Neurology Flashcards
What are the risk factors for developing Alzheimer’s disease?
Age
CVD
Depression
Low educational attainment
- Low social engagement and support
- head trauma, learning difficultie
What is dementia?
- a state characterised by impairment of 2 or more cognitive domains
- persisting for more than 6 months
- severe enough to impact on ability to function
- not primarily attributed to another mental disorder
what is mild cognitive impairment?
- a state characterised by impairment of one or more cognitive domains
- not severe enough to impact on ability to function
- synonymous with ‘prodromal’
(preclinical –> prodromal –> symptomatic)
(symptomatic is then alzheimer’s)
What are the cognitive domains?
- memory: semantic (worldly, no context)/episodic (autobiographical)
- visuospatial: perception/contruction/praxis
- language: naming/fluent/structure/comprehensive
- executive function: planning/ working memory/ inhibition
- social cognition/behavioural: theory of mind/empathy/insight
- attention: speed/multitasking
What genes put you at risk of AD?
APP and presenilin genes (PSEN1, PSEN2)
- APP on chromosome 21, so increased risk for people with down syndrome
alleles of apolipoprotein E4 = not as good at clearing beta plaques
What are the amyloid beta plaques and how do they contribute to AD?
- Senile plaques deposits of beta amyloid aggregate.
- Amyloid precursor protein is found on neurons (helps it to grow and repair after injury).
- If it is broken down normally by alpha secretase and gamma secretase it is soluble and easy to remove
- However if it is broken by beta secretase the leftover fragment isn’t soluble and creates a monomer called beat amyloid.
- These are sticky and can in between neurones and their signalling, They also increase inflammation which damages surrounding neurones
What are neurofibrillary tangles and how do they contribute to AD?
Neurons held together by cytoskeleton = is made up of microtubules a protein ‘tau’ makes sure they stay together.
Beta amyloid build-up outside the neurones initiates pathways which leads to the activation of kinase.
This leads to the phosphorylation of tau
The tau protein changes shape, stops supporting the microtubules, and clumps up with other tau proteins, forming neurofibrillary tangles
Neurones with tangles and non-functioning microtubules can’t signal as well, and sometimes end up undergoing apoptosis.
As neurones die, the brain starts to atrophy.
What are some of the symptoms of AD?
- Agnosia- can’t recognise things
- Apraxia can’t do basic motor skills
- Aphasia speech difficulties
- Cognitive impairment
- Agitation and emotional lability
- Depression and anxiety
- Sleep cycle disturbance:
- Motor disturbance: wandering is a typical feature of dementia
What are the symptoms of episodic memory loss?
Repetitive in conversation
- struggles to follow plot of TV shows
- May forget appointments
- increasingly reliant on partner, lists
- limited knowledge of current affairs
signs
- poor retention of address
- cannot recall breakfast
- cannot recall pictures from naming test
- Ribbot’s phenomena = better remote long term memory than what they did yesterday
What are causes of ‘rapid onset dementias’?
CJD
Vasculitis
Limbic encephalitis
SOl
seizures
infections
metabolic = Wernicke’s
Normal pressure hydrocephalus
usually treatable so want to diagnose quickly
How can the activities of daily life be affected in AD?
Loss of independence: increasing reliance on others for assistance with personal and domestic activities:
Early stages: problems with higher level function (e.g. managing finances, difficulties at work)
Later stages: problems with basic personal care (e.g. washing, eating, toileting) and motor function (e.g. walking, transferring)
What is the diagnostic criteria for AD?
Functional ability: inability to carry out normal functions
Impairment in 2 or more cognitive domains
Differentials excluded
What imaging is used for AD?
CT/MRI:
exclude other diagnosis
help determine type of dementia;
will show medial temporal lobe atrophy
What is the definitive diagnosis for AD
Brain biopsy after autopsy
What are the two cognitive assessments that can be performed to assess AD?
Mini mental state examination (MMSE)
Montreal cognitive assessment scale (MoCA)
Primary care: 6 Cognitive Impairment Test (6CIT)
ACE II
What is assessed in the cognitive assessments?
Attention and concentration
Recent and remote memory
Language
Praxis (planned motor movements)
Executive function
Visuospatial function
What is the treatment for AD?
Non-pharmacological:
programmes to improve/maintain cognitive function
Mild to moderate AD:
acetylcholinesterase inhibitors e.g., Donepezil and rivastigmine
Moderate to severe AD:
N-methyl-d-aspartic acid receptor antagonist memantine (anti-glutamate transmitter)
What is Parkinson’s disease (PD)
A neurodegenerative disorder characterised by the loss of dopaminergic neurons within the substantia nigra pars compacta (SNPC) of the basal ganglia.
What are the risk factors for developing PD?
Age: prevalence is 1% in 60-70 and 3% in those above 80
Gender: men are 1.5 times more likely than females to develop PD
Family history
Describe the pathophysiology of PD
progressive loss of dopamine-producing neurons meaning there is a reduction in the amount of dopamine produced at the substantia nigra
Loss of these neurons results in reduction in action of the direct pathway and a resultant increase in the antagonistic indirect pathway which has a restrictive action on movement.
Therefore bradykinesia and rigidity are key symptoms
There is also formation of protein clumps Lewy bodies
What are the 3 key presentations of PD?
Bradykinesia
Tremor
Rigidity
PD symptoms usually start unilaterally and then become bilateral later in the disease course.
What does the bradykinesia look like in PD?
Handwriting gets smaller
Only take small steps (shuffling gait)
Difficulty initiating movement
Difficulty turning around when standing
Reduced facial movements and expressions
What does the tremor look like in PD?
- A unilateral resting tremor.
- Described as pill rolling tremor looks like they are rolling pill between thumb and finger
- The tremor is worse at resting and when they are distracted like using the other hand
- Frequency of 4-6 times a second
What is the difference between an essential and Parkinson’s tremor?
Essential - active tremor + worsens with movement
Parkinson’s - pill rolling, resting tremor
What is the rigidity like in PD?
if you take their hand and passively flex and extend their arm at the elbow you will feel tension in their arm that gives way to movement in small increments (little jerks)
Described as cogwheel
What are some other symptoms of PD?
Depression
Sleep disturbance and insomnia no REM
Loss of sense of smell
Postural instability
Cognitive impairment and memory problems
What are the differences between a PD tremor and a benign essential tremor?
PD= asymmetrical
BET= symmetrical
PD= frequency= 4-6
BET= 5-8
PD= worse at rest
BET= better at rest
PD= improves with intentional movement
BET= worse
PD= no change with alcohol
BET= better with alcohol
What is used to diagnose PD?
PD is a clinical diagnosis and should be suspected in a patient with bradykinesia and at least one of the following:
- Tremor
- Rigidity
- Postural instability
What is the management for PD?
Motor symptoms NOT affecting quality of life:
A choice of one of the following:
- Dopamine agonist (non-ergot derived)
- Pramipexole, ropinirole - Monoamine oxidase B inhibitor
- Selegiline, rasagiline
- Stop breakdown of circulating dopamine - Levodopa
Motor symptoms affecting the quality of life:
- L.Dopa
Synthetic dopamine levodopa given with a drug that stops it being broken down.
These are peripheral decarboxylase inhibitors:
- Carbidopa and benserazide.
- Co-benyldopa (levodopa and benserazide)
- Co-careldopa (levodopa and carbidopa)
What is the prognosis for PD?
PD is a chronic and progressive condition with no cure.
Overall, life expectancy is reduced with the mortality being 2-5 times higher for those aged 70-89 years old. Also, the risk of dementia is up to 6 times higher in PD patients.
Management: Essential Tremor
The tremor is not harmful and does not require treatment if it is not causing functional or psychological problems.
Medications that may improve symptoms are:
- Propranolol (a non-selective beta blocker)
- Primidone (a barbiturate anti-epileptic medication)
What can cause vascular dementia?
Ischaemic stroke
Small vessel disease
Haemorrhage
Other: cerebral amyloid, which is a cause of small vessel disease.
Deposition of amyloid in small arteries.
CADASIL, which is due to mutation in the NOTCH3 gene and leads to arterial thickening and occlusion.
Describe the pathophysiology of VD?
- Once blood supply to the brain falls below the demands to the tissue, it’ considered an ischaemic stroke.
- The tissue damage is permanent because the dead tissue liquifies in a process called
*liquefactive necrosis** - Brain tissue necrosis leads to a loss of mental function in the area where the loss has occurred
How do the symptoms of VD appear?
They appear suddenly and the brain function decline is STEP-WISE e.g. it decreases with each stroke
Symptoms will vary depending on which vessels are affected in stroke/atherosclerosis
What is motor neurone disease?
Motor neurone disease is an umbrella term that encompasses a variety of specific diagnoses.
They are neurodegenerative diseases that affect both upper and lower motor neurones but sensory neurons are spared
What is the most common type of Motor Neurone Disease?
Amyotrophic lateral sclerosis (ALS) accounts for 50% of cases
What are some other types of MND?
Progressive muscular atrophy: LMN only
Primary lateral sclerosis: UMN only
Progressive bulbar palsy: affects muscles of talking and swallowing (second most common) LMN only
ALS: affects both UMN and LMN
What gene is implicated in some cases of ALS?
SOD1
What are the risk factors for MND?
Increasing age (over 60)
Male
Pesticides
Heavy metals
Rugby
What are the general symptoms of MND?
- Progressive weakness of the muscles throughout the body affecting the limbs, trunk, face and speech.
- The weakness is often first noticed in the upper limbs. There may be increased fatigue when exercising.
- They may complain of clumsiness, dropping things
more often or tripping over. They can develop slurred speech (dysarthria). - struggle to breathe especially at night due to only relying on diaphragm to breathe and it weakens earlier (wake up with headache)
What are the signs of LMN disease?
Muscle wasting
Reduced tone
Reduced reflex
Fasciculations
Loss of power
Babinski reflex negative
What is the cause of LMN injury?
motor neurone - MND< spinal muscular atrophy, polio infection
motor nerve roots - radiculopathy, guillain barre
motor nerves - neuropathies
Neuromuscular junction - myasthenia gravis
Muscles disorder - myositis, myopathies
What are the signs of a UMN lesion?
Increased tone (no UMN to stop actions of LMN)
Brisk reflexes
Rigidity + spasticity
Babinski reflex positive
What is never affected in MND?
