slurred speech, aphasia, problems with vision. ischemic area involved determines the type of focal deficit that is seen in the patient.
stroke - symptoms lasting longer than than 24 hours
Sudden onset neurological deficits
Confusion, altered conscious level
Severe sudden headache which may be associated with neck stiffness
Sentinel headache(s) may occur in preceding weeks
Weakness: sudden loss of strength in face or limbs
Sensory loss: paraesthesia or numbness
Speech problems: dysarthria
Visual problems: loss or diplopia
Dizziness or ataxia
Nausea and vomiting
Specific cranial nerve deficits or Horner’s syndrome (miosis, ptosis and facial anhidrosis)
Difficulty with fine motor coordination and gait
Neck or facial pain
Carotid bruit, AF, previous TIA or IHD may
Posterior circulation strokes should be suspected if acute, persistent, continuous vertigo/dizziness with nystagmus, nausea and vomiting, head motion intolerance and new gait unsteadiness (symptoms of acute vestibular syndrome)
In thalamic stroke: cognition and consciousness are intact
TIA presentation
TIA - symptoms lasting <24 hours; usually resolve within the hour Unilateral weakness or sensory loss Dysphagia Ataxia, vertigo or incoordination Syncope Sudden transient loss of vision in one eye (amaurosis fugax) Homonymous hemianopia Cranial nerve defects
brain location Infarct and presentation
cerebral - Contralateral sensory loss or hemiplegia
Initially flaccid then spastic (UMN lesion)
Dysphagia: homonymous hemianopia; visuo-spatial deficits
brainstem -
Quadriplegia, disturbances of gaze and vision
Locked in syndrome (aware but unable to respond)
lacunar (small penetrating arteries -
Basal ganglia, internal capsule, thalamus and pons
Five lacunar stroke syndrome (LACS): ataxic hemiparesis, pure motor, pure sensory, sensorimotor and dysarthria/clumsy hand
Stroke/TIA assessment
ABCDE assessment of individual: vitals, screen for CVD or neurological problems (FAST test, fundoscopy)
Bloods: FBC, chemistry profile, blood glucose, prothrombin time, partial thrombin time (PTT)
ECG to exclude arrhythmias
Ask individual and witnesses about clinical features of event including neurological deficits and associated symptoms like headaches, vomiting or decreased conscious level
Time of onset, activity and progression (if they awoke with symptoms it is classed as when they were last awake without symptoms)
Assess for risk factors for CVD (smoking, diabetes, hyperlipidaemia, alcohol and drug abuse, pregnancy, recent trauma, illness of surgery)
MRI of brain better to identify ischaemia or CT (not as good)
Management of stroke
Hospitalisation/specialist stroke unit
ABCDE: avoid hypoxia, aspiration, keep BG and BP normal, keep NBM but hydration
AFTER exclusion of haemorrhage cause: 300mg aspirin (given for at least 2 weeks then 75mg daily); alteplase thrombolysis (within 4.5 hr onset)
thromectomy for large artery occlusion
follow ups:
seen by stroke specialist 72 hrs
assess continence of bladder and bowel
monitor nutrition
cognitive impairment assessments, mini mental state examinations
assess mood and wellbeing (depression screening etc)
mouth care especially for those tube fed or swallowing problems
sexual dysfunciton
spasticity and contractors
end of life care: palliative teams
TIA
Transient episode of neurological dysfunction caused by focal brain, spinal cord or retinal ischaemia without acute infarction; majority will resolve within first hour
More common in women and black and mexican-american people
Should be suspected when presenting with typical symptoms that rapidly resolve but also less classic symptoms such as unilateral vision loss, transient aphasia or vertigo
Have considerable risk of early recurrent cerebral ischaemic events
Strong risk factors include AF, valvular disease, CHF, HTN, diabetes, carotid stenosis, smoking, alcohol abuse and increasing age
Evaluation and initiation of secondary prevention should occur rapidly
Aetiology:
In situ thrombosis of intracranial artery or artery-artery embolism of thrombus as result of stenosis or unstable athlerosclerotic plaque (16%)
Cardioembolic events (29%): thrombus in heart may form secondary to AF or impaired ejection fraction
Small vessel occlusion (16%): micro atheromas, fibrinoid necrosis and lipohyalinosis of small penetrating vessels seen (if in brainstem and internal capsule will produce significant symptoms). HTN and diabetes predispose to small ischaemic lesions
Occlusion from hypercoagulability, dissection, vasculitis, vasospasms or sickle cell occlusive disease (3%)
Unknown cause (36%)
stroke/TIA investigations and differentials
Bloods: FBC, glucose, PTT, lipid profile
ECG
brain MRI with diffusion
Differential diagnosis: Hypoglycemia Seizure with post seizure (Todd’s paralysis) Complex migraine Intracranial haemorrhage, abscess or mass Conversion disorder MS Peripheral neuropathy Global hypoperfusion/syncope
Stroke/TIA severity scores
national institutes of health stroke scale: performance scored on each, totalled and severity rating given:
Level of consciousness
Best gaze
Visual fields tested
Facial palsy
Motor arm (each scored separately)
Motor leg (“”)
Limb ataxia - presence in more than one limb
Sensory loss pinprick
Assess aphasia using picture description
Dysarthria: ask to repeat words on defined list
Tests of inattention or extinction in 2 sensory modalities, vision and light touch
ABCD2 SCORE: used to help predict risk of stroke following TIA:
Age >60 - 1 point
BO >140/90 - 1 point
Clinical deficit of unilateral weakness - 2 points or clinical deficit of speech impairment - 1 point
Duration >60 minutes - 2 points or duration 10-59 minutes - 1 point
Diabetes - 1 point
Risk categories:
Low 0-3 points - 2 day stroke risk 1%
Intermediate 4-5 points - 2 day stroke risk 4.1%
High 6-7 points - 2 day stroke risk 8.1%
TIA management
loading dose aspirin (PPI cover) and arrange stroke specialist assessment within 24 hours (unless bleeding disorder/anticoagulation)
urgent admission for anyone suspected to have had one or more TIA, has suspected cardioembolic source or severe carotid stenosis
if TIA occurred >1 week ago:
refer to specialist within 7 days
assess for AF and arrhythmias
give information to individual and family (stroke association website) and safety net call 99 if symptoms return
6 month follow up, review annually health and social care needs
smoking cessation, lifestyle changes (5 portions fruit and veg a day; two portions of oily fish per week; reduce saturated fats; keep salt intake low and reduce processed foods; alcohol within 14 units per week spread over at least 3 days)
aspirin, clopidogrel etc. 75 mg daily
adjusted dose warfarin
statin 20-80mg daily (aim for non-HDL >40% reduction) but AVOID STATINS in primary intracerebral haemorrhage
antihypertensives
monitor BP
screen and treat other underlying conditions
notify DVLA and assess fitness to drive, discuss need to return to work with rehabilitation team
Primary and secondary prevention for TIA
Primary prevention: Lifestyle and modifiable risk factors BP <130/80 if QRISK >10% BP 140/90 if QRISK <10% Low dose aspirin if risk
Intracranial venous thrombosis
Thromboembolism of cerebral veins/venous sinuses
F:M ratio 3:1 most common in young women
Very rare: 0.5% of strokes
Pathology:
Cerebral infarction can result from tissue congestion and obstruction. Cerebral haemorrhage may occur with the venous thrombosis and may even be a presenting feature. Symptoms are related to the area of thrombosis. Examples include:
Lateral sinus thrombosis, which can cause headache (similar picture to pseudotumor cerebri).
Involvement of the jugular bulb, which may cause jugular foramen syndrome.
Cavernous sinus thrombosis, which may cause compressive symptoms with cranial nerve palsies.
Thrombosis of veins/venous sinuses causes increased venule and capillary pressure and decreased CSF absorption
Reduced capillary perfusion and Increased ICP
Leads to cytotoxic oedema and disruption of blood brain barrier
Parenchymal haemorrhage (60%) or cerebral herniation
Intracranial venous thrombosis risk factors
Genetic thrombophilias Vasculitis Pregnancy and childbirth Hormonal contraceptives Infection (meningitis, HIV, TB, otitis media, mastoiditis, oral infections) Nephrotic syndrome Cancers Head injury
Presentation of IVT and diagnosis
Highly variable; often diagnosed late with initial diagnosis of isolated intracranial HTN syndrome
Can be acute <48 hours, subacute 48 hours - 30 days or chronic >1 month
60-90% show progressive headache usually gradually increasing; unlike SAH, worsening with valsalva or lying down
Seizures, FND (most commonly paresis)
Encephalitis: altered mental state, movement disorders and coma
Papilloedema, decreased visual acuity, proptosis, chemosis, ophthalmoplegia
Associated with sepsis
Diagnosis:
MRI venogram gold standard (CT can initially be normal)
Management of IVT
Anticoagulation: LMWH (dalteparin/enoxaparin) immediately followed by 3-12 months warfarin (INR 2.5 aim)
Analgesia
Anti-epileptics
Potentially surgical decompression
Some patients left with persistent neuro deficits
Syncope
Temporary loss of consciousness usually related to insufficient blood flow to the brain from hypotension or insufficient CO
Followed by complete recovery
Primary syncope events are benign; known as vasovagal events
Very common. In the UK 1 in 2 people experience syncope at some point in their life
Most common cause being vasovagal syncope
Vasovagal syncope
the three Ps:
Suggested by absence of features to suggest an alternative diagnosis and the presence of features suggestive of uncomplicated faint
Posture: occurring after long period of standing
Provoking factors: pain or medical procedure/fear
Prodromal symptoms: sweating/feeling warm or hot beforehand
Other cause: a vasovagal is only classed in the ABSENCE of the following causes
Orthostatic hypotension
Cardiac abnormalities
Epilepsy
Syncope investigations and history taking
Investigations: Focussed history taking ECG BP Cardiac examination
History taking: When did it happen Was it singular or multiple/has it happened before What was the environment like Any chance of pregnancy? Any known heart problems Any episodes of SOB Any chest pain Episodes of tongue biting? Was there any incontinence? Was anyone there to witness it? Can they describe what happened etc. Did you hit your head? How did you fall?