Eye muscles (oculomotor movements)
Sensory function and sphincters (onuf’s nucleus)
What are the symptoms of MND?
Progressive weakness
Fatigue - nocturnal respiratory insufficiency (so wake up in night when CO2 builds up)
Falls
Speech and swallow issues
What are the investigations for MND?
MND is a clinical diagnosis
Electromyography: in MND there will be evidence of fibrillation potentials
Nerve conduction studies: may show modest reductions in amplitude
MRI spine: imaging can help exclude spinal pathology which may mimic MND, such as cervical cord compression and myelopathy
What are some differentials for MND?
MS
Polyneuropathies
Myasthenia gravis
GBS
What is the management for MND
No treatment - large amount of neurones have died before symptoms present
- Riluzole = prolongs survival by 2-4 months by protecting motor neuron damage form glutamate
Respiratory support: patients with reduced FVC can use non-invasive ventilation at home, usually BiPAP; prolongs survival by 7 months
Supportive treatment:
Antispasmodics: such as baclofen
What is the main cause of death in motor neurones disease?
death 75-80% within 5 years
- most commonly due to respiratory failure
What is Multiple Sclerosis?
An autoimmune cell-mediated demyelinating disease of the central nervous system
What are some risk factors for developing MS?
Vitamin D deficiency
Family history: HLA-DR2 is implicated; 30% monozygotic twin concordance
EBV infection: the virus with the greatest link to MS
Smoking
Obesity
Describe the pathophysiology of MS
(don’t fully know the cause)
T-cells get through the blood brain barrier and are activated by myelin. The T-cell then changes the BBB to allow more immune cells to get in the brain
MS is a type IV hypersensitivity reaction: T-cells release cytokines and these recruit more immune cells whilst also damaging the oligodendrocytes (+ axones)
B-cells will make antibodies that will destroy the myelin of the oligodendrocytes. leaving behind areas of plaque/sclera
How does MS progress over time?
- In early disease, re-myelination can occur and symptoms can resolve. (thin myelin forms so conduction reduced in heat)
- In the later stages of the disease, re-myelination is incomplete and symptoms gradually become more permanent.
- A characteristic feature of MS is that lesions vary in their location over time, meaning that different nerves are affected and symptoms change over time
MS lesions change location over time is that they are
What are the different types of MS?
Relapsing-remitting:
Secondary progressive
Primary progressive
Progressive relapsing
Clinically isolated syndrome (kind of counts)
What is Relapsing-remitting: MS?
The most common pattern 85% of cases
Episodic flare-ups separated by periods of remission. There isn’t full recovery after flare ups so disability increases over time.
60% will develop secondary within 15 years
What is secondary progressive MS?
Initially, the disease starts with a relapsing-remitting course, but then symptoms get progressively worse with no periods of remission
What is primary progressive MS?
Symptoms get progressively worse from disease onset with no periods of remission
Accounts for 10% of cases and is more common in older patients
What is Progressive relapsing MS?
One constant attack but there are bouts superimposed during which the disability increases even faster
What is clinically isolated syndrome MS?
This describes the first episode of demyelination and neurological signs and symptoms.
- MS cannot be diagnosed on one episode as the lesions have not been “disseminated in time and space”.
- Patients with clinically isolated syndrome may never have another episode or develop MS. If lesions are seen on MRI scan then they are more likely to progress to MS
What are the signs and symptoms of MS?
- Optic nerve affected:
Optic neuritis
Eye movement abnormalities- double vision VI nerve - Spinal cord affected:
- Focal weakness (incontinence, limb paralysis, Bells palsy)
- Focal sensory symptoms ( numbness, pins and needles,
- Lhermitte’s sign is an electric shock sensation that travels down the spine and into the limbs when flexing the neck.) - Cerebellar white matter affected
Ataxia - Cerebral hemispheres:
- large variety but can be silent - Medulla + pons
- dysarthria, double vision, vertigo, nysatgmus - Uhthoff’s phenomenon: worsening of symptoms following a rise in temperature, such as a hot bath
What is optic neuritis?
Demyelination of the optic nerve which present with:
- Unilateral reduced vision
- Central scotoma (enlarged blind spot)
- Pain on eye movement
- Impaired colour vision (red)
What is the primary investigation for MS and what would it show?
MRI of brain and spine:
- Will show demyelinating plaques called Dawson’s fingers
- High signal L2 lesions
- Old lesions will not enhance with contrast, whereas newer lesions will.
This provides evidence of dissemination of lesions in time and space which is required for a diagnosis of MS
What other investigations are done for MS?
Lumbar puncture
- can detect oligoclonal IgG bands in the CSF
Nerve conduction studies
- delayed conduction speeds
Evoked Potentials (VEP)
- measures speed of messages along nerves
What is the diagnostic criteria used to diagnose MS?
McDonald criteria
What is the McDonald criteria based on?
2 or more relapses and either:
- Objective evidence of two or more lesions
- Objective evidence of one and a reasonable history of a previous relapse
‘Objective evidence’ is defined as an abnormality on neurological exam, MRI or visual evoked potentials
What is used to treat a MS relapse?
Oral or IV methylprednisolone
(reduce time to recover from illness but don’t treat illness)
Plasma exchange: to remove disease-causing antibodies
What is used for maintenance of MS?
Disease-modifying drugs
Beta-interferon: decreases the level of inflammatory cytokines
Monoclonal antibodies e.g. alemtuzumab (anti-CD52) and natalizumab (anti-α4𝛃1-integrin)
Glatiramer acetate: immunomodulator drug which acts as a ‘decoy’
Fingolimod: a sphingosine-1-phosphate receptor modulator that keeps lymphocytes in lymph nodes so they can’t cause inflammation
What is used to treat muscle spasticity in MS?
- baclofen
- gabapentin
Why is stem cell transplant used in MS?
- use chemotherapy to kill off all their immune cells
and give them a stem cell transplant - MS is a inflammatory disease so new immune cells should not attack myelin
- doesn’t improve demyelination as damage is already done but stops the disease progressing
What are some complications of MS?
Genitourinary: urinary tract infections, urinary retention and incontinence
Constipation
Depression: offer mental health support if required
Visual impairment
Mobility impairment: offer physiotherapy, orthotics and other mobility aids
Erectile dysfunction
What is Huntington’s disease?
An autosomal dominant genetic neurodegenerative condition that causes a progressive a progressive deterioration in the nervous system.
What causes HD?
- It is a trinucleotide repeat disorder that involves a genetic mutation in the HTT gene on chromosome 4
- There is a repeat of CAG which codes for glutamine 36 times in a row so patients have 36 glutamine in a row on the Huntington protein
- These mutated proteins aggregate within neuronal cells of the caudate and putamen causing neuronal cell death.
- This leads to decreased ACh and GABA synthesis.
- This leads to dopamine increase leading to excessive movement
What is anticipation and how is it linked to HD?
It is a feature of trinucleotide repeat disorders. When copying the HTT gene, DNA polymerase can lose track of which CAG it’s on and so add extra CAGs. This is called repeat expansion.
This leads to successive generations having more repeats in the gene resulting in:
- Early age of onset
- Increased severity of the disease
What are the symptoms of HD?
Patients will be asymptomatic until 30-50.
Symptoms:
- Begin with Cognitive, psychiatric or mood problems
- Chorea (involuntary, abnormal movements)
- Eye movement disorders
- Dysarthria: speech difficulties
- Dysphagia: swallowing difficulties
- Dementia
How is diagnosis of HD made?
Made at a specialist genetic centre that looks for the number of CAG repeats.
This will involve pre and post test counselling
What is the treatment for HD?
No treatment
- supporting person and family
- maintaining quality of life = OT + PT
- speech and language therapy
- genetic counselling
- end of life care planning
What medications are given for symptoms relief in huntington’s?
Medications that can suppress the disordered movement:
Antipsychotics (e.g. olanzapine)
Benzodiazepines (e.g. diazepam)
Dopamine-depleting agents (e.g. tetrabenazine)
What is meningitis?
Meningitis refers to inflammation of the pia and arachnoid mater. Micro-organisms infect the cerebrospinal fluid (CSF).
What are the 3 main features of ‘meningism’?
Stiffness of the neck
Photophobia
Severe headache
What other symptoms would present with bacterial or viral meningitis?
Fever
Feel unwell
Rash (characteristically haemorrhagic in meningococcal meningitis)
What are the viral causes of meningitis?
80% viral
- Enteroviruses (including Echo virus, Coxsackie virus)
- Herpes simplex virus
- Mumps virus
- varicella zoster virus
- Lymphocytic chorio meningitis virus
And historically, Poliovirus (also an enterovirus)
What are the main bacterial causes of meningitis for each age group?
Neonates = E.coli, Group B strep, Listeria monocytogenes
Infants = N.meningitis + H.influenza, Strep pneumoniae
Adults = N.meningitis + S.pneumoniae
Elderly = S. pneumoniae + N.meningitis + listeris monocytogenes
What are the causes of fungal meningitis?
Cryptococcus neoformans
Candida
What are some risk factors for developing meningitis?
Immunocompromised: such as being in the extremes of age, infection (HIV), and medication (Chemotherapy) Listeria monocytogenes
M. Tuberculosis
Non-immunised: at risk ofH. influenza, pneumococcal and meningococcal meningitis
Crowded environments: students living in halls of residence are a commonly affected demographic
What is encephalitis?
Refers to inflammation of the cerebral cortex
What are the main clinical symptoms of encephalitis?
Lethargy + fatigue
Decreased level of consciousness
Fever
Focal neurology
Occasionally there may be fits
What are the viral causes of encephalitis?
Herpes simplex virus
Varicella zoster virus
Parvoviruses
HIV
Mumps virus
Measles virus
Where are the signs of encephalitis?
Pyrexia (fever)
Reduced GCS
Aphasia
Hemiparesis
Cerebellar signs
What are some behavioural changes that occur in encephalitis?
Memory disturbance
Psychotic behaviour
Withdrawal or change in personality
What are some investigations for encephalitis?
Throat swab
HIV serology
MRI of head will show evidence of inflammation will be normal in 1/3 of cases
Lumbar puncture and CSF investigation including a PCR for HSV
What is the treatment for encephalitis?
Aciclovir = should be given in all cases where it is suspected
Ganciclovirmay = be preferred in other herpesvirus infections, such as HHV-6
What CSF cell count results would signify a bacterial cause of infection?
a neutrophil predominant leucocytosis
a raised protein (from dying bacteria)
reduced CSF glucose to serum glucose ratio (metabolically active bacteria, use up glucose as a source of energy)
What are the two tests to look for meningeal infection?
Kernigs Test
Brudzinski’s Test
What is Kernigs Test?
Involves patient lying on their back and flexing one hip and knee to 90 degrees and then slowly straightening the knee whilst keeping the hip flexed. This causes the meninges to stretch
A positive test will be where there is spinal pain or resistance to the movement
What is Brudzinski’s Test?
Involves lying a patient on their back and gently using your hands to lift their head and neck off the bed and flexing their chin to their chest
A positive test is when a patient involuntarily flexes their hips and knees
What is another classic sign of meningococcus meningitis?
non-blanching rash
that everybody worries about as it indicates the infection has caused disseminated intravascular coagulopathy (DIC) and subcutaneous haemorrhages.
Other causes of bacterial meningitis do not cause this rash
What is the empirical antibiotic therapy used against bacterial causes of CNS infection in hospital?
IV cefotaxime or ceftriaxone
What medication should be prescribed alongside antibiotics for meningitis treatment?
IV dexamethasone (corticosteroids)
- reduce risk of long term neurological complications from some causes of meningitis
what medication should be given in primary care for meningitis?
IM benzylpenicillin
then admit the patient ASAP!!
What is the management for meningitis?
- Assess GCS
- blood culture
- broad spectrum antibiotics = Ceftriaxone or cefotaxime
- Steroids = IV dexamethasone
- lumbar puncture = definitive diagnosis
What are the contraindications for a lumbar puncture?
Abnormal clotting (platelets/coagulation)
Petechial rash
Raised intracranial pressure
GCS <9
Where is a lumbar puncture usually taken from?
Between L3/L4
Since spinal cord ends L1/2
When would you do a CT head before a lumbar puncture in meningitis?
Aged > 60
Immunocompromised
History of CNS disease
Seizures <1 week
GCS <14
Focal neurological signs
Papilloedema
Atypical history
What are close contacts of patients with meningitis offered?
Notified
Antibiotic prophylaxis = single dose of ciprofloxacin
What is the CSF count and culture characteristic for a viral meningitis infection?
Predominant lymphocyte response
Only moderately raised protein
CSF glucose more than 50% of the level of serum glucose
What antimicrobial agent is used to treat a viral meningitis or encephalitis infection?
IV aciclovir
How does an infection get into the brain?
- extra-cranial infection e.g. nasopharynx, ear, sinuses
- neurosurgical complications e.g. post op, infected shunts, trauma
- via blood stream e.g. bacteraemic
What is the pathophysiology of meningitis?
- bacteria in blood
- bacteria enter CSF and can be isolated from immune cells due to BBB
- replication
- blood vessels become leaky
- so WBCs can enter the CSF, meninges and brain
- meningeal inflammation +/- brain swelling
What types of meningitis are acute and which are more commonly chronic?
Bacterial and viral meningitis are usually acute
Fungal is more chronic
What will a lumbar puncture show for bacterial meningitis?
high pressure
cloudy
high WBCs
neutrophils
low glucose
high protein
What will a lumbar puncture show for viral meningitis?
normal or elevated pressure
clear
increased lymphocytes
high glucose
low protein
what will a lumbar puncture results show for fungal/TB meningitis?
elevated pressure
cloudy/fibrin web
Increased lymphocytes
low glucose
high protein
What is spinal cord compression SCC?
Results form processes that compress or displace arterial, venous and cerebrospinal fluid spaces as well as the cord itself.
What is the main cause of SCC?
Metastatic cancer lesions
Where are the metastasis most commonly from the cause SCC?
Breast
Lung
Prostate
Thyroid
Kidney
Myeloma
Lymphoma
What area of the spine is most commonly affected by metastasis in SCC?
Thoracic 60%
Lumbar 30%
Cervical 10%
What are some other causes of SCC?
Disc herniation
Disc prolapse
Primary spinal cord tumour
Infection
Haematoma
What are the symptoms of SCC?
Back pain
Progressive weakness of legs with UMN signs
Sensory loss 1-2 cord segments below level of lesion
UMN signs below level of lesion
LWN signs at the level of the lesion
What is a late sign of SCC?
Bladder and anal sphincter involvement: hesitancy, frequency and painless retention
What are the sensory ascending spinal tracts?
- dorsal column medial lemniscus system
- fine touch, vibration and proprioception - spinocerebellar tracts
- posterior = proprioceptive from lower limb
- cuneo = upper limbs
- anterior = lower limb
- rostral = upper ipsilateral - anterolateral systme
- anterior spinothalamic tract = crude touch, pressure
- lateral spinothalamic tract = pain + temperature
What does injury to DCMl pathway result in?
loss of proprioception and fine touch
- ipsilateral loss (if lesion in spinal cord)
- decussation in medulla oblongata
Gracillis = ground lower limbs
Cunneatus = upper limbs
What does injury to anterolateral system result in?
Impairment of:
pain and temperature (lateral)
Crude touch and pressure (anterior)
- contralateral loss (decussate in spinal cord)
What does injury to spinocerebellar tracts cause?
ipsilateral loss of muscle co-ordination (unconscious)
- likely to be injury alongside descending motor tracts
What are the motor descending spinal tracts?
Pyramidal tracts
- originate in cerebral cortex, carrying motor fibres to the spinal cord and brain stem
- voluntary control of musculature
Extrapyramidal tracts
- originate in brain stem, carrying motor fibres to the spinal cord
- responsible for the involuntary and automatic control of all musculature
No synapses = so all upper motor neurones
What is the function of descending motor tracts?
Corticospinal tracts – supplies the musculature of the body.
Corticobulbar tracts – supplies the musculature of the head and neck.
What are the two types of spinal cord injury?
- complete spinal cord injury
- total loss of motor and sensory function below level of injury - Incomplete spinal cord injury
- partial loss of motor and sensory function below level of injury
What would the features of a complete spinal cord compression be?
Loss of all motor and sensory function below the level
What are the types of incomplete spinal cord injury?
- anterior cord syndrome
- central cord syndrome
- Brown-Sequard syndrome
- Mixed syndromes
What would be the features of an anterior spinal cord compression?
Disruption of the anterior spinal cord:
- Loss of motor function below the level
- Loss of pain and temperature sensation
- Preservation of fine touch and proprioception
What would be the features of a posterior spinal cord compression?
Disruption of posterior spinal cord or posterior spinal artery (rare)
- Loss of fine touch and proprioception (posterior column)
- Preservation of pain and temperature sensation (anterior column)
What are the features of brown-sequard syndrome?
Hemi section of spinal cord:
- Ipsilateral paralysis
- Ipsilateral loss of vibration and proprioception
- Contralateral loss of pain and temperature
What is the primary investigation if suspecting a Spinal cord compression?
MRI of spinal cord
Biopsy/surgical exploration
What is the treatment for SCC?
Surgical decompression
- Laminectomy: removal of the lamina/spongy tissue between the discs to relieve pressure
- Microdiscectomy: removal of the herniated tissue from the disc
What is bilateral sciatica a red flag for?
Cauda equina syndrome
What is cauda equina syndrome?
It is a neurosurgical emergency which occurs when the bundle of nerves below the end of the spinal cord are compressed
What are the causes of Cauda Equina?
Lumbar disc herniation: the most common cause of CES
Trauma
Spinal tumour
Lumbar spinal stenosis: narrowing of the spinal cord may result in CES. This can be congenital or acquired e.g. spinal osteoarthritis (spondylosis), rheumatoid arthritis, and a slipped vertebra (spondylolisthesis)
Epidural abscess or haematoma
What are the key presentations of Cauda Equina Syndrome?
Saddle anaesthesia = (loss of sensation in the perineum) can you feel wiping after poo
Loss of sensation in bladder and rectum (not knowing when they’re full)
Urinary retention or incontinence
Faecal incontinence
What are the investigations for CES?
Examinations: PR exam, knee and ankle reflexes
MRI spine is gold standard
Bladder ultrasound: to determine whether there us urinary retention
What is the treatment for CES?
Emergency decompressive laminectomy: surgery should be performed within24-48 hoursof symptom onset.
The incidence of thromboemboli in patients with CES is remarkably high, therefore patients require adequate thromboprophylaxis
What is the main differential of CES?
Conus medullaris
What is Broca’s and Wernicke’s area?
Broca - motor control of speech
Wernicke - understanding of speech
What are mononeuropathies?
Lesions of individual peripheral or cranial nerves
- causes are usually local e.g. trauma or entrapment (tumour)
What are the types of mononeuropathies?
most affect the upper limb
- median nerve e.g. carpal tunnel syndrome
- ulnar = compression at elbow
- axillary neuropathy = shoulder dislocation/relocation
- radial neuropathy = spiral fractures of the radius
lower limb
- L5 radiculopathy = foot drop
Presentation: mononeuropathy
pain/paraesthesia/numbness in the distribution of the named nerve
examination
- majority of patients have no signs
Investigations: mononeuropathy
EMG
nerve conduction studies
What is Myasthenia Gravis?
A chronic autoimmune disorder of the postsynaptic membrane at the neuromuscular junction of skeletal muscle
What are the risk factors for developing Myasthenia Gravis?
Female 2x as common
Autoimmune: linked to rheumatoid and SLE
-Thymoma or thymic hyperplasia: 10-15% have a thymoma and 70% have thymic hyperplasia
What is the main cause of disease in Myasthenia Gravis?
Acetylcholine receptor antibodies present (85%)
These bind to the receptors on the post-synaptic membrane and prevent ACh from binding and causing muscle contraction
The problem is worsened during exercise as more of the receptors become blocked up. So the more the muscles are used the weaker they get. Gets better with rest
These antibodies also activate the complement system which damages cells further making the problem worse
What are the two other antibodies that can cause MG and how do they do it?
- Muscle specific kinase (MuSK)
- low-density lipoprotein receptor-related protein 4 (LRP4).
They are both proteins that are important for making the acetylcholine receptor. These antibodies lead to inadequate acetylcholine receptors causing MG
What are the signs of MG?
Proximal muscle weakness: often affecting the face and neck
Ptosis (drooping eyelid)
Complex ophthalmoplegia: cannot be isolated to one cranial nerve
Head drop: a rare sign due to weakness of cervical extensor muscles
Myasthenic snarl: a ‘snarling’ expression when attempting to smile
What are the symptoms of MG?