Management of syncope
Offer information for vasovagal syncope and advice on condition, possible trigger events and strategies for avoiding them; reassure them prognosis is good
Orthostatic hypotension: offer information on condition and lifestyle changes; review and see if symptoms improve; specialist referral for initiation of drug therapy if measures do not improve
Suspected cardiac cause: referred urgently for cardiac assessment; review within 24 hours by specialist
Suspected epilepsy: referred to specialist for neurological assessment within 2 weeks
People awaiting referrals should be advised NOT to drive
Multiple sclerosis
Acquired immune mediated inflammatory condition of the CNS resulting in areas of demyelination, gliosis and secondary neuronal damage throughout the CNS
Typically first develops in young adults
Most common non-traumatic cause of significant neurological disability in people younger than 40 years
Cause is unknown, considered autoimmune where the myelination of axons in the brain and spinal cord are destroyed
Pathophysiology:
Relapse involves demyelination causing conduction difficulties resulting in presentations of neurological deficits
During remissions myelin attempts repair and ends with fibrosis and scarring
More relapses will result in slower recoveries due to fibrosis and tissue damage previously leading to pregressive deterioration and eventually leading to secondary progressive MS without remission as CNS is too damaged to recover
Patterns of MS disease
Relapsing remitting (RRMS): most common pattern. Episodes of symptoms (relapses) are followed by recovery (remissions) and periods of stability. Typically after several relapses residual damage to parts of the CNS remains resulting in only parietal recovery during remissions
Secondary progressive (SPMS): occurs when there is a gradual accumulation of disability unrelated to relapses, which become less frequent or stop completely. About ⅔ of people with RRMS progress to SPMS
Primary progressive (PPMS): steady gradual worsening of the disease from the onset without remissions. This occurs in about 10-15% of people with MS
MS presentation
Vary greatly
Natural history of RRMS is unpredictable; severity and frequency of relapses varies and time taken to progress to secondary progressive MS or significant permanent disability is greatly varied
Optic neuritis: eye pain and pain with moving the eye; vision loss/loss of colour vision/loss of peripheral vision
Transverse myelitis: weakness in arms and legs; tingling/numbness; bladder dysfunction or bowel motility issues
Cerebellar related symptoms: loss or balance or coordination
Brainstem syndromes: difficulty speaking; breathing; parietal or complete hearing loss; paralysis
MS investigations and complications
Clinical history and presentation
MRI
Bloods specific to MS plaques in later disease
LP - CSF analysis
complications
MS lesions can develop almost anywhere in CNS so can include fatigue, spasticity, ataxia, tremor, mobility problems, visual problems, pain, bladder problems, sexual problems, mental health problems
Various drug.non-drug therapies used to help manage these complications
Management of MS
Aims to treat with disease modifying therapies; may reduce number and severity of relapses and delay disability progression (immunotherapies; immunoglobulins etc)
Patient information on condition; patient and family support as life is often very unpredictable (can be up and about then suddenly need 6 months rehabilitation to walk again)
Suspect MS: refer to consultant neurologist (only they can make diagnosis)
Suspected relapse: rule out infections (UTI and RTI primarily); contact persons MS team promptly to discuss appropriate management. Often includes oral methylprednisolone 0.5g daily for 5 days which may shorten and decrease severity of relapse
Comprehensive review at least annually (normally in secondary care)
Require MDT: occupational therapies for home; physiotherapists; social care (assessment of needs and financial assistance); psychological support; local and national charity organisations
Guillain-barre syndrome
Disorder causing demyelination and axonal degeneration resulting in acute, ascending and progressive neuropathy
Characterised by weakness, paresthesia and hyporeflexia
Around 75% patients have history of preceding infection, usually respiratory or GI and large number of organisms have been linked (campylobacter jejuni, epstein barr virus, cytomegalovirus, mycoplasma and HIV) most common infection associated is gastroenteritis related viral infections
This association with infection suggests antibodies to infectious organisms also attack antigens in peripheral nerve tissue (autoimmune)
Higher incidence in men
Peak ages 15-35 years and 50-75 years
If patient comes in with rare neuro symptoms following recent infection; it is Guillain barre until proven otherwise
Presentation of Guillain-barre syndrome
60% cases onset occurs around 3 weeks after viral illness
Usually shows ascending pattern of progressive symmetrical weakness, starting in lower extremities
Can take days/weeks to progress upwards
Facial weakness, dysphagia or motor speech disorders can develop
Severe cases may lead to respiratory failure
May show neuropathic pain particularly in legs or back pain
Reflexes are reduced or absent
Sensory symptoms include paraesthesia and sensory loss starting in lower extremities
Autonomic symptoms include reduced sweating, reduced heat tolerance, paralytic ileus and urinary hesitancy
Signs: Hypotonia Altered sensation or numbness reduced/absent reflexes Fasciculation (muscle twitch) Facial weakness usually symmetrical but can be asymmetrical Autonomic dysfunction - fluctuations in HR, arrhythmia, labile BP ad variable temperature Respiratory muscle paralysis
Guillain-barre syndrome investigations and management
Electrolytes, serum and urine osmolarity: usually inappropriate ADH secretion
LP: elevated CSF protein may be up to 1-2 weeks after weakness onset
Antibody screen for NS
Spirometry: FVC to determine admission to ICU and intubation
Nerve conduction studies
ECG: foten 2nd or 3rd degree blocks and rhythm disturbances
Management: Plasma exchange IV immunoglobulin Corticosteroids DVT prophylaxis Admission to ICU and intubation Pain relief for neuropathic pain
Guillain-barre syndrome complications and prognosis
Persistent paralysis Respiratory failure Hypotension or HTN Thromboembolism Pneumonia Skin breakdown Cardiac arrhythmias Urinary retention Psychiatric issues - depression, anxiety
Prognosis:
Outcome mostly is excellent but neurological problems persist in up to 20% patients, half of these are severely disabled
Poor prognosis is associated with rapid symptom progression, increasing age, prolonged ventilation
Huntington’s disease
Progressive neurodegenerative disorder with distinct phenotype including chorea (involuntary movement disorder) and dystonia (involuntary muscle contractions), incoordination, cognitive decline and behavioural difficulties
Estimated between 1 in 10,000-20,000
Most common genetic cause of chorea
Some cases symptoms start before age 20 with behavioural disturbances and learning difficulties (juvenile huntington’s disease) more common when inherited from the father
Huntington’s presentation and progression
Typical onset is middle age but can manifest at any age
Often prodromal phase of mild psychotic and behavioural symptoms
Early signs: personality change, self-neglect apathy with clumsiness, fidgeting with fleeting facial grimaces
Behavioural problems (may lead to conflicts, relationship breakdowns, job loss etc)
Depressed mood
Leads to progressive chorea, rigidity and dementia
Frequently associated with seizures
Chorea is initially mild but may be severe and cause uncontrollable limb movements
As disease progresses chorea is gradually replaced by dystonia and Parkinsonian features
Dysarthria, dysphagia and abnormal eye movement are common; can also show tics and myoclonus
Can also develop cardiomyopathies and skeletal muscle wasting
Progression:
Associated with increasing depression, bradykinesia, cognitive impairment and aggression
Behavioural difficulties include apathy, lack of initiative, dysphoria, irritability, agitation or anxiety, poor self care, poor judgement and inflexibility
Late features include spasticity, clonus, supranuclear gaze palsy and extensor plantar responses
Rate of cognitive decline is very variable
HD investigations
MRI and CT (mod-severe shows loss of striatal volume and increased size of frontal horns and lateral ventricles)
Genetic testing and extensive genetic counselling
Testing for alternative causes of movement disorders (SLE, APS, thyroid disease, Wilson’s disease)
Predictive testing:
Genetic testing: two blood samples taken to double check; section of Huntington gene contains cytosine, adenine and guanine repeated numerous time in faulty gene; it can tell if the fault is present, but NOT when it may present/develop
Four types of results recognised:
Under 27 repetitions is NORMAL
Between 27-35 is normal but small chance repeats may increase in future generations
Between 36-39 is abnormal but chance person is affected later on in life or not at all
Over 40 is unequivocally abnormal
Management of HD and prognosis
Currently no cure or slowly of disease progression
Hyperkinesia and psychiatric symptoms respond well to pharmacotherapy
Neuropsychological deficits and dementia remain untreatable
Patient information; patient and family support; support groups
MDT of neurology; GP; psychiatry; speech and language therapy; physiotherapy; genetic clinics; social work; palliative care etc.