Muscle weakness that gets worse throughout the day
Diplopia: double vision
Slurred speech
Fatigue in jaw while chewing
What are the investigations for MG?
Look for presence of autoantibodies:
- Acetylcholine receptor (ACh-R) antibodies (85% of patients)
- Muscle-specific kinase (MuSK) antibodies (10% of patients)
- LRP4 (low-density lipoprotein receptor-related protein 4) antibodies (less than 5%)
CT/MRI of thymus gland to look for thymoma
What is another test that can be performed if unsure about MG?
Edrophonium test:
patients given IV Edrophonium chloride (neostigmine).
Will prevent breakdown of ACh by cholinesterase enzymes and improve symptoms temporarily
What are the treatments for MG?
First-line: Reversible acetylcholinesterase inhibitors (neostigmine/pyridostigmine)
Second-line: Immunosuppressants: prednisolone/azathioprine
Consider monoclonal antibodies (rituximab)
Thymectomy can also improve symptoms.
What is the main complication of MG?
Myasthenic crisis often triggered by another illness such as respiratory tract infection . Causes an acute worsening of symptoms and can lead to respiratory failure
What is the treatment for a Myasthenic crisis?
Patients may require non-invasive ventilation with BiPAP or full intubation and ventilation.
Medical treatment of myasthenic crisis is with immunomodulatory therapies such as IV immunoglobulins and plasma exchange.
What is the main differential for MG?
Lambert-Eaton myasthenic syndrome
What drugs should be avoided in MG?
Antibiotics;
- aminoglycosides (e.g. gentamicin)
- fluoroquinilones (e.g. ciprofloxacin)
- Macrolides (e.g. azithromycin)
- tetracyclines (e.g. doxycycline)
- quinolones
Neuromuscular blocking agents
Magnesium sulfate
penicillamine
Cardiac drugs - BB
neurological - lithium + phenytoin
What is Guillain-Barré syndrome?
An acute paralytic polyneuropathy. It is an autoimmune, rapidly progressive demyelinating condition of the peripheral nervous system
What are the risk factors for developing Guillain-Barré syndrome?
Male
Age 15-35 and 50-75
Malignancies
Vaccines (flu)
Infections
What are the most common infections that trigger Guillain-Barré syndrome?
Campylobacter jejuni (most common)
Cytomegalovirus
EBV
What causes Guillain-Barré syndrome?
A pathogenic antigen resembles myelin gangliosides in the peripheral nervous system.
The immune system targets the antigen and attacks the myelin sheath of sensory and motor neurones
It occurs in patches along the length of the axon so is called segmental demyelination
What antibodies are found in 25% of people with Guillain-Barré syndrome?
Anti-ganglioside antibodies (anti-GMI)
Describe the disease course of Guillain-Barré syndrome?
Symptoms usually start within 4 weeks of the preceding infection. The symptoms typically start in the feet and progresses upward.
Symptoms peak within 2-4 weeks, then there is a recovery period that can last months to years.
What are the signs and symptoms of Guillain-Barré syndrome?
infection 2 weeks prior
Symmetrical ascending weakness (starting at feet and moving up the body)
Reduced reflexes
Loss of sensation and pain
Cranial nerve involvement such as facial nerve weakness
Autonomic features (sweating, raised pulse)
Struggling to breathe
What is used to diagnose Guillain-Barré syndrome?
A clinical diagnosis that is evidenced by progressive weakness and hyporeflexia in the weaker limbs.
The Brighton criteria is used for diagnosis.
What are the differentials of Guillain-Barré syndrome?
Myasthenia gravis
Transverse myelitis
Polymyositis
What is the treatment for Guillain-Barré syndrome?
IV immunoglobulins IG
or Plasma exchange (alternative to IV IG)
Venous thromboembolism prophylaxis (PE is the leading cause of death)
What is the prognosis for Guillain-Barré syndrome?
80% will fully recover
15% will be left with some neurological disability
5% will die
What is Bell’s palsy?
Neurological condition that presents with rapid onset of unilateral facial paralysis
Why is the top half of the face sometimes spared in Bell’s palsy?
The lower half of the faces only has contralateral innervation
Top half has bilateral. forehead sparing
How can you tell between an UMN and LMN Bell’s palsy?
UMN injured: means the contralateral side is weak but the forehead is not
LMN - weakness of all the muscles on the ipsilateral side of the face
Management: Bell’s palsy
Prednisolone within 72 hrs of onset
- can be combined with antiviral e.g. aciclovir
Lubricating eye drops
Define epilepsy?
A neurological disorder in which a person experiences recurring seizures.
(needs to have at least 2 seizures to be diagnosed as epilepsy)
What are the 2 different types of seizure?
Generalised:when both hemispheres are affected always a loss of consciousness
Focal : when the affected area is limited to one half of the brain or sometimes even smaller like a single lobe can progress to bilateral
What are the different subtypes of generalised seizure?
Tonic
Atonic
Clonic
Tonic-clonic
Myoclonic
Absence
What are the two types of focal seizure?
Simple (without impaired awareness)
Complex (with impaired awareness)
What are the general clinical manifestations of seizures for each stage?
Prodromal phase:
Confusion, irritability or mood disturbances
Early-ictal phase:
Aura: warning felt before a seizure. These can include sensory, cognitive, emotional or behaviour changes.
Ictal phase:
Will vary depending on seizure type
Post-ictal phase:
Confused, drowsy and irritable during recovery
What is a tonic, clonic and tonic-clonic seizure?
Tonic seizure: the muscles become stiff and flexed which will cause the patients to fall backwards
Clonic seizures: violent muscle contractions (convulsions)
Tonic-clonic: there is loss of consciousness and tonic (muscle tensing) and clonic (muscle jerking episodes).
Typically the tonic phase comes before the clonic phase. (tongue biting, incontinence, groaning and irregular breathing.
After the seizure there is a prolongedpost-ictal periodwhere the person is confused, drowsy and feels irritable or depressed.
What is the medication for generalised tonic-clonic seizures?
males:Sodium valproate
female:Lamotrigineorlevetiracetam
What is an Atonic seizure?
Known as drop attacks. This is where the muscles suddenly relax and become floppy which can cause the patient to fall usually forward.
They don’t usually last longer than 3 minuets. They typically begin in childhood. They may be indicative ofLennox-Gastaut syndrome.
What is the management for a Tonic and Atonic seizure?
males: sodium valproate
females: Lamotrigine
What is a myoclonic seizure?
They present as sudden brief muscle contractions like a sudden jump. The patient usually remains awake during the episode.
They occur in various forms of epilepsy but typically happen in children as part of juvenile myoclonic epilepsy.
What is the management for a myoclonic seizure?
males: sodium valproate
females: levetiracetam
What is an absence seizure?
Impaired awareness or responsiveness. Patient becomes blank and stares into space before returning to normal. Motor abnormalities are either absent or very minor e.g. eyelid flutters or repetitive lip smacking
Common in children. Most patients (> 90%) stop having absence seizures as they get older.
What is the management for an absence seizure?
First line: ethosuximide
2nd line:
males = sodium valproate
female = lamotrigine or levetiracetam
What is a simple focal seizure (focal aware seizure)?
No loss of consciousness
The patient is aware and awake
Will have uncontrollable muscle jerking
What is a complex focal seizure (focal impaired awareness seizure)?
There is loss of consciousness
Patient is unaware
What is the most common region of the brain affected in a focal seizure?
Temporal lobe
What are the features of a temporal lobe seizure?
They affect hearing, speech, memory and emotions:
- Hallucinations
- Memory flashbacks
- Déjà vu
- Doing strange things on autopilot
Can also include audio symptoms such as buzzing, ringing and vertigo
What are the features of a frontal lobe seizure?
Motor symptoms: pelvic thrusting, bicycling and tonic posturing
Bizarre behaviour
Vocalisations
Sexual automatisms
What are the features of a parietal focal seizure?
Paraesthesia
Visual hallucinations
Visual illusions
More subjective and difficult to diagnose than other areas
What are the features of a Occipital focal seizure?
Visual hallucinations
Transient blindness
Rapid and forced blinking
Movement of head or eyes to the opposite side
What can happen if a focal seizure spreads?
Focal to bilateral tonic-clonic: a focal seizure may spread to affect a wider network of neurons involving both hemispheres.
Traditionally termed a secondary generalised seizure.
What is the medication for focal seizures?
1st line: Lamotrigine or levetiracetam
2nd line: carbamazepine, oxcarbazepine or zonisamide
What is the mechanism of Carbamazepine?
sodium channel blocker; prevents repetitive and sustained firing of action potentials
What are the side effects of Carbamazepine?
- Reduce effect of Oral Contraceptive Pill
(by CYP450 induction > faster metabolism) - leucopenia, thrombocytopenia, hydronatraemia + liver dysfunction
Can you take Carbamazepine when pregnant?
DONT STOP
- risk of harm from seizures is greater than risk of foetal malformations (very low risk)
- immediately start taking folic acid
What is the mechanism of sodium valproate?
increases the activity of GABA, which has a relaxing effect on the brain, teratogenic
What are the investigations for seizures?
- urinalysis or urine cultures, blood cultures (exclude sepsis, encephalitis, or meningitis)
- CT scan
- Blood: blood glucose (rule out hypoglycaemia)
- ECG (to exclude a heart problem)
- EEG (only useful while having a seizure to track electrical patterns in brain, won’t visualise much afterwards)
What are the other treatments for epilepsy?
Vagus nerve stimulation = send electrical stimulations to left vagus nerve to calm down irregular electrical brain activity
Surgery:
Temporal lobe resection MC
Corpus callosotomy = cut callosum to stop epilepsy spreading to other side of brain
Lesionectomy
Hemispherectomy or hemispherotomy
Ketogenic diet
- high fat, low carbs, controlled protein protein diet
- brain use ketone bodies instead of glucose
Which anti-epileptic medication is teratogenic?
sodium valproate
- drugs which cause structural/functional malformations in a developing fetus
What is required for a diagnosis of epilepsy?
Must have had 2 or more seizures more than 24 hours apart
MRI/CT: examine the hippocampus look for underlying cause
EEG: 3H2 wave absence
Bloods: rule out metabolic/infection
What are the different causes of seizures? (pneumonic)
VITAMIN DE
- Vascular
- Infection
- Trauma
- Autoimmune- SLE
- Metabolic
- Idiopathic
- Neoplasms
- Dementia and drugs (cocaine)
- Eclampsia
What is the medical emergency associated with epilepsy?
Status Epilepticus
How do you treat status Status Epilepticus?
ABCDE
- Give IV benzodiazepam e.g. lorazepam 4mg over 5 mins and repeat 5 minuets after if it doesn’t work
- if no stopping after 15 mins give IV sodium valproate or levetiracetam
(If seizure persist then give IV phenobarbital or phenytoin) - Anesthetist = anaesthetise to stop seizures
What are the driving rules for a seizure?
Adults who present with an isolated seizure:
Should stop driving for6 months, providing no cause is found on brain imaging and there is no epileptiform activity on EEG
If the above conditions are not met or the patient has known epilepsy:
Patients must be seizure-free for12 monthsbefore they may qualify for a driving license
If seizure-free for 5 years, a ‘til 70 licence is usually reinstated
What are febrile convulsions?
A type of seizure that occurs in children with a high fever
- children aged 6 months to 5 yrs
What are the 2 types of febrile convulsions?
- simple febrile convulsions = generalised, tonic clonic seizures that last 15 mins and occur only once
- Complex febrile convulsions = consist of partial or focal seizures, last more than 15 mins or occurs multiple times during the same febrile illness
How do you diagnose febrile convulsions?
Child around 18months presenting with a seizure during a high fever
rule out other diagnoses e.g. epilepsy, meningitis, intracranial space occupying lesion, trauma
Management: febrile convulsion
- manage source of infection and control fever with analgesia
- if complex give parents advice on how to deal with seizures
What is the cause of an extra-dural haemorrhage?
Traumatic – typically caused by bleeding from the meningeal arteries as a result of skull fracture that tears the dura
Middle meningeal runs close to the pterion
What is the clinical presentation of extra-dural haemorrhage?
Patient may have extensive traumatic injuries
In other cases, patient sustains a head injury but appears to be OK for a while (‘lucid period’)
Bleeding into the extradural space > rapid rise in intracranial pressure (ICP) > brain is compressed
Headache, drowsiness, rapid neurological deterioration
Death if not treated rapidly (neurosurgical emergency
What is the imaging look like for an Extra-dural haemorrhage?
Acute (fresh) bleed appears hyperdense on CT (bright white)
Convex; does not conform to surface of the brain as bleeding is limited by dural attachments to the skull (lemon shape)
Compression of the brain – midline shift (falx cerebri, lateral ventricles)
Skull fracture may be seen
What is the cause of a sub-dural haemorrhage?
Usually caused by trauma – typically a fall leading to bleeding from dural bridging veins
Low pressure bleeding
Gradual rise in ICP (over several weeks or months)
Most likely in patients with brain atrophy (elderly, dementia, history of excess alcohol intake) – bridging veins
What is the clincal presentation for sub dural haemorrhage?
Typical picture is of gradual cognitive deterioration
May be a history of a fall, maybe not
Patient may have old bruising on their head (or elsewhere) suggesting frequent / recent falls
What is the appearance of imaging for sub dural?
(banana shape)
Chronic (old) bleed appears hypodense on CT (dark)
Concave; conforms to surface of the brain as bleeding is not limited by dural attachments
Compression of the brain – signs include midline shift (falx cerebri, lateral ventricles)
What are the investigations for a SDH?
Immediate CT head
WHat is the cause of a sub-arachnoid haemorrhage?
Usually spontaneous from rupture of an aneurysm on a cerebral artery
Can be traumatic, but this is less common
What is the management for a SDH?
Drainage:
- small SDH are drained via a burr hole washout a small tube called
- large SDH requires a craniotomy which is when part of the skull bone is removed
What are the complications a the raised intercranial pressure in a SDH?
Supratentorial herniation: cerebrum is pushed against the skull or the tentorium, can compress the arteries that nourish the brain leading to an ischaemic stroke
Infratentorial herniation: cerebellum is pushed against the brainstem, can compress the vital area in the brainstem that control consciousness, respiration, and heart rate
What is the clinical presentation for subarachnoid?
Typically present with sudden onset severe ‘worst-ever headache’ (‘thunderclap’)
Patient may:
be conscious
have reduced GCS
be unconscious (poorer prognosis)
- vomiting
- blurred vision
- cranial nerve deficits
Can be rapidly fatal
What is the appearacne of a sub-arachnoid bleed?
Acute (fresh) bleed is hyperdense on CT (bright white)
Blood seen in fissures and cisterns +/- ventricles
(not a large mass like SD and ED)
Blood in the SA space:
irritates the meninges
irritates cerebral vessels and causes vasospasm > hypoxic injury
may track back into the ventricular system > hydrocephalus
(looks like spider)
Investigations: SAH
- CT head non-contrast
- If postive
- CT angiogram / MRI angiogram (can do weeks down the line to check for aneurysms) - If CT negative > Lumbar puncture 12hrs after onset (xanthochromia)
Management: SAH
Initial
1. Surgery = coiling vs clipping
2. nimodipine to reduce vasospasm
3. systolic BP <160
4. Neuro-obs, HDU care
5. manage fluid balance
Complications of SAH
- vasospasm
- rebreeding
- due to increase intra cranial pressure
What is the cause of an intra-cerebral haemorrhage?
- Spontaneous due to aneurysm or vessel rupture
- Small perforating vessels prone to rupture, especially if hypertensive
- Clinical presentation determined by the size of the bleed and brain region affected
E.g.
Coma
Weakness (facial, limbs)
Seizure
What is the appearance of a intra-cerebral bleed on imaging?
Acute (fresh) bleed is hyperdense on CT (bright white)
Blood seen in the substance of the brain
Mass effect seen if large (e.g. midline shift)
What is an epidural haemorrhage?
Bleeding above the dura matter
(same as extra dural haemorrhage)
Who is an EDH most common in?
They usually occur in young adults
What are the symptoms of a EDH?
Reduced GCS: loss of consciousness after the trauma due to concussion
- There might be a lucid interval after initial trauma if there is a slower bleed. This is followed by rapid decline.
Headaches
Vomiting
Confusion
Seizures
Pupil dilation if bleeding continues
What are the differentials for a EDH?
Epilepsy
Carotid dissection
Carbon monoxide poisoning
Subdural haematoma
Subarachnoid haemorrhage
What is the management for a EDH
Clot evacuation
Craniotomy: part of the skull bone is removed in order to remove accumulated blood below.
Followed by ligation of the vessel.
Define: Giant Cell Arteritis
AKA temporal arteritis
Type of systemic vasculitis affecting the medium and large arteries
Presentation: Giant cell arteritis
Unilateral headache
- scalp tenderness
- jaw claudication
- blurred or double vision
- loss of visions if untreated
Associated features of:
- Symptoms of polymyalgia rheumatica (e.g., shoulder and pelvic girdle pain and stiffness)
- Systemic symptoms (e.g., weight loss, fatigue and low-grade fever)
- Muscle tenderness
- Carpel tunnel syndrome
- Peripheral oedema
How to make a diagnosis of giant cell arteritis?
- Clinical presentation
- Raised inflammatory markers, particularly ESR (usually more than 50 mm/hour)
- Temporal artery biopsy (showing multinucleated giant cells)
- Duplex ultrasound (showing the hypoechoic “halo” sign and stenosis of the temporal artery)
Immediate Management: GCA
40-60mg prednisolone daily with no visual symptoms or jaw claudication
500mg-1000mg IV methylprednisolone daily with visual symptoms or jaw claudication
Long term management: GCA
Once the diagnosis is confirmed and the condition is controlled, the steroid dose is slowly weaned over 1-2 years.
Other medications include:
- Aspirin 75mg daily decreases vision loss and strokes
- Proton pump inhibitor (e.g., omeprazole) for gastroprotection while on steroids
- Bisphosphonates and calcium and vitamin D for bone protection while on steroids
What are red flags for headaches in regard to the features of the headache?
Sudden-onset reaching worse severity within 5 minuets (subarachnoid haemorrhage)
New-onset over 50 (GCA/ space occupying lesion)
Progressive/persistent headache or one that has changed dramatically (space-occupying lesion/ subdural haematoma)
What are the causes of spinal fractures?
trauma
- road traffic collisions
- falls from height
- sports injuries
- violence or assault
disease and degenerative changes
- osteoporosis
- cancer
- infections
- arthritis
What are red flag precipitating factors for headaches?
Recent head trauma within 3 months (subdural haematoma)
Headache worse lying down (raised ICP)
Headache worse on standing (CSF leak)
Household contacts with similar symptoms (CO poisoning)
What are red flag associated symptoms of headaches?
Fever, photophobia or neck stiffness (meningitis or encephalitis)
New neurological defect (raised ICP/stroke)
Visual disturbance (GCA/ acute closure glaucoma)
Vomiting (raised ICP, brain abscess and CO poisoning)
What are the risk factors for spinal fracture?
Osteoporosis
Cancer
Trauma, such as a car accident or fall
Age
Tobacco use
Alcohol abuse
Chronic steroid use
Poor nutrition
What is an important investigation to carry out for a headache?
fundoscopy = (look at retina at back of eye) look for papilledema which indicates a raised intracranial pressure + idiopathic intracranial hypertension
Presentation: spinal fracture
- Pain: in the back, neck or limbs
- Paraesthesia: may indicate a spinal cord injury
- Weakness/paralysis: may indicate a spinal cord injury
- Impaired bowel and bladder function = indicate spinal cord injury
What are the features of idiopathic intracranial hypertension?
females
- headache, intermittent, generalised
- worse lying down
- visual obscurations
- usually high BMI
- papillodema
I = normal CT
T = modify risk factors = lose weight first acetazolamide/diuretics/topiramate
Management: spinal fracture
- maintain cervical spine immobilisation
- treat life threatening injuries
- perform ‘log roll’ examination of the back to asses the spine
- neurological examination
- C-spine radiograph and CT neck
- analgesia
- surgical intervention
What are the risk factors for migraines?
Family history
Female gender 3 times more common
Obesity
Triggers
What are some triggers for migraines?
CH- Chocolate
OC- Oral contraceptive
OL- alcohOL
A- anxiety
T- travel
E- exercise
CHOCOLATE
Other triggers can be red wine, bright lights and menstruation
What are the different types of migraine?