Chorea: benzodiazepines, valproic acid, dopamine depleting agents and neuroleptics can help. Often results are not consistent
Predominant bradykinesia and rigidity - levodopa or dopamine agonists may help
Refer depressional symptoms promptly (SSRIs 1st line)
Antipsychotic medications for psychotic symptoms
Neural and stem cell transplantation
Deep brain stimulation for palliative treatment of choreatic movements, dystonia and stiffness
Progression:
Currently progressive disorder
Death usually from intercurrent illness like pneumonia
Suicide second most common cause of death
Cerebral palsy
Umbrella term for group of permanent movement and posture disorders that limit activity
Underlying cause is acquired pathology within developing brain during the prenatal, neonatal or early infant period
Impaired movement results from centrally mediated abnormal muscle tone leading to spasticity
Can also incorporate disorders of sensation, perception, cognition, communication and behaviour. Effects can include secondary MSK problems, neurogenic bladder and/or bowel, GORD, excessive saliva production and feeding/swallowing difficulties
Epilepsy also associated
cause Congenital brain malformation Congenital infection Other factors disrupting blood supply Postnatal meningitis MRI identifies white matter damage (more often in spastic CP), basal ganglia or deep grey matter damage (associated with dyskinetic CP)
CP risk factors
Multiple gestation Chorioamnionitis Maternal respiratory or gastro-urinary infection treated in hospital Maternal thyroid disease, iodine deficiency, TORCH (toxoplasmosis, rubella, cytomegalovirus, herpes simplex) Maternal thrombotic disorders leading to neonatal emboli from placental thrombosis Teratogen exposure (warfarin) Fetal genetic and metabolic disorders Fetal brain malformations Placental abruption Preterm birth and low birth weight Respiratory distress Birth asphyxia Neonatal sepsis
Complications of CP
Feeding difficulties Drooling Aspiration, recurrent chest infections Vomiting, regurgitation, GORD Osteopenia, osteoporosis Constipation Incontinence Visual impairment Hearing impairment Epilepsy Learning disability Communication difficulties Behavioural difficulties Mental health problems Pain Sleep disturbance Impaired social interaction and participation Reduced QoL and life expectancy
CP presentation
Varies from mild to severe
Mild spasticity and contracture in one side of the body, interferes with fluid movement and fine manual dexterity
Sensory inattention
Focal epilepsy
More severe forms: all four limbs may be affected with mixed spasticity and dyskinesia
Can be substantial contractures and scoliosis may need wheelchair
Associated learning difficulties and cortical visual impairment
Life expectancy may be reduced
CP investigations and management
Clinical history and presentation; delayed milestones etc.
Enhanced clinical assessment and developmental follow up programme by MDT
Management:
Refer any child with suspected CP symptoms to specialist paediatric neurology
Refer to child development service for MDT assessment in order to facilitate early diagnosis and intervention
Refer urgently to child development service all children with risk factors exhibiting abnormal motor features
Refer to child development service for further assessment all children with delayed motor milestones and persistent toe walking
Manage any other presenting symptoms (GORD, infections etc.) while awaiting referrals as needed
Prognosis:
Underlying brain lesion is non-progressive but functional and neurological manifestations of condition evolve over time
Brain tumours
Most in adults are supratentorial and high grade gliomas and meningiomas
Can develop at any age but most common in those between 50-70 YO
Can’t truly be differentiated into benign or malignant; instead high grade (rapidly growing and aggressive) and low grade (slow growth may or may not be successfully treated) are used instead
‘Benign’ brian tumors can still account for significant morbidity and mortality as continue to grow and cause adverse effects from space occupying
Primary tumors account for 2% all tumours in UK
Lifetime risk of developing is 1 in 77
Metastases from other cancers in the brain are 10 times more common than primary tumours
Brain tumour risk factors
Ionising radiation
Vinyl chloride associated with high grade gliomas
Immunosuppression related to cerebral lymphomas
Inherited syndromes like neurofibromatosis, von Hippel-lindau, tuberous sclerosis
Mobile phone use: needs investigation
Possible increased risk with oil refining, embalming, textiles also need further investigation
Brain tumour histological types
High grade: gliomas and glioblastoma multiforme, primary cerebral lymphomas, medulloblastomas
Low grade: meningiomas, acoustic neuromas, neurofibromas, pituitary tumours, pineal tumours, craniopharyngiomas
Secondaries: common malignancies spread to brain include lung, breast, stomach, prostate, thyroid, colorectal, melanoma and kidney cancers
Brain gliomas
Occurs in brain and spinal cord
Bein in glial cells
Cancer in astrocytes is called astrocytoma/glioblastoma (most common in all ages; can be low or high grade; low grade common in children, high grade common in adults). Common symptoms are headache and seizures. Low grade treated with surgery and radiotherapy; high grade with surgery, radio and chemotherapy
Grade 4 astrocytomas also called glioblastoma multiforme; around 80% of astrocytomas are grade 4.
Cancer in oligodendrocytes is called oligodendrogliomas
Cancer in ependymal cells is called ependymomas
Meningiomas
Tumours starting in meninges covering brain and spinal cord
Most are ‘benign’ low grade 1 or atypical grade 2, can also be malignant grade 3 but this is rare
Most common benign brain tumour in UK
More common in women
Most common in cerebrum and cerebellum but can start anywhere in meninges
Symptoms depend on where tumour is but includes seizures, weakness, loss of sight, hearing loss
Active monitoring for low grade and treatment is signs of tumor growing
Surgery to remove as much as possible but might not be able to remove all
Radiotherapy if cannot remove all, is fast growing or risk or returning or unable to have surgery
Primary lymphomas of brain and spinal cord
AKA primary CNS lymphoma. Rare
Most start in cerebrum but can also start in spinal cord (primary spinal) or eyes (primary intraocular) or leptomeninges (ary leptomeningeal lymphoma)
Most common type of lymphoma is diffuse large B cell
Symptoms include headaches, blurred vision, changes to personality, seizures, difficulty walking and balancing but depend on area involved
Treatment - surgery not usually possible without severe side effects so chemotherapy is first line (rituximab) or radiotherapy if chemo is CI, tumor left behind after initial treatment or to target whole brain (boost dose)
Medulloblastoma
Second most common brain tumor in children but most common malignant high grade children brian tumour
Around 55 children diagnosed each year
Develops at back of brain in cerebellum
Likely to grow quickly and spread to other brain areas and spinal cord; usually spread by time they’re diagnosed
Commonly diagnosed in children aged 3-4 and 8-10; more common in boys
Is an embryonal tumour
Four groups: classical (80%), anaplastic or large cell, nodular or desmoplastic (most common in infants) and medulloblastoma with extensive nodularity (MBEN)
1st line treatment is surgery and some cases chemo to first shrink tumors
Chemo and radiotherapy decided on case by case basis depending on type, spread and child age
Brain tumour presentation
New unexplained headache or neurological symptoms
Headache typically worse in morning
Nausea and vomiting
Seizures
Progressive focal neurological deficits eg diplopia, visual field defect, neurological deficits affecting upper/lower limb
Cognitive or behavioural symptoms
Symptoms related to mass - frontal lobe lesions may show personality changes, disinhibition, parietal lobe lesions may cause dysarthria
Papilloedema
Brain tumour investigations
New onset unexplained headaches or neuro symptoms needs full neurological history and examination
Bloods: CRP, ESR, coagulation screen, ADH, hypercalcaemia
CT/MRI diagnostic imaging of brain and spine
Technetium brain scan
MRA/MRS to define changning size or blood supply
Biopsy and tumour removal
Brain tumour management
Surgery to resect tumours where possible +/- photodynamic therapy
Surgery for treated hydrocephalus or reduce mass effect
Radiation can be curative (external beam radiotherapy) and is treatment of choice for metastatic brain tumors or leptomeningeal metastases
Stereotactic radiosurgery, focal radiotherapy is given to target thus avoiding exposure to normal brain tissue
Chemotherapy important in palliative care adjunct to surgery and radiotherapy
Analgesics, anticonvulsants, anticoagulants and steroids to treat symptoms
Analgesic, anticonvulsants and anticoagulants and steroids (if cerebral oedema present) for symptoms relief and reduce mass effect of raised ICP
Dexamethasone
Brain tumour complications
Acute haemorrhage into tumour
Blockage of CSF outflow causing hydrocephalus
Sudden increases in ICP can cause brain herniation
Complications of radiotherapy: acute toxicity, chronic subacute encephalopathy with somnolence and headaches (6-16 weeks after therapy), prolonged radiation may lead to impaired intellectual capacity
Peripheral nerve lesions
wrist drop: radial nerve palsy
foot drop: perineal nerve injury
Radial nerve palsy/wrist drop and pathology
Three posterior divisions of brachial plexus that form the posterior cord
Largest and most frequently injured is the radial nerve
Segmental origin is C5-8 but also sensory component from T1
Radial nerve supplies the extensor muscles allowing extension of wrist and fingers in addition to supplying the triceps; extending the elbow
Pathology:
Radial nerve injury results from acute trauma or compression of radial nerve
Saturday night syndrome (named because it can be acquired sleeping with arm over back of chair in drunken stupor, compressing the plexus)
Due to compression of lower part of brachial plexus. The median and ulnar nerves can also be involved as it is a brachial plexus injury
Nerve function usually recovers within a few weeks
Caused by any kind of trauma or compression of the nerve
Can also damage radial nerve in fractures of humerus particularly if displaced due to radial nerve running so close to humerus
Radial nerve palsy presentation, investigations and management
Loss of extensor mechanism of wrist/elbow
Loss of sensation in thumb, first two fingers and medial palm
Investigations:
Nerve conduction studies
High contrast USS to identify location, severity and cause
MRI to visualise lesions like ganglion or lipoma
Management:
Lesions from compression such as SNS and simple fractures usually recover spontaneously
General measures to reduce inflammation such as splints and anti-inflammatory drugs
Complex trauma needs exploration with view of surgical repair
Entrapment requires surgical decompression
Wrist drop, claw hand and hand of benediction nerve injury overview
wrist drop: radial nerve injury in superficial branch - loss of elbow, wrist and digit extensions, decreasing grip strength
claw hand: ulnar nerve injury in distal ulnar nerve - hyperextension of digits and flexion of 4/5th digits or at rest
hand of benediction: median nerve injury at Proximal end - extension of 1-3rd digits when trying to make fist
Foot drop
Inability to raise front part of foot due to weakness or paralysis of tibialis anterior muscle
When walking there is high stepping gait, exaggerated raising step, often walk on tiptoe on the other foot to compensate
Swing out affected leg out to side to avoid catching it on the floor
Usually due to injury of peroneal nerve
Peroneal nerve begins from L4-5, S1-2 and joins the tibial nerve (L4-S3) to form the sciatic nerve
Just above knee sciatic nerve divides into two - tibial and peroneal
Deep peroneal nerve innervates tibialis anterior (dorsiflexion of ankle)
Common peroneal runs down to back of knee winds round top of fibula, very near the surface at this point can easily be bruised or compressed
Causes of foot drop
L4-5 disc herniation can cause compression on L5 causing foot drop
Lumbosacral plexus injury eg pelvis fracture
Sciatic nerve injury eg hip dislocation: common peroneal division of sciatic nerve is commonly injured during fracture/dislocation of hip
Knee injuries: dislocation
Established compartment syndrome (late sign) from excessive tissue swelling and compression
Cauda equina
stroke/TIA
MS, CP
Diabetes, peripheral neuropathy
Brain tumors
Motor neuron disease
temporary foot drop
Crossing legs: can compress peroneal nerve on uppermost leg particularly if slim
Prolonged kneeling or squatting: certain yoga positions etc
Wearing a leg cast: enclosing the ankle and ending just below the knee can exert pressure on peroneal nerve causing foot drop
foot drop investigations and management
Clinical diagnosis
Neurological examination
Nerve conduction studies
Underlying condition: XR, USS, CT, MRI
Management:
Treat underlying cause if possible (lifestyle changes if from certain positioning etc)
Some nerve damage can heal, but can take up to two years for full recovery, chronic conditions may result in permanent foot drop.