Migraine without aura
Migraine with aura
Silent migraine (just the aura without the headache)
Hemiplegic migraine
What are the headache symptoms of a migraine?
Last between 4-72 hours:
- Pounding or throbbing in nature
- Usually unilateral (can be bilateral more common in children)
- Photophobia
- Phonophobia (loud noises)
- Aura
- Nausea and vomiting
What is aura?
Aura is the term used to describe the visual changes associated with migraines symptoms can be:
- Sparks in vision
- Blurring vision
- Line across vision
- Loss of different visual fields
What is a hemiplegic migraine?
They can mimic a stroke. Need to rule out if patient has symptoms:
- typical migraine
- Sudden onset
- Hemiplegia
- Ataxia
- Change in consciousness
What are the 5 stages of a migraine?
- Prodromal stage = subtle symptoms such as yawning, fatigue or mood changes
- Aura
- headache
- Resolution the headache can fade away and be relieved by vomiting or sleeping
- Postdromal stage
What is the diagnostic criteria for migraines with aura?
At least TWO headaches filling criteria:
One or more typical fully reversible aura symptoms including:
- Visual symptoms such as zigzag lines and/or scotoma
visual aura is the most common type of aura.
- Sensory symptoms such as unilateral pins and needles or numbness.
- Speech and/or language symptoms such as dysphasia.
At least three of the following:
- At least one aura symptom spreads gradually over at least 5 minutes.
- Two or more aura symptoms occur in succession.
- Each individual aura symptom lasts 5-60 minutes.
- At least one aura symptom is unilateral.
- At least one aura symptom is positive.
- The aura is accompanied, or followed within 60 minutes, by headache. (can be confused with stroke until headache starts)
What is the diagnostic criteria for migraines without aura?
FIVE attacks fulfilling this criteria
Duration = Headache lasting 4–72 hours in adults or 2–72 hours in adolescents.
Headache with at least TWO of the following characteristics:
- Unilateral location (more commonly bilateral in children).
- Pulsating quality — may be described as ‘throbbing’ or ‘banging’ in young people.
- Moderate or severe pain intensity.
- Aggravation by, or causing avoidance of, routine activities of daily life (for example walking or climbing stairs).
Headache with associated symptoms including at least one of:
- Nausea and/or vomiting.
- Photophobia (sensitivity to light) and phonophobia (sensitivity to sound).
What is the management for migraines?
Analgesia (avoid opioids)
Oral triptan ( 500mg sumatriptan) as the headache starts
What are triptans?
They 5HT (serotonin) receptor agonists and they cause:
- smooth muscle contraction in arteries
- Peripheral pain receptors to inhibit activation of pain receptors
- Reduce neuronal activity in the central nervous system
What are the medications used for migraine prophylaxis?
Propranolol
Topiramate (don’t use in pregnancy as it is teratogenic and can cause cleft lip)
Amitriptyline
What are the non-pharmacological treatments for migraines?
Acupuncture: if both propranolol and topiramate are ineffective or unsuitable
Riboflavin (vitamin B2): **may be effective in some people, but avoid in pregnancy
What is a tension headache?
The most common form of a primary headache
- Can be episodic (<15 days/month) or chronic (>15 days a month for at least 3 months)
What are the causes of tension headaches?
Missed meals
Stress
Overexertion
Lack of sleep
Depression
What are the symptoms of tension headaches?
Bilateral with a pressing/tight sensation of mild-moderate intensity
Nausea or vomiting
Photophobia
Phonophobia
What is the main risk factor for a tension headache?
stress
What are cluster headaches?
Severe unilateral headaches often periorbital that come in clusters of attacks
What is the typical presentation for cluster headaches?
A typical patient with cluster headaches in your exams is a
– 50 year-old male smoker.
- Attacks can be triggered by things like alcohol, strong smells and exercise.
How long can a cluster headache last?
Patient may suffer 3 – 4 attacks a day for weeks or months followed by a pain-free period lasting 1-2 years.
Attacks last between 15 minutes and 3 hours.
What are the symptoms of a cluster headache?
Severe and intolerable pain
unilateral
Red swollen watering eye
Pupil constriction
Eyelid dropping
Nasal discharge
Facial sweating
What is the acute management of cluster headaches?
Triptans (6mg sumatriptan subcut)
High flow oxygen 100%
What are the prophylaxis for cluster headaches?
Verapamil (CCB)
Lithium
Prednisolone (a short course for 2-3 weeks to break the cycle during clusters)
What are the risk factors for Trigeminal Neuralgia?
females
50-60
increases with age
unilateral
MS
What are the causes of Trigeminal Neuralgia?
Normally due to compression of the trigeminal nerve by a vascular loop often the superior cerebellar artery
What are triggers for TGN?
Light touch
Washing
Shaving
Talking
Cold weather
What is the headache like in Trigeminal Neuralgia?
Electric Shock Pain that lasts for seconds to minutes across the face
90% unilateral
10% bilateral
What is the diagnostic criteria for trigeminal neuralgia?
Clinical Dx
3 or more attacks with characteristic unilateral facial pain and Symptoms
MRI - exclude secondary causes/other pathology
What is the treatment for TGN?
- First line: Carbamazepine
- Second line: microvascular decompression or ablative surgery may be considered in refractory patients
What is Horner’s syndrome?
- lesion of the sympathetic chain supplying the eye.
can arise as an isolated injury or as a manifestation of a systemic disease process.
Presentation: Horner’s syndrome
- Miosis (constriction of the pupil),
- Ptosis (drooping of the upper eyelid)
- Anhidrosis (absence of sweating of the face)
on ipsilateral side of face
What is the classification of the location of Horner’s syndrome?
classified according to the site of the lesion
sympathetic chain involved in Horner’s consists of 3 consecutive neurones
- central lesion = Anhidrosis of face, arm and trunk
- preganglionic lesion = Anhidrosis of face
- post ganglionic lesion = No anhidrosis
What are the causes of Horner syndrome based on the location of the lesion?
Central lesions: sentral
S – Stroke
S – Multiple Sclerosis
S – Swelling (tumours)
S – Syringomyelia (cyst in the spinal cord)
Pre-ganglionic lesions: Torso
T – Tumour (Pancoast tumour)
T – Trauma
T – Thyroidectomy
T – Top rib (a cervical rib growing above the first rib and clavicle)
Post-ganglionic lesions: Cervical
C – Carotid aneurysm
C – Carotid artery dissection
C – Cavernous sinus thrombosis
C – Cluster headache
Investigations: Horner’s syndrome
- eye drops
- apraclonidine = reverse pupillary constriction (cause dilation in Horner’s)
- hydroxyamphetamine = will dilate a constricted Horner’s pupil if preganglionic lesions (and not a postganglionic lesion)
What are mononeuropathies?
Lesions of individual peripheral or cranial nerves
- causes are usually local e.g. trauma or entrapment (tumour)
What are the features of a median nerve lesion in the wrist and anterior interosseous?
C6-T1 = nerve of precision grip + LOAF
Wrist = carpal tunnel syndrome, weakness of abductor pollicis brevis; sensory loss over the radial 3 1/2 fingers and palm
Anterior interosseous nerve lesions = weakness of flexion of the distal phalanx of the thumb and index finger
Proximal lesions = may show combined effects
What is the presentation of an ulnar nerve palsy/lesion?
C7-T1
Claw hand
(4th + 5th fingers ‘claw’ up)
- sensory loss over medial 1 1/2 fingers and ulnar side of the hand
- wasting of hypothenar eminence
What is the treatment for a ulnar nerve palsy?
Splint for elbow
simple analgesia
rest + avoid pressure on nerve
What is the presentation of a radial nerve palsy?
C5-T1
Wrist drop
Muscles involved = BEAST
Brachioradialis
Extensors
Abductor pollicis longus
Supinator
Triceps
What is the treatment for a radial nerve palsy?
Splint and simple analgesia
What are the causes of brachial plexus lesion?
- Trauma**
- Radiotherapy
- Prolonged wearing of heavy rucksack
- Cervical rib
- Thoracic outlet compression
- Neuralgic amyotrophy
What are the signs of a brachial plexus lesion?
Pain/ paraesthesia and weakness in the affected arm in a variable distribution
What are the causes of a phrenic nerve lesion?
C3,4,5 keeps the diaphragm alive
- Lung cancer
- TB
- Paraneoplastic syndrome
- Myeloma
- Thymoma
- Cervical spondylosis/ trauma
- Thoracic surgery
- Infections e.g. HZV, HIV, Lyme disease
- Muscular dystrophy
What are the signs of a phrenic nerve lesion?
- Orthopnoea
- Raised hemi-diaphragm on chest x-ray
What nerve causes a ‘foot drop’?
Common peroneal nerve L4-S1
What are the effects of a tibeal nerve lesion?
L4-S3
- inability to stand on tiptoes, invert foot and flex the toes
- sensory loss over the sole
What is mononeuritis mulitplex? And causes?
- A type of peripheral neuropathy where there is damage to several individual nerves due to systemic causes
- Electromyography helps define the anatomic site of lesion
What are the causes of mononeuritis multiplex?
WARDS PLC
- Wegner’s granulomatosis
- AIDS
- Rheumatoid arthritis
- Diabetes
- Sarcoidosis
- Polyarteritis nodosa
- Leprosy
- Carcinoma
What are the signs of cranial nerve 1 lesion?
- Reduced taste and smell
- Ammonia taste remains as it stimulates the pain fibres carried in the trigeminal nerve
What are the causes of a cranial nerve lesion?
- Trauma
- Frontal lobe tumour
- Meningitis
- fracture
- Raised ICP
- Diabetes
- hypertension
What are the signs of cranial nerve 2 lesion?
- Visual field defects:
- Start as small areas of visual loss (scotomas).
- Monocular severe sight impairment: lesions of one eye or optic nerve
- Bilateral severe sight impairment
- Bitemporal hemianopia
- Homonymous hemianopia: loss of the same half (left or right) of the visual field of both eyes, on the opposite side to the lesion (eg, a right side lesion causes loss of the left side of the visual field of both eyes).
- Pupillary abnormalities
- Optic neuritis
- Pain on moving the eye
- Loss of central vision
- Afferent pupillary defect
- Papilloedema
- Optic atrophy
- Pale optic discs
- Reduced acuity
What is diabetic neuropathy?
a complication of diabetes that results in nerve damage that develops gradually and is caused by long term high blood sugar levels
Presentation: diabetic neuropathy
- sensitivity to touch
- loss of sense of touch
- difficulty with coordination when walking
- numbness or pain in your hands or feet
- burning sensation in feet, especially at night
- muscle weakness or wasting
- bloating or fullness
- nausea, indigestion, or vomiting
- diarrhea or constipation
- dizziness when you stand up
- excessive or decreased - sweating
- bladder problems such as incomplete bladder emptying
- vaginal dryness
- erectile dysfunction
What are the different types of diabetic neuropathy?
- peripheral neuropathy
- affects feet and legs = numbness, tingling, muscle weakness - autonomic neuropathy
- digestion problems
- sexual and bladder problems
- cardiovascular problems - proximal neuropathy
- affects hips, buttocks, thighs - focal neuropathy
- damage to one specific nerve, causing weakness in that area
What causes diabetic neuropathy?
high blood sugar levels sustained over a long period of time
- damage to the blood vessels caused by high cholesterol levels
- mechanical injury such as injuries caused by carpal tunnel syndrome
- lifestyle factors such as smoking or alcohol use
Define: radiculopathy
compression of nerve roots as they exit the spinal cord and spinal column, leading to motor and sensory symptoms
- usually cervical or lumbar
Presentation: radiculopathy
- radiating limb pain
- sharp shooting in character
- some have sensory loss
nerve roots usually affected in upper limb = C6 and C7
Nerve root affected in lower limb = L5 and S1
What is the myotome, dermatome and reflex of C6?
Myotome = thumb
Dermatome = biceps
Reflex = biceps jerk
What is the myotome, dermatome and reflex of C7?
myotome = middle finger
dermatome = triceps
reflex = triceps jerk
What is the myotome, dermatome and reflex of L5?
Myotome = dorsal of foot/big toe
Dermatome = dorsiflexion (stand on heels)
Reflex - N/A
What is the myotome, dermatome and reflex of S1?
myotome = ankle, lateral aspect of foot/sole of foot/little toe
dermatome = plantar flexion (stand on toes)
reflex = ankle jerk
What are types of muscular dystrophy?
- Duchennes muscular dystrophy
- Beckers muscular dystrophy
- Myotonic dystrophy
- Facioscapulohumeral muscular dystrophy
- Oculopharyngeal muscular dystrophy
- Limb-girdle muscular dystrophy
- Emery-Dreifuss muscular dystrophy
What is Duchenne’s muscular dystrophy?
A genetic condition that cause gradual weakening and wasting of muscles.
What is the inheritance pattern of Duchenne’s muscular dystrophy?
X-linked recessive
What causes Duchenne’s muscular dystrophy?
caused by a defective gene for dystrophin on the X chromosome.
Dystrophin is a protein that helps hold muscles together at the cellular level
What will a muscle biopsy in Duchenne’s muscular dystrophy show?
Biopsy of the tissue show changes in the muscle itself but not in the nerves which is used to distinguish it between neuropathies
What are the symptoms of Duchenne’s muscular dystrophy?
Symptoms usually present at around 3-5:
- Weakness is muscles around pelvis
- Walking late
- Waddling gait
- Enlarged calves
What is Gower’s sign?
Children with proximal muscle weakness use a specific technique to stand up from a lying position. This is called Gower’s sign.
To stand up, they get onto their hands and knees, then push their hips up and backwards like the “downward dog” yoga pose. They then shift their weight backwards and transfer their hands to their knees. Whilst keeping their legs mostly straight they walk their hands up their legs to get their upper body erect. This is because the muscles around the pelvis are not strong enough to get their upper body erect without the help of their arms.
What are the investigation for DMD?
- High creatinine kinase level
- Genetic testing looking for dystrophin mutations (can be diagnostic)
- Muscle biopsy
- Electromyogram: distinguish between neuropathic and myopathic pathology
What are the treatments for DMD?
- Occupational therapy
- Oral steroids = have been shown to slow the progression of muscle weakness
- Creatine supplementation can give an improvement in muscle strength
What are the complications of DMD?
- Respiratory failure because of a weak diaphragm
- Scoliosis
- Dilated cardiomyopathy and arrhythmias: dystrophin protein is also expressed in heart muscle.
How is Beckers’ muscular dystrophy different to Duchenne’s?
Dystrophin gene is less severely affected and maintains some of its function
- clinical course is less predictable and symptoms only present around 8-12 yrs
What are the features of myotonic dystrophy?
Myotonic dystrophy is a genetic disorder that usually presents in adulthood. Typical features are:
- Progressive muscle weakness
- *Prolonged muscle contractions
- Cataracts
- Cardiac arrhythmias
Define: neurofibromatosis
Genetic condition that causes nerve tumours to develop throughout the nervous system
- benign tumours
- 2 types (type 1 is MC)
What is neurofibromatosis type 1 gene?
Found on chromosome 17
- codes for a protein called neurofibromin = tumour suppressor protein
- autosomal dominant
What is the diagnostic criteria for neurofibromatosis type 1?
CRABBING” mnemonic:
C – Café-au-lait spots (more than 15mm diameter is significant in adults)
R – Relative with NF1
A – Axillary or inguinal freckling
BB – Bony dysplasia, such as Bowing of a long bone or sphenoid wing dysplasia
I – Iris hamartomas (Lisch nodules), which are yellow-brown spots on the iris
N – Neurofibromas = can be on skin as well
G – Glioma of the optic pathway
Management: neurofibromatosis
Diagnosis is based on the diagnostic criteria. Genetic testing can be helpful.
There is no treatment for the underlying disease process. Management involves monitoring, managing symptoms and treating complications.
What are the complications of neurofibromatosis type 1?
Migraines
Epilepsy
Renal artery stenosis, causing hypertension
Learning disability
Behavioural problems (e.g., ADHD)
Scoliosis of the spine
Vision loss (secondary to optic nerve gliomas)
Malignant peripheral nerve sheath tumours
Gastrointestinal stromal tumour (a type of sarcoma)
Brain tumours
Spinal cord tumours with associated neurology (e.g., paraplegia)
Increased risk of cancer (e.g., breast cancer and leukaemia)
What is neurofibromatosis type 2?
- chromosome 22
- codes for merlin = tumour suppressor protein important in Schwann cells
- lead to schwannomas
- autosomal dominant
(associated with acoustic neuromas)
What are the different types of brain tumour?
Gliomas
Meningiomas
Pituitary
Hemangioblastoma
Acoustic neuroma
Accounts for less than 2% of all malignant tumours but 20% of childhood cases
Presentation: brain tumour
- progressive focal neurological symptoms e.g. change in behaviour, personality
- vision change
- raised intracranial pressure
What are the causes of increased intracranial pressure?
Brain tumours
Intracranial haemorrhage
Idiopathic intracranial hypertension
Abscesses or infection
What headache symptoms indicate intracranial hypertension?
Constant headache
Nocturnal (occurring at night)
Worse on waking
Worse on coughing, straining or bending forward
Vomiting
Papilloedema on fundoscopy
What are the features of papilloedema on fundoscopy?
- Blurring of the optic disc margin
- Elevated optic disc (look for the way the retinal vessels flow across the disc to see the elevation)
- Loss of venous pulsation
- Engorged retinal veins
- Haemorrhages around the optic disc
- Paton’s lines, which are creases or folds in the retina around the optic disc
What are gliomas?
Tumours of the glial cells in the brain or spinal cord and surround neurones
- Glial cells include astrocytes, oligodendrocytes and ependymal cells
grade 1 to 4 in severity:
- Astrocytoma (the most common and aggressive form is glioblastoma)
- Oligodendroglioma
- Ependymoma (least malignant)
What are meningiomas?
Tumours growing from the cells of the meninges
- usually benign
- take up space causing raised intracranial pressure
What cancers most often spread to the brain?
Lung
Breast
Renal cell carcinoma
Melanoma
What are acoustic neuromas?
Benign tumours of the Schwann cells that surround the auditory nerve that innervates the inner ear
- unilateral
Presentation: acoustic neuroma
- CN 8: Unilateral sensorineural hearing loss (often the first symptom)
- Unilateral tinnitus
- Dizziness or imbalance
- Sensation of fullness in the ear
-CN 5: absent corneal reflex - CN 7: Facial nerve palsy (if the tumour grows large enough to compress the facial nerve)
Investigations: acoustic neuroma
Refer urgently to ENT
MRI of cerebellopontine angle
Audiometry
Management: acoustic neuroma
Refer urgently to ENT
Conservative: management with monitoring may be used if there are no symptoms or treatment is inappropriate
Surgery: remove the tumour (partial or total removal)
Radiotherapy: to reduce the growth
Why do brain abscesses form?
when an area of cerebral inflammation becomes necrotic and encapsulated by glial cells and fibroblasts
- oedema around abscess may cause raised intracranial pressure
What are the causes of brain abscess?
- direct extension of cranial infections
- head wounds
- hematogenous spread e.g in bacterial endocarditis
- unknown causes
Presentation: brain abscess
- headache
- nausea
- vomiting
- lethargy
- seizures
- personality changes
- papilledema
- fever, chills and leukocytosis may develop before infection is encapsulated
Investigations: brain abscess
MRI
contrast enhanced CT
Management: brain abscess
- antibiotics = ceftriaxone or cefotaxime plus metronidazole
- CT guided aspiration or surgical drainage
- corticosteroids, anti seizure drugs
Define: narcolepsy
rare condition in which the brain loses its normal ability to regulate the sleep-wake cycle
2 types
What is the cause of narcolepsy?
Orexin (hypocretin) is a neurotransmitter involved in the regulation of sleep, wakefulness and appetite
Loss of orexin secreting neurons in the hypothalamus result in narcolepsy
Due to to autoimmunity
Presentation: narcolepsy
- Excessive daytime sleepiness (EDS): exclude other causes.
- Disrupted nighttime sleep and/or vivid dreams.
- Cataplexy: “conscious collapse” caused by muscle atonia, often in response to sudden emotion such as laughter or surprise; rarely seen by the clinician so diagnosis often based on the characteristic description.
- Hypnagogic/hypnopompic hallucinations: dream-like hallucinations at the point of emerging from/entering REM sleep.
- Sleep paralysis: paralysis while awake and conscious, again at the transition between REM sleep and wakefulness.