Braces or splints to help keep foot in normal position (Ankle foot orthoses (AFOs) specialised ankle splints)
Physiotherapy exercises to strengthen muscles aiding walking symptoms, stretching exercises to prevent stiffness in heel. Learning to use high stepping or swinging gait
Specialised shoes with spring loaded bases helping prevent foot dropping while walking, can use cuff around ankle, spring above and hook in shoelace connecting to spring and pulling foot up during walking
Nerve stimulation: electrical stimulation can improve in some cases, usually used in those with disabilities like MS, stroke etc. (neuromuscular electrical stimulation/functional electrical stimulation)
Surgery depending on cause to graft or repair nerve
Carpal tunnel
Caused by compression or irritation of the median nerve within the carpal tunnel
Anything causing reduction in volume or increase in pressure within compartment can cause
Incidence peaks in 50s and 70s
Mostly in women (earlier age)
Evidence of genetic component although exact mechanism is unknown
Main risk factor is occupational - dentists and shipyard workers using high powered vibrating tools
Carpal tunnel in an anatomical compartment of the hand; bound by three sides of carpal bone and transverse carpal ligament (flexor retinaculum)
Contains 9 tendons and 1 nerve: median nerve, four tendons of flexor digitorum superficialis, four of flexor digitorum profundus and flexor pollicis longus
Associations with carpal tunnel syndrome
Pregnancy Overuse Trauma Obesity Renal failure Diabetes Inflammatory arthropathy Post-Colles fracture flexion/extension injury Use of walking aids Conditions encroaching on space within carpal tunnel (aneurysm, neurofibroma, haemangioma, lipoma, ganglion, xanthoma and gouty tophi) Menopause Inflammatory arthritides of wrist Acromegaly Renal dialysis Amyloidosis
Presentation of carpal tunnel syndrome
Tingling
Numbness
Pain in median nerve distribution can become more persistent, radiating to forearm, elbow, arm or shoulder
(thumb, index and middle finger)
Often worse at night causing wakening
Weakness in hand grip and opposition of thumb
Muscle wasting in thenar compartment in severe cases
Can be bilateral although may not be present at the same time
Investigations for carpal tunnel syndrome
Phalen’s maneuver: raise arms shoulder level, bring dorsum of hands together in midline, hold wrist in flexed position for 30-60 seconds - +ve test causes tingling and numbness over median distribution
Tinel test: tapping lightly over median nerve at wrist causes distal paraesthesia in median nerve distribution
Compression test: pressure over proximal carpal tunnel (wrist crease) with thumbs causes paraesthesia in median nerve distribution
Diagnostic doubt: electroneurography is GOLD STANDARD
Electromyography
USS
MRI
Management for carpal tunnel and prognosis
Conservative:
For mild or early disease or minimal symptoms
Symptoms may resolve within 6 months (most likely in young people <30) if symptoms are unilateral and short duration or in women who fluid retention in pregnancy is main cause
Splints beneficial to sleep in
Minimise exacerbating activities
Steroid injections - also used for diagnostic
Physiotherapy and stretching
Surgical:
Patients with severe symptoms, motor weakness of thumb or persistent sensory or motor disturbance then surgery for decompression can be done
Can produce surgery related pain, hand weakness and complications from surgery
Prognosis:
Symptoms can resolve within 6 months in about ⅓ patients particularly younger patients
Poor prognosis often associated with bilateral symptoms and positive phalen test
However the severity of symptoms often doesn’t correlate will with extent of nerve compression
Diabetic peripheral neuropathy and risk factors
Nerve damage that often occurs in diabetes
pain thought to be caused by Schwann cell dysfunction causing demyelination or from axonal loss
More than 50% type 2 diabetics older than 60 have this complication
Persistent painful neuropathy affects up to 1 in 4 people with diabetes
Nerves divided into:
Sensory
Motor
Autonomic: BP, breathing, swallowing, HR etc.
Risk factors for diabetics: Smoking History of periods of poor glycaemic control Prevalence increases with increased duration of diabetes >40 years old Signs of neuropathy HTN Coronary heart disease
Sensory neuropathy
Affects nerves recepting pain, touch, temperature and other sensations
May affect nerves in feet and legs, arms and hands (less common than lower half)
Symptoms include: tingling, numbness, unable to feel pain, unable to detect changes in temperature
Unable of position of joint, unable to coordinate movement of the joint
Burning or shooting pains tend to be worse at night
Feet are most at risk of sensory neuropathy: unaware of minor injuries, can lead to infections and ulcerations
Autonomic neuropathy
Damage to ANS causes variety of symptoms
Problems with bowel functioning (bloating, constipation, diarrhea)
Blood pressure problems, hypotension causing dizziness/syncope etc
Loss of awareness of blood sugar (hypoglycaemia)
Loss of bladder control
Irregular palpitations
Motor neuropathy
Damage to motor nerves causes weakness and wasting of muscles supplied by affected nerves
Symptoms include difficulty walking, falls, problems using hands etc
Can cause muscle twitching and cramping
Diabetic neuropathy
50% of people with diabetic polyneuropathy may have no symptoms; only diagnosed by careful regular examinations
Important to also rule out other causes of these symptoms (B12 deficiency presents very similarly, TFTs etc.)
Diabetic neuropathy can also cause severe pain, tingling, weakness and muscle wasting in thighs and pelvis (called diabetic amyotrophy) usually caused by poor glucose control and symptoms usually improve when BG control improves
Diabetic neuropathy management
Regular annual/6 month surveillance to detect changes and give early intervention
Tight glycaemic control
Prevention of foot trauma
Neuropathic pain: Duloxetine FIRST LINE, amitriptyline, gabapentin or pregabalin initial treatments
Capsaicin cream for localised neuropathic pain who can’t tolerate oral treatments
Opioids other than tramadol should be AVOIDED unless specialist arrangement
Review drug treatments early for titration or when changing doses for adverse effects and tolerability
Referral to pain clinic for severe pain, significant limitation of activity or underlying condition deteriorating
Acute peripheral neuritis
Often abrupt onset
Not related to duration of diabetes
Can completely resolve
Symptoms include burning foot pain, often worse at night
Associated with poor glycaemic control but sometimes initially establishing good glycaemic control
Examination may be normal apart from hyperaesthesia
acute painful neuropathy
Results from rapid improvement of BG control
Self limiting condition that improves over time
May need regular treatment for several weeks to be effective:
1st line simple analgesics (paracetamol, aspirin, bed cradles)
Next try neuropathic pain management
Prognosis and prevention of diabetic neuropathy
Diabetic peripheral neuropathy is major cause of morbidity and increased mortality
Increases risk of burns, injuries and foot ulceration
More likely to undergo lower limb amputations
Annual foot checks, compliance with diabetic review appointments
Good glycaemic control severely reduces risk of neuropathy
Smoking cessation and good self care (regular checking of feet etc) improves prognosis
B12 deficiency signs and symptoms
Dizziness High temp Mood changes Changes to mobility pale/jaundiced skin glossitis/mouth ulcers Disturbed vision Fatigue Paraesthesia
Spinal cord lesions types
Trauma Spinal stenosis Herniated disc MS Tumours Congenital malformations often associated with spina bifida/scoliosis Spinal abscess Vertebral osteomyelitis
Spinal cord tumours
Primary spinal cord tumours are rare (2-4%)
Start in spinal cord
More common in adults than children
Most common types are: astrocytomas or ependymomas (usually begin within the spinal cord), meningiomas, neurofibromas and schwannomas (spinal nerves)
Extramedullary tumours grow either in meninges or nerve roots, do not begin within the spinal cord itself but can still affect spinal cord function by compression. Eg meningiomas, neurofibromas, nerve sheath tumours and schwannomas
Spinal cord tumour presentation
Persistent progressive back pain Worse at night affects sleep History of cancers Weight loss Fatigue Lethargy Night sweats Loss of appetite Not activity related Muscle weakness/numbness or change in sensation Change in bowel or bladder function
Spinal cord tumour investigations and management
MRI
Biopsy
Blood tests
LP
Management:
Depends on tumor type, position and age of patient
Surgery to remove as much as possible
Radiotherapy if not all removed, high grade or returns post surgery
Steroids
Chemotherapy if returns again after treatment
herpes zoster neuropathy overview
HZ pain may be classified as:
Acute-phase pain, including prodromal, lasting 30 days
Sub-acute pain, which may occur between 30-120 days
Chronic-phase pain, also known as PHN, which persists for more than 3 months after rash has healed
Progression of rash: Erythematous patches Vesicles Pustules Scabs Most common dermatomes infected are the thoracic (50%)
first line treatment for pain:
- TCAs and SNRIs
2nd line: opioids including tramadol
3rd line topical capsaicin
HIV neuropathy presentations
Neuropathic pain examples include:
Distal sensorimotor polyneuropathy (DSP)
Antiretroviral toxic neuropathy (ATN)
Acute HZ and PHN
Mononeuritis multiplex, i.e. sensorimotor deficit in the distribution of single or multiple cranial, spinal or peripheral nerves
Diffuse infiltrative lymphocytosis due to CD8 T-cell infiltration into glands, organs and nerves
Radiculopathy due to nerve damage by the HIV retrovirus itself or through co-infection with the cytomegalo virus (CMV)
Musculoskeletal pain, e.g. caused by polyarthritis, psoriatic arthritis, myopathies associated with HIV and HAART and HIV-wasting syndrome
Gastrointestinal and gynaecological pain
About 50% of patients complain of headaches.
A CD4 count >200 × 109 indicates an uncomplicated headache but a CD4 count <200 × 109 may indicate a central nervous system (CNS) malignancy.
A CD4 count indicates the number of T-helper lymphocytes per cubic millimeter of blood and is a good predictor of immune health.
HIV neuropathy management
Management: guided by HIV specialists establish CD4 and HIV load treat with antiviral drugs to improve immunity = often improves neuropathy symptom management with simple analgesia
If the pain is severe in ATN, HIV physicians may consider withdrawing one of the NRTIs. However, withdrawal may result in HIV becoming resistant to drugs
gabapentin and pregabalin might be considered as first-line treatments and opioids reserved as a second-line agent
biopsychosocial aspects of pain should be explored and other treatments, e.g. TENS, acupuncture and CBT, can be considered.
trigeminal neuralgia and presentation
peak incidence to be between 45-69 years of age and a higher incidence in women than men Trigeminal neuralgia (TN) is a severe, incapacitating pain in the distribution of one of the divisions of trigeminal nerve (5% ophthalmic branch, 40% maxillary and 55% mandibular branch) characterized by pain that is:
Sudden Usually unilateral Severe Brief Stabbing Recurrent can be bilateral in certain conditions such as multiple sclerosis, or with an intracranial tumour.
classified TN into two types:
Classical TN, where no other cause is found apart from vascular compression of the trigeminal nerve
Symptomatic TN, all other patients
trigeminal neuralgia management
Carbamazepine is a first-line drug. It is well established and proven to be effective but its usage is restricted by adverse effects. e.g. Steven-Johnson syndrome and toxic epidermal necrolysis.
Oxycarbamazepine is a second-line agent. It is a prodrug of carbamazepine and has fewer side-effects compared to carbamazepine. Other second-line agents used are pregabalin, lamotrigine and baclofen.
IV Phenytoin can be used in acute settings.
Medical therapy can fail due too poor tolerance of drugs.
surgical:
cryotherapies, laser ablation
alcohol injections, phenol injections
microvascular decompression
Psychological co-morbidities, e.g. anxiety and depression can co-exist in these patients. The presence of co-morbidities increase the patient’s perception of pain.
The multidisciplinary team should address these issues.
Chronic IDP
Chronic inflammatory demyelinating polyneuropathy
Rare neurological disorder where nerve root and peripheral nerve inflammation and destruction of myelination occurs
This can lead to nerve conduction slowing or loss of nerve fibres
Causes weakness, paralysis and impaired motor function especially in the arms of legs, usually symmetrically
Sensory disturbance can also occur
Can follow a pattern of relapse and remission, or slow steady progression and can vary greatly between individuals
Guillain-Barre syndrome is an acute form of IDP and the most common form of the condition
Exact cause is unknown but thought to be autoimmune
Chronic IDP variants
Multifactorial: asymmetric symptoms may involve different nerves, called Lewis-Sumner syndrome/MADSAM
Pure sensory variants with imbalance and incoordination but no muscle weakness
Distal variant where sensory and motor deficits do not involve proximal arms and legs
Pure motor variant with no sensory abnormality
Chronic IDP presentation
Slowly progressive (over at least two months) Symmetric weakness in muscles of hip/shoulder and hands/feet Sensation changes/loss of sensation Incoordination Numbness, tingling Fatigue Burning pain Difficulty swallowing Diplopia Muscle atrophy Deep tendon reflexes are reduced/absent Abnormal gait Response to touch/pain may be impaired
Chronic IDP diagnosis and management
Symptoms must be present for at least two months Clinical diagnosis Nerve conduction studies Electromyography LP and CSF analysis
Management:
Steroids - prednisolone
IV immunoglobulins
Plasma exchange
semi and diplegic gait pathologies
Hemiplegic gait: elbow flexion and knee extension. Walk with leg circumduction due to extension of knee. Cannot swing arms symmetrically/one arm is flexed permanently. Leg is internally rotated. Most commonly seen in stroke
Diplegic gait: both sides have spasticity worse in lower extremities. Abnormally NARROW boase, dragging legs. Extreme tightness of hip adductors causing legs to cross in midline (SCISSORS GAIT) in extreme cases. Commonly seen in cerebral palsy.
Neuropathic and myopathic gaits
Neuropathic gait: foot drop causing patient to increase height of leg to avoid tripping. Unilateral cases caused by peroneal nerve palsy, L5 radiculopathy. Bilateral causes include carcot-marie-tooth disease and other peripheral neuropathies (diabetic)
Myopathic gait: hip girdle muscles weak unilaterally leading to drop in pelvis on contralateral side (TRENDELENBURG SIGN) with bilateral weakness shows pelvis drop on both sides leading to WADDLING. Seen in muscular dystrophy
Choreiform, ataxia, parkinsonian, sensory gaits
Choreiform gait: displays irregular chorea, dystonia, athetosis. Shows jerky movements in all extremities not limited to walking. Seen in huntington’s disease
Ataxia gait: clumsy, staggering walk with wide based gait swinging from side to side. Likely to walk diagonally TOWARD the side of the lesion. Cannot walk heel-toe or in straight line. Seen in cerebellar disorders
Parkinsonian gait: rigidity and bradykinesia. Stooped with head and neck forward, flexed knees. Everything is flexed including upper limbs. SHUFFLING GAIT, small little steps taken, may have difficulty initiating walking in first place. Seen in parkinson’s disease
Sensory gait: proprioceptive loss causes feet to SLAM into the floor to gain signals that feet are on the floor. Exacerbating in the dark due to lack of eyes reinforcing proprioceptive information. Is a STOMPING GAIT. lift legs very high off the ground to avoid tripping also. Seen in B12 deficiency, tabes dorsalis or peripheral nerve disease (diabetics)
Scoring of power for neuropathy exams
0 - no contraction
1 - flicker/trace of contraction
2 - active movement with gravity eliminated
3 - active movement against gravity
4 - active movement against gravity and resistance
5 - normal power
Benign essential tremor and presentation
Common conditions associated with older age
Characterised by fine tremor affecting all voluntary muscles
Most noticeable in hands but can affect head, jaw, vocals etc
Features: Fine tremor Symmetrical More prominent on voluntary movement Worse when tired, stressed or after caffeine Improved by alcohol Absent during sleep
essential tremor differentials and management
Parkinson’s MS Huntington’s chorea Hyperthyroidism Fever Medications (salbutamol, antipsychotics)
Management:
No definitive treatment; it is not harmful and needs no treatment if not causing functional or psychological problems
Alcohol can improve symptoms but must be used with caution - no more than 14 units a week spread over at least 3 days
Propranolol or primidone can be tried to improve symptoms if they are significant; can be taken long term or intermittently for social occasions etc.
Surgery (thalamotomy and thalamic deep brain stimulation) can be consider for extreme cases
Parkinson’s disease
Progressive reduction of dopamine in the basal ganglia of the brain, leading to disorders of movement.
Symptoms are characteristically asymmetrical one side affected more so
Classic triad of symptoms:
Resting tremor
Rigidity
Bradykinesia
Pathophysiology:
Basal ganglia situation in middle of the brain are responsible for coordinating habitual movements such as walking, looking round, controlling voluntary movements and learning specific movement patterns
Part of basal ganglia called the substantia nigra produces dopamine, essential for correct functioning of basal ganglia
In parkinson’s there is progressive gradual fall in dopamine production
Parkinson’s presentation and factors associated
Increasing age
Unilateral tremor: pill rolling tremor more pronounced when resting and improves with voluntary movement
Cogwheel rigidity - resistance to passive movement of joint (test elbow)
Bradykinesia - movements get slower and smaller (handwriting smaller, small shuffling gait, difficulty initiating movement from standing to walking, difficulty turning round when standing, reduced facial movements and expressions HYPOMIMIA)
Factors often affecting parkinson’s patients: Depression Sleep disturbance and insomnia Anosmia (loss of sense of smell) Postural instability Cognitive impairment and memory problems
compare essential and Parkinson’s tremor
parkinson’s:
- asymmetrical
- slower
- worse at rest
- improves with intentional movement
- other Parkinson’s features
- no change with alcohol
benign essential tremor:
- symmetrical
- faster
- improves at rest
- worse with intentional movement
- no other features
- improves with alcohol
Parkinson’s plus syndromes and diagnosis
A group of neurological conditions similar to Parkinson’s disease but with atypical characteristics Can mimic other conditions Most common types are: - multiple system atrophy - dementia with Lewy bodies - progressive supra nuclear palsy - corticobasal degeneration
Diagnosis:
Clinical presentation, examination and history
Must be diagnosed by specialist (NICE recommend using UK parkinson’s disease society brain bank clinical diagnostic criteria)
Multiple system atrophy
Rare Parkinson’s plus condition where neurons of multiple systems in brain degenerate
Affects basal ganglia and other areas
Degeneration of basal ganglia leads to parkinson’s presentation
Degeneration in other areas can lead to autonomic dysfunction (postural hypotension, constipation, abnormal sweating and sexual dysfunction) and cerebellar dysfunction (ataxia)
Dementia with Lewy bodies
Rare type of dementia associated with features of parkinsonism (parkinson plus syndrome)
Causes progressive cognitive decline
Some symptoms include visual hallucinations, delusions, disorders of REM sleep and fluctuating consciousness
Progressive supranuclear palsy
Rare parkinson plus disorder causes balance, stability issues when walking or standing
Rigid stiff muscles especially the neck and spine making body movement difficult
Symptom most distinguishing from Parkinson’s is eye movements occur in addition to body movements
Problems with speech and swallowing are also very prominent as are depression and emotional difficulty
Rarely causes tremors
Progresses faster than Parkinson’s and often leads to disability within 5-10 years
corticobasal degeneration
Rare parkinson plus syndrome, causes many areas of the brain overtime to shrink
Initially may be unilateral but eventually affects both sides with progression
Symptoms are stiff, rigid, tremor, balance and coordination problems, speech problems, concentration difficulty, progressive movement, thinking and memory issues
Gradually may have difficulty understanding spoken or written language and can develop ‘alien limb’ where movements cannot be controlled
Parkinson’s plus syndrome management
Initiated and guided by specialists tailed to individual patients aimed at symptom relief and minimising side effects
Levodopa: synthetic dopamine given orally usually combined with drug preventing it being broken down in body before it can enter the brain (peripheral decarboxylase inhibitors like carbidopa and benserazide) combination drugs are co-beneldopa and co-careldopa
This is most effective treatment but becomes less effective over time often reserved when other treatment no longer manage
Dyskinesia is main side effects of dopamine excess - excessive motor activity: dystonia (exaggerated movements or abnormal postures), chorea (involuntary jerking) or athetosis (involuntary twisting or writhing movements usually in finger, hands or feet)
COMT inhibitors: COMT enzyme metabolises levodopa in body and brain so this prevents breakdown
Dopamine agonists: eg bromocriptine, pergolide etc. mimic dopamine in basal ganglia and stimulate dopamine receptors. Less effective than levodopa, usually used to delay use of levodopa, used in combination with levodopa to reduce dosage. Prolonged use can cause pulmonary fibrosis
Monoamine oxidase-B inhibitors: eg selegiline, rasagiline. monoamine oxidase-B breaks down NTs like dopamine, serotonin and adrenaline. Blocks action and increases effects of NTs.
dementia
Syndrome caused by number of brain disorders causing memory loss, decline is some aspects of cognition, difficulties with activities of daily living
Three groups of symptoms:
Cognitive impairment: difficulty with memory, language, attention, thinking, orientation, calculation and problem solving
Psychiatric or behavioural disturbances: change in personality, emotional control, social behaviour, depression, agitation, hallucinations and delusions
Difficulties with activities of daily living such as driving, shopping, eating and dressing
Common causes of dementia: Alzheimer’s Vascular dementia Dementia with Lewy bodies Frontotemporal dementia Mixed dementia Parkinson’s
dementia diagnosis
Diagnosis:
Comprehensive history and physical examination
Attention and concentration
Orientation
Memory short and long term
Praxis - can they get dressed, lay a table
Language function
Executive function - problem solving etc
Psychiatric features - depression, anxiety, psychotic symptoms
Medication review
Formal screen for cognitive impairment
Screen and appropriately treat depression
Consider bloods: FBC, ESR, CRP, U+Es, LFT, eGFR, Ca2+, TFT, B12 and folate
CXR/MRI
CSF analysis
Diagnostic criteria for all dementias:
Cognitive or behavioural symptoms which:
Affect ability to function in normal activity
Represent decline from previous level of function
Cannot be explained by delirium or other psychotic disorder
Have been established by history taking from patient/informant and formal cognitive assessment
Involve impairment of at least two of following domains:
Ability to acquire and remember new information
Judgement, ability to reason or handle complex tasks
Visuospatial ability
Language functions
Personality and behaviour
dementia presentation and management
Presentation:
Subtly different depending on type
Alzheimers tends to be insidious onset while vascular dementia typically has gradual increase in severity
management Within 6 week referral Early discussion for advance planning Memory assessment service Valid consent sought for treatments where possible Assess carer needs
Alzheimer’s
Most common cause of dementia
Involves progressive degeneration of the cerebral cortex
Widespread cortical atrophy
Neurons affected develop surrounding amyloid plaques, neurofibrillary tangles and produce less ACh
Cause yet unknown
Patients have irreversible global progressibe brain function impairment leading to reduced intellectual ability
Neurodegeneration begins at least a decade before clinical onset
Risk factors: Ageing Caucasian Family history Apo E4 variant gene Midlife obesity Smoking (controversial)
Alzheimer’s presentation
Onset progresses slowly over 7-10 years
Early stage symptoms: memory lapses, forgetting names and places, difficulty finding words, inability to remember events, forgetting appointments etc
Progressive symptoms: language difficulties, apraxia, problems with planning and decision making, confusion
Later stages: wandering, disorientation, apathy, psychiatric symptoms, behavioural problems, altered eating habits, incontinence
Alzheimer’s investigations and management
Investigations:
Cognitive impairment tools
Exclude other causes
Management: Group cognitive stimulation programmes Memory enhancement strategies CBT AChE inhibitors Palliative and end of life care
Encephalopathy
General term describing disease affecting function or structure of the brain
Many types some permanent and some temporary, some congenital and some acquired and may be progressive
Types: Chronic traumatic Glycine Hashimotos Hepatic Hypertensive Hypoxic ischaemic Toxic-metabolic Infectious Uremic Wernicke’s
symptoms, investigations and management of encephalopathy
Mental changes: memory, concentration, problem solving, personality changes, lethargic and drowsy
Neurological symptoms: muscle weakness, poor decision making, involuntary twitching, tremors, speech or swallowing difficulty, seizures
Investigations: Bloods cultures, hormonal profile, ESR, CRP, U+Es, LFTs CSF analysis CT/MRI EEG
management
depends on cause
Wenicke’s and uremic and infectious encephalopathy
Wenickes encephalopathy:
Result of vit B1 deficiency, long term alcoholism, poor nutrition, poor food absorption
Can lead to korsakoff syndrome if untreated
Uremic encephalopathy:
Result of kidney failure caused by uremic toxins in blood
Causes mild confusion to deep coma
Infectious encephalopathies Transmissible spongiform encephalopathies are also known as prion diseases. Prions are proteins that occur naturally in the body, but they can mutate and cause diseases that gradually damage and deteriorate your brain (neurodegenerative diseases). Prion diseases include: chronic wasting disease fatal familial insomnia kuru Creutzfeldt-Jakob disease
vasculitis
Inflammation of blood vessels
Rare
Can be primary or secondary (result of infection or in association with another condition like rheumatoid arthritis)
Most commonly is idiopathic
Infection and inflammatory diseases are second most common causes
Can also be drug induced or neoplastic
vasculitis classification
Infective causes: where there is direct invasion by pathogens into the vascular wall, resulting in inflammation such as rickettsial vasculitis, syphilitic aortitis and aspergillus arteritis.
non-infective: large vessel (GCA), medium vessel (Kawaksaki disease), small vessel (immune complex), variable vessel (Behcet's disease) single organ (isolated arthritis), systemic disease associated (rheumatoid) and probably associated (Hepatitis)
small vessel vasculitis presentation and differentials
Palpable purpura 1-3mm can form plaques or ulcers Tiny papules Splinter haemorrhages Urticaria Vesicles Livedo reticularis
differentials: Henoch-schonelein purpura Hepatitis C HIV Drug hypersensitivity reactions IBD
medium vessel vasculitis presentation and differentials
Ulcers Digital infarcts Nodules Livedo reticularis Papulo-necortic lesions Hypertension - damage to renal vessels
differentials GPA vasculitis Kawasaki disease GCA Rheumatoid arthritis Infections eg TB
large vessel vasculitis presentation and differentials
End organ ischaemia
HTN
Aneurysms
Dissection or haemorrhage or rupture
differentials Kawasaki disease Rheumatoid arthritis Syphilis and TB GCA Takayasu arteritis
Lyme disease
Caused by bacterium Borrelia burgdorferi which is a spirochaete
Disease is caused by infection and body immune response to the infection
Different strains cause different clinical manifestations
Transmitted from host to host by ixodes ticks or deer ticks
Ixodes ticks emerge from larval form in summer and feeds on one animal host, then becomes nymph and feeds only once again on another host (humans can be victims in this stage)
In autumn the adult tick emerges to feed on deer again once. Humans can also be host at this stage. The tick must be significantly infected to pass on spirochaete infection
Often the host clears the infection and remains asymptomatic but seropositive OR elicits immune response causing clinical presentation
Lyme disease presentation
early/stage 1/localised disease: circular rash at site of infestation within 3-36 days. Rash is round/oval, pink/red or purple. Often central erythema with sparing around it giving a target like appearance. Eventually resolves after some weeks
later/stage 2 disease/disseminated: flu like symptoms; malaise, muscle and joint pains, fever, tiredness, nausea or vomiting
Neurological disorders can occur in 10% untreated cases: uni/bilateral facial nerve palsies, meningism, meningitis, mild encephalitis, peripheral mononeuritis
Cardiovascular problems
Lymphocytomas - bluish red nodular lesions typically on earlobe or nipple
late/stage 3 disease: arthritis, acrodermatitis chronica, late neurological disorders (polyneuropathy, chronic encephalomyelitis, vertigo and psychosis), chronic lyme disease
Lyme disease management
Remove tick with fine tipped tweezers pull upwards without twisting
Clean site after with antiseptic
Rash: Treat oral antibiotics for 2-3 weeks doxycycline or amoxicillin
Seek advice from infectious diseases for any other symptoms with likely history of tick bite
young onset movement disorders
Unwanted movements or difficulty moving in the way intended in children
Includes a broad variety of conditions with wide variety of causes, affecting multiple parts of the body and can change in location and severity over time
May occur spontaneously or only with movement/specific movements or can have trigger factors or situations where they can worsen
Causes include brain injury, head trauma, infection, inflammation, metabolic disturbances, underlying diseases, toxicity or medication side effects
Types of movement disorder:
- essential tremor
- myoclonus
- dystonia
- tic disorders (Tourette’s)
- ataxia (Friedreich ataxia or ataxia telangiectasia)
- Parkinsonism
Tic disorders
sudden involuntary movements or sounds that come and go. Daily common in children. Muscle tics can involve any body part and vary in severity. Children are usually able to suppress tics temporarily but feel a building sense of ‘wrongness’ as they resist. If tics persist despite treatment then a child may need to see a specialist. About 10-15% children will see tics progress and become potentially disabling but most tic disorders resolve or become minimal by time they reach their 20s.
Tourette’s syndrome: if a child has both vocal and motor tics that last longer than a year. A neuropsychiatric disorder characterised by sudden repetitive rhythmic motions. Has a strong genetic trait but thought to be multifactorial and associated with ADHD and OCD disorders. Patients typically present with persistent multiple vocal or motor tics usually before age 18. Other factors include echolalia (meaningless repetition of another person’s spoken words), making obscene gestures and foul language (pathognomonic but only appears in 10% cases). Treatment is supportive with habit reforming
Parkinsonism in children
children with Parkinson’s disease have at least two symptoms: muscle rigidity, balance problems, frequent falls, slow movement of tremors. By itself is the least common of young onset movement disorders. Most often occurs as a side effect of medications. Can result from genetic brain degeneration such as Wilson disease, juvenile Huntington’s and lysosomal disorder and various forms of brain injuries
Ataxia in children
failure of motor coordination caused by injury or dysfunction of the cerebellum. Symptoms are clumsiness, poor balance, irregular movements and inability to perform fine tuned or smooth muscles. Some cases come abruptly e.g. after stroke, inflammation or infection and typically improve as they are treated. Others can come and go repeatedly such as those caused by epilepsy, genetic mutations, metabolic disorders or atypical types of migraines.
Friedreich ataxia: is the most common inherited ataxia in the developed world (autosomal recessive). Patients have mutation in dataxin gene (chromosome 9) leading to impaired frataxin production (mitochondrial polypeptide). Usually show in teens/2-s with progressive ataxia, weakness and loss of proprioception and joint position.
Ataxia telangiectasia: rare autosomal recessive condition presents usually in early childhood as: progressive cerebellar ataxia, basal ganglia dysfunction, immunodeficiency (specifically IgA leading to recurrent infections), increased risk of cancers, telangiectasia anywhere on body, elevated alpha fetoprotein levels
dystonia in children
faulty brain signals causing groups of muscles to abnormally contract in uncoordinated manner e.g. both antagonist pairs of muscles are activated simultaneously. Causes painful twisting movements and awkward contorted postures. Often triggered by specific actions like writing or walking but can happen at rest. Symptoms often start in one body part then spread to other areas
myoclonus in children
very quick sudden involuntary muscle jerks that cannot be suppressed. Can vary in frequency between individuals, may occur randomly or semi-rhythmic patterns and can be up to 50 times per minute. Can sometimes have triggers like certain postures or being startled. Often a benign condition with no long term health effects but must be evaluated by neuro specialists to identify type and cause (includes CJD, MS, SLE). Can occur after severe brain injury or from metabolic disorders/neurodegenerative diseases. Can also be part of myoclonic epilepsy so is recommended children presenting with myoclonus should be tested for epilepsy too. May respond to clonazepam or sodium valproate. Can present in childhood/adolescence
tremor in children
can run in families, can be a temporary part of a child’s motor development or be long lasting. Unlike other movement disorder children can sometimes consciously suppress tremors
prion disease
Group of several conditions
Prion is a type of protein normally found in the brain on surface of many cells
Can become abnormal and clump; that can trigger normal proteins in the brain to fold abnormally resulting in dysfunction and brain damage
This can cause memory impairment, personality changes, movement difficulty
Can affect both humans and animals and sometimes is spread via contaminated meats
Most common form is creutzfeldt-jakob disease (CJD)
They are rare but generally fatal diseases
types of prion disease
Can be genetic (familial CJD) or sporadic (develops suddenly without known risk factors)
Most cases are sporadic and tend to affect those aged 60
Can be caused by exposure to infected tissue
Symptoms develop quickly and can be fatal
Most cases death occurs within a year
Variant CJD:
Mad cow disease
Eating diseased meat can cause in humans, usually affects younger people
VPSPr:
Extremely rare, similar to CJD but protein is less sensitive to digestion
More likely in those 70+ with family history of dementia
Gersstmann-straussler-scheinker GSS:
Extremely rare but occurs in those typically 40 years old
Kuru:
Seen in new guinea
Caused by eating infected human brain tissue
Now is rare due to awareness of condition
Fatal insomnia:
Rare hereditary disorder causing sleep difficulty
Also sporadic form present
prion disease risk factors and presentation
Risk factors:
Family history
Eating infected meats
Infection from receiving contaminated corneas or contaminated medical equipments
Symptoms: Rapidly developing dementia Difficulty walking and gait changes Hallucinations Muscle stiffness Confusion Fatigue Speech difficulty
prion disease investigations and management
Investigations:
MRI brain
CSF analysis
Electroencephalogram to analyse brain waves
Bloods
Neurologic and visual exams to see nerve damage and vision loss
Management: No cure but aimed at slowing disease Prevention - infectious meats, contaminated medical equipment dealt with/cleansed End of life care and planning Prions disease is always fatal
Whipple’s disease and risk factors
Chronic relapsing multisystem disease characterised by weight loss, chronic cough, fat accumulation in intestine, mesenteric lymph nodes and stool
Now thought to be caused by infection of tropheryma whipplei and defective cell mediated immunity
Extremely rare condition
Risk factors: Middle age or elderly Males Caucasian Family history HLA-B27 antigen Sewage plant workers, farmers and agricultural workers
Whipple’s disease presentation
Can be asymptomatic Polyarthralgia - transient and episodic (often prodromal symptom) GI symptoms: abdo pain, diarrhoea, anorexia and weight loss, distension, flatulence, steatorrhea, GI bleeding Fever Chronic cough Hyperpigmentation LAP Anaemia Cardiac abnormalities Eye involvement Pulmonary involvement Skin problems
Whipple’s disease investigations and management
Investigations: High clinical suspicion Bloods and malabsorption tests are non-specific Biopsy of affected tissue PCR of bacterial RNA (CSF analysis)
Management:
Antibiotics - need expert microbiology advice
Repeat PCR at end of treatment
Without treatment progression is fatal
cauda equina
Spinal emergency
Disease affecting the tail end of the spinal cord (cauda equina)
Cauda equina is formed by nerve roots in caudal to level spinal cord termination
The syndrome is caused by compression of the nerves causing bladder/bowel dysfunction, reduced sensation in saddle area and sexual dysfunction with possible neurological deficit in lower limb
Causes: Herniation of lumbar disc is most common cause Tumours Trauma Infection including epidural abscess Congenital Spongylolithesis Postoperative haematoma After spinal manipulation sarcoidosis
cauda equina presentation
Most cases have sudden onset and progress rapidly within hours or days but can evolve slowly and patients do not always have pain
Low back pain with pain in legs (uni or bilateral) motor/sensory abnormality
Lower limb weakness
bowel/bladder dysfunction with saddle anaesthesia
Urinary retention/incontinence, bowel incontinence/constipation
Sexual dysfunction
cauda equina Ix, Mx and complicaitons
Investigations: Clinical diagnosis MRI Myelography or CT Urodynamic studies
Management:
Immobilise spine if trauma
Surgery to remove blood, bone, tumours, herniated discs or growths
Lesion debulking for space occupying lesions (tumours/abscesses)
Radiotherapy is malignant
NSAIDs if ankylosing spondylitis
Infection treated
Address lifestyle issues, physiotherapy PRN
Complications:
Paralysis
Sensory abnormalities
bladder/bowel and sexual dysfunctions
neurosarcoidosis and presentation
Sarcoidosis is an auto-inflammatory disorder characterised by granulomatous inflammation in affected tissues, most commonly the lungs, skin, eyes and liver
Sarcoidosis affects the nervous system in 10% of cases
Has varying degrees of severity
Is very difficult to diagnose without histological confirmation
Presentation:
Subacute, rarely acute
Malaise, fever
Night sweats
Lofgren’s syndrome: fever, arthritis of ankles, erythema nodosum and hilar LAP
Heertfordt’s syndrome: granulomatous uveitis, parotid and submandibular salivary gland swelling and cranial neuropathy (usually facial nerve)
Lung involvement: cough, SoB, chest tightness, stabbing pains, often asymptomatic
Eyes, skin, cardiac, hepatic, renal, bones and joint involvement also common
neurological involvement in neurosarcoidosis
Isolated cranial neuropathies: facial nerve most common (70%, mostly unilateral); optic neuropathy is common (subacute optic neuritis; optic perineuritis)
Peripheral nerve involvement 10%: peripheral neuropathy, acute inflammatory demyelinating polyradiculoneuropathy.
5% brain and spinal cord lesions
Pituitary and hypothalamic involvement associated with diabetes insipidus, endocrine symptoms most commonly
Pachymeningitis: dural inflammation causing headaches, hydrocephalus
Leptomeningitis
Vasculitis
Cauda equina
management of neurosarcoidosis
Corticosteroids are basis of treatment in all granulomatous diseases
Immunosuppressants like infliximab
Small risk of relapsing
olfactory nerve overview and assessment
Origin: cerebral hemisphere Innervation of nasal mucous membranes Function: smell Dysfunction: ansomia Clinical evaluation: non-noxious aromatic substances ie coffee, lemon, garlic etc. test esch nostril separately and note abnormalities
optic nerve overview and assessment
Origin: cerebral hemisphere
Function: visual acuity (test with snellen chart for far vision, Jaegers chart or fingers for near vision), visual fields (confrontation), fundi and optic discs (look through pupil with ophthalmoscope to see termination of optic nerve)
Evaluation: visual acuity = near/far field tests.
Colour vision = colour matching.
Visual field = confrontation test
accommodation reflex
oculomotor III, trochlear IV and abducens VI nerve overview and assessment
oculomotor nerve III
Origin: midbrain
Innervation: eyelid, ciliary, sphincter of iris
Function: eye movement medially, superiorly, inferiorly and externally; pupil constriction, shape and equality; elevates upper eyelid and accommodation reflex
Dysfunction: unable to move eye up/down/side to side (DYSCONJUGATE GAZE); ptosis uni or bilateral; loss of accommodation reflex
Trochlear nerve IV:
Origin: midbrain
Innervation: superior oblique muscle
Function: down and inward movement of eye
Dysfunction: loss of downward and inner eye movement (DYSCONJUGATE GAZE)
(same tests as III oculomotor)
abducens nerve VI Origin: pons Innervation: lateral rectus muscle Function: outward lateral eye movement Dysfunction: loss of lateral outward eye movement (DYSCONJUGATE GAZE)
ASSESSMENT:
- H test (eye movements)
- observe for asymmetry, direct light response, consensual response, accommodation reflex, extra ocular movements (abducens)
trigeminal V nerve overview and assessment
Origin: pons/midbrain
Innervation: three branches: ophthalmic, maxillary and mandibular to masseter and temporal muscles, skin and mucous membranes in head (including dura mater)
Function: sensation of pain, touch, temperature, motor movement of masseter and temporal muscles
Dysfunction: if sensation in all three branches indicates PERIPHERAL INJURY. brainstem/upper cervical cord injury can cause loss of sensation in one or more branches. Loss of corneal reflex.
Evaluation:
- sensation
- motor exam of chew muscles
- jaw jerk reflex
- corneal reflex
Facial nerve VII overview and assessment
Origin: pons and medulla
Innervation: anterior ⅔ tongue; facial muscles, scalp, ear and neck
Function: control facial muscles, motor limb of blink and corneal reflex, secretion of salivary and lacrimal glands, sensation of taste ⅔ anterior tongue
indications:
Ipsilateral weakness/paralysis = Bell’s palsy or LMN lesions
weakness of lower face = UMN lesions eg CVA
Bilateral weakness/paralysis = myasthenia graves or Guillain Barre syndrome
parasympethetic - loss of salivation
sensory - loss of taste
combined - speech difficulty or drooling
evolution:
- facial movements
- corneal reflex
vestibulocochlear nerve VIII overview and assessment
Origin: pons and medulla
Innervation: cochlear (ear); vestibular (ear)
Function: hearing and balance, maintains body position and proprioception
Dysfunction: ataxia; unsteady gait, vertigo, balance disturbances
Evaluation: RULE OUT wax, pus, blood or foreign body before testing; this branch is not normally tested unless patient gives history of vertigo or balance disturbance
Rinne’s test = normal = AC>BC; sensoneural hearing loss = AC>BC; conductive hearing loss = BC>AC (RINNE -VE)
Weber’s test - sensoneural hearing loss will travel to NORMAL ear
conductive deafness will travel to SAME SIDE as lesion
turning test
glossopharyngeal IX nerve overview and assessment
Origin: medulla
Innervation: mucous membranes of tonsils, pharynx, posterior ⅓ tongue, pharyngeal muscles, carotid sinus and carotid body
Function: taste from posterior ⅓ tongue, afferent of gag reflex, swallowing
Dysfunction: loss of taste
Evaluation: ask about changes to taste recently, ask to swallow water and observe symmetry,
Vagus nerve X overview and assessment
Origin: medulla
Innervation: muscles of larynx, pharynx, soft palate, parasympathetics to thoracic and abdominal viscera
Function: muscles of larynx, pharynx, soft palate, sensation from heart, lungs, GIT, carotid sinus and carotid body, efferent limb gag reflex and swallowing
Dysfunction: loss of gag reflex and swallow reflex, loss of carotid sinus response
Evaluation:
- soft palate and uvula
- cough
- swallow
spinal accessory nerve XI overview and assessment
Origin: medulla
Innervation: sternocleidomastoid and trapezius
Function: motor function of these muscles
Dysfunction: muscle weakness
Evaluation: look for asymmetry
hypoglossal nerve XII overview and assessment
Origin: medulla
Innervation: muscles of tongue except palatoglossus
Function: tongue movement
Dysfunction: unilateral lesions can cause paresis, atrophy, furrowing, fibrillation and fasciculation on affected half. On protrusion tongue deviates towards affected side due to contralateral action of genioglossus muscle
Flaccid paralysis: dysphagia, dysarthria, dyspnea, difficulty chewing foods
tropical disease
Diseases that are prevalent/unique to tropical and subtropical regions
They are less prevalent in temperate climates due to cold seasons usually determining insect populations
Insects such as mosquitoes/flies are most common disease carriers (vectors)
These may carry a parasite, bacterium or virus infectious to humans or animals
Vaccines are not available for most diseases listed and many do not have cures
Human exploration, deforestation and air travel has increased incidences of these diseases
diseases: Chagas Schistosomiasis Leishmaniasis taeniasis/cysticercosis Malaria Dengue and severe dengue Trypanosomiasis Leprosy Buruli ulcer Guinea worm disease Ebola haemorrhagic fever Cholera Soil transmitted helminth infections Raft valley fever
Malaria
Malaria is a protozoan parasite widespread in tropics and subtropics
Mortality is 0.2% and each year 500 million are affected
In endemic areas mortality is mainly in infants and those who survive to adulthood acquire significant immunodeficiency
Transmitted by bite of infected female anopheline mosquitoes; can also be vertically transmitted mother to baby
Types:
Plasmodium falciparum - most fatal; symptoms of mild infection which rapidly progress to fever with rigors, severe multiorgan failure, coma and death
Non-falciparum malaria - P. vivax, P. ovale, P.malariae which cause more benign illness but may relapse after treatments
Pathogenesis of malaria
Insect bite; sporozoites pass through skin and via bloodstream to the liver
Multiple inside hepatocytes as merozoites
After a few days the infected hepatocytes rupture releasing merozoites into the blood
Enter erythrocytes and cause subsequent rupture
Causes anaemia and release pyrogens causing rigors and fever
The sequence of entering and rupturing RBCs occurs harmoniously every day causing rigor, fever then severe sweating in constant cycle throughout infection
Infected RBCs with P. falciform adhere to the endothelium of small vessels causing vascular occlusion, severe organ damage to multiple organs
P. ovale and P. vivax remain latent in the liver and this is responsible for relapses that may occur after treatment
Tests will show gametocytes of the mosquito in the blood
When more than 1% RBCs are infected it may cause cerebral malaria or blackwater fever
Malaria presentation
Incubation period 10-14 days in P. vivax, P. ovale and P. falciform infection
18 days to 6 weeks incubation in P. malariae infection
Abrupt onset of rigors, fever (>40 degrees)
Tachycardia
Followed by profuse sweating for some hours later
May show anaemia and hepatosplenomegaly
Nausea and vomiting, dry cough, headache, fatigue, pain
Hypoglycaemia is severe complication of malaria presents similarly to cerebral malaria so must be ruled out
Cerebral malaria: altered consciousness, confusion, convulsions, coma and eventually death.
Blackwater fever: severe haemolysis, haemoglobinuria (dark brown/black urine)
Malaria investigations and management
Investigation:
Blood testing: FBC (anaemia), creatinine and urine output, clotting screen, glucose, BM, ABG/lactate, urinalysis
Microscopy of thick (diagnosis of malaria) and thin (percentage of infected RBCS and species identification) blood smear
Rapid diagnostic test detects parasite antigen
prevention:
ABC:
aware of risks
bite avoidance: repellants, permethrin impregnated clothing, sleeping under impregnated bednets
chemoprophylaxis: mefloquine, doxycycline, chloroquine, macaroni: start drug at least 1 week before return and 4 weeks after without interruption
neurocysticercosis
Most common helminthic disease of CNS
Cysticercosis is most common cause of neurocysticercosis
Caused by taenia solium, pork tapeworm from undercooked pork
Lifecycle:
Consumption of infected undercooked pork leads to intestinal infection (taeniasis) and shedding of T. solium eggs in stool
Invasive disease occurs when the shed eggs are ingested via faeco-oral transmission
Larvae penetrate intestinal wall and cause disseminated disease (cysticercosis) involving the skin, skeletal muscle and brain
Neurocysticercosis is due to larval cyst infecting the CNS causing fits and focal signs
presentation, diagnosis and management of neurocysticercosis
Determined by site and number of lesions (cysticerci) within CNS
Epilepsy 70% cases
FND 20%
motor/sensory loss, language disturbance, involuntary movements
Headache, visual loss, meningitis, hydrocephalus, cognitive impairment
Diagnosis:
CT/MRI (cysts in muscle and brain)
Serology +ve for parasite
Treatment:
Albendazole for non-calcified lesions
Control of seizure
High ICP/hydrocephalus - neurological advice
prosopagnosia, visual apperceptive agnosia, simultagnosia and achromatopsia
Prosopagnosia:
facial blindness
neurological disorder characterised by inability to recognise faces while other aspects of visual processing remain intact
usually associated with the fusiform gyrus within the ventral temporal cortex
visual apperceptive agnosia:
person fails to recognise objects due to functional impairment of occipital-temporal areas of brain
simultagnosia: neurological disorder of inability to perceive more than one object at a time. caused by ventral occipital-temporal lesions (mild form) or dorsal occipital-parietal lesions (more severe form)
achromatopsia:
partial or total absence of colour vision. usually also has increased sensitivity to light and glare (photophobia), nystagmus and reduced sharpness of vision (low visual acuity). can be acquired from cortical damage or can be autosomal recessive congenital colour vision disorder