Investigations: narcolepsy
Polysomnography
Multiple sleep latency testing
(sleep latency = amount of time it takes to fall asleep)
CSF orexin levels
Management: narcolepsy
- good sleep hygiene
- scheduled naps
- CNS stimulants e.g. methylphenidate
- antidepressants (for cataplexy) e.g. clomipramine, SSRI
- sodium oxybate = potent sedative to improve nocturnal sleep quality
Define: bulbar palsy
Refers to a set of signs and symptoms linked to the impaired function of the lower cranial nerves (4-7)
What are the causes of bulbar palsy?
- brainstem strokes and tumours
- degenerative disease = ALS
- autoimmune diseases = Guillain-barre syndrome
- genetic diseases
Presentation: bulbar palsy
- dysphagia
- reduced/absent gag reflex
- slurred speech
- aspiration of secretions
- dysphonia
- dysarthria
- drooling
- difficulty chewing
- nasal regurgitation
Management: bulbar palsy
- no known treatment
- symptom management: medications (drooling), feeding tube, speech and language therapy
- condition specific treatments
What is ataxia?
problems with balance and coodination
- heterogenous group of disorders
- many different causes
- majority permanent and progressive
What are the symptoms of cerebellar dysfunction?
- dizzy
- falls, stumble
- difficulty focusing/double vision/’oscillopsia’ (
- slurred speech
- problems with swallowing
- tremor
- problems with dexterity/ fine motor skills
What are the clinical signs of cerebellar dysfunction?
DANISH
Dysdiadochokinesis
Ataxia
Nystagmus
Intention tremor
Scanning speech, disarthria
Hypotonia, hyporeflexia
What are the investigations for ataxia?
Blood = FBC, LFTs, ESR, CRP, Gluten, B12, HbA1c, folate
HLA typing for DQ2/DQ8
MRI brain = demonstrate cerebellar atrophy
CT if MRI contraindicated
What is the management of cerebellar ataxia?
no cure so tailored to the individual patient
- specialist services
Define: cerebral palsy
A non-progressive, permanent neurological condition commonly affecting normal movement and posture
What is the cause of cerebral palsy?
Damage to the developing brain which can occur while the baby is in utero, during birth or in the neonatal period
- caused by hypoxia, haemorrhage or infection
What are the risk factors for cerebral palsy?
antenatal RF
- multiple gestation
- chorioamnionitis
- maternal TORCH infections
Perinatal RF
- prematurity
- low birth weight
- birth asphyxia
- neonatal sepsis
postnatal RF
- meningitis
- severe hyperbilirubinaemia (jaundice)
Presentation: cerebral palsy
delayed motor milestones
not sitting by 8 months
not walking by 18 months
hand preference before 12 months
- tone abnormalities (floppies or stiffness)
- abnormal movement
- feeding problems
- persistent toe walking
No regression in milestones
What are the types of cerebral palsy?
- spastic cerebral palsy = velocity dependent hypertonia (spasticity) and hyperreflexia
- Dyskinetic cerebral palsy = involuntary, uncontrolled, recurring movements, fluctuating muscle tone and persistent primitive reflexes
- Ataxic cerebral palsy = loss of muscular coordination resulting in ataxia and tremor
Investigations: cerebral palsy
clinical diagnosis
- MRI scan
Management: cerebral palsy
conservative
- physiotherapy
- occupational therapy
- speech and language therapy
- dietician input
medical
- Hyoscine hydrobromide or glycopyrronium bromide: excess drooling
- Diazepam: pain
- Baclofen: hypertonia
- Botulinum toxin type A injections: used if spasticity is severely affecting function or causing significant pain
Surgical
- hip replacement
What are the complications of cerebral palsy?
Problems with feeding and aspiration
Drooling
Constipation
Visual and hearing impairment
Epilepsy
Learning disability
Speech difficulty
Osteopenia and osteoporosis (especially if non-mobile)
Sleep disturbance
define: hypoxic-ischaemic encephalopathy (HIE)
Occurs in neonates as a result of hypoxia during birth
hypoxia = lack of oxygen
Ischaemia = restriction in blood flow
encephalopathy = malfunctioning of the brain
HI can lead to permanent damage = cerebral palsy
What are the causes of HIE?
- Maternal shock
- Intrapartum haemorrhage
- Prolapsed cord, causing compression of the cord during birth
- Nuchal cord, where the cord is wrapped around the neck of the baby
What are the grades of HIE?
Sarnat staging
Mild
- poor feeding, generally irritability and hyper-alert
- resolves within 24 hrs
- normal prognosis
Moderate
- poor feeding, lethargic, hypotonic and seizures
- can take weeks to resolve
- up to 40% develop cerebral palsy
Severe
- reduced consciousness, apnoeas, flaccid, and reduced or absent reflexes
- up to 50% mortality
- up to 90% develop cerebral palsy
Management: HIE
Supportive neonatal care
- neonatal resuscitation
- optimal ventilation
- circulatory support
- nutrition
- acid base balance
- treatment of seizures
- therapeutic hypothermia
What is therapeutic hypothermia?
Babies near or at term considered to have HIE
- actively cooling the core temperature of the baby
- baby is transferred to a neonatal ICU and actively cooled using cooling blankets and a cooling hat
(temp 33-34)
- continued for 72 hrs and baby is gradually warmed over 6 hrs
What is the purpose of therapeutic hypothermia?
- reduce inflammation and neurone loss after the acute hypoxic injury
Define: meniere’s disease
Long term inner ear disorder that causes recurrent attacks of vertigo
- due to excessive buildup of endolymph in the labyrinth of the inner ear
- causes a higher pressure than normal and disrupting sensory signals
Presentation: Meniere’s disease
- 40-50 years old
- unilateral
- vertigo episodes (last 20 mins to few hrs)
- hearing loss (fluctuates at first and them becomes permanent, affects low frequencies first)
- tinnitus (again temporary episodes then become permanent)
- sensation of fullness in the ear
- unexplained falls without loss of consciousness
- imbalance
- spontaneous nystagmus during an acute attack (unidirectional)
Investigations: meniere’s disease
clinical based on signs and symptoms
- ENT specialist
- audiology assessment
Management: meniere’s disease
Management involves:
- Managing symptoms during an acute attack
- Prophylactic medication to reduce the frequency of attacks
For acute attacks, short-term options for managing symptoms include:
- Prochlorperazine
- Antihistamines (e.g., cyclizine, cinnarizine and promethazine)
Prophylaxis is with:
- Betahistine
Define: chronic fatigue syndrome
AKA Myalgic Encephalomyelitis
- chronic illness that causes extreme fatigue that cannot be explained by any other medical condition
Presentation: Chronic fatigue syndrome
- post exertion malaise = symptoms get worse after exercise or mental effort and don’t improve after sleep or rest (can last 24hrs or a few days)
- start suddenly or gradually over months/years
- persistent and profound fatigue
- thinking problems e.g. memory loss, poor concentration
- sleeping difficulties
- headaches
- muscle pain, joint pain
- GI changes e.g. constipation, diarrhoea
- sensitivities to light or noise
- problems with temp regulation
What causes chronic fatigue syndrome?
- infection e.g. COVID
- environmental toxins
- physical trauma e.g. surgery
- genetics
- physical or emotional stress
How to diagnose chronic fatigue syndrome?
- no test
- rule out other organic causes
- must have symptoms for 6 months +
Management: chronic fatigue syndrome
No cure
1. Pacing and rest = breaks down your activity into short bursts interspersed with rest so you don’t trigger symptoms
- Stepwise symptom management = ranking symptoms and starting with most problematic first
- go to bed at same time each night
- avoid caffeine, alcohol, nicotine
- make time to rest
- keep dairy to notice your triggers
Define: normal pressure hydrocephalus
Abnormal buildup of CSF within the brain and spinal cord
- it is either over production of CSF or a problem with draining or absorbing CSF
What are the causes of hydrocephalus?
- Aqueductal stenosis = MC leading to insufficient drainage of CSF (in cerebral aqueduct between 3rd and 4th ventricle)
- arachnoid cysts
- Arnold-chiari malformation = cerebellum herniates downwards through the foramen magnum clocking outflow of CSF
- chromosomal abnormalities and congenital malformations
Presentation: hydrocephalus
cranial bones in babies are not fused so babies will have an enlarged and rapidly increasing head circumference
- bulging anterior fontanelle
- poor feeding and vomiting
- poor tone
- sleepiness
Management: hydrocephalus
Ventriculoperitoneal shunt
- drains CSF from the ventricles into another body cavity (often peritoneal cavity)
What are the complications of a ventriculoperitoneal shunt?
- Infection
- Blockage
- Excessive drainage
- Intraventricular haemorrhage during shunt related surgery
- Outgrowing them (they typically need replacing around every 2 years as the child grows)
Define: herpes zoster virus
aka Shingles
Acute unilateral, self limiting inflammatory disease of cerebral ganglia, posterior nerve roots and peripheral nerves in a segmented distribution, caused by varicella zoster virus (chickenpox)
Presentation: herpes zoster
- rash in single dermatome distribution
- pruritus
- dyesthesia
How is herpes zoster caused?
Caused by reactivation of the varicella zoster virus within the dorsal root or cranial nerve ganglia
- chickenpox virus migrates from skin lesions to spinal and cranial sensory ganglia where it becomes dormant
- reactivation occurs when cell mediated immunity wanes
- once reactivated the virus spreads through the affected sensory nerve, causing neuronal damage and reaches the dermatome in the skin where a vesicular rash develops
Management: shingles
antiviral drug commenced within 72hrs of rash onset
- treat if >50 years (post herpatic neuralgia rare if <50 years) and within 72 hours of rash or if 1 of the following:
- active ophthalmic;
- Ramsey Hunt;
- eczema;
- non-truncal involvement;
- moderate or severe pain;
- moderate or severe rash - shingles treatment if not within 72 hours:
consider starting antiviral drug up to 1 week after rash onset if:
- high risk of severe shingles or continued vesicle formation;
- older age;
- immunocompromised;
- or severe pain
medication used
- acyclovir (IV for immunocompromised)
- famciclovir
- valaciclovir
What are the complications of herpes zoster?
- post herpetic neuralgia = pai persisiting 3 or more months after resolution of rash
- temporal paralysis = follows dermatome involvement and may affect bladder and bowel function
- disseminated HZ (mainly in immunocompromised patients)
- neurologic complications of HZ e.g. encephalitis, myelitis, meningitis, retinitis
Glasgow Coma Scale
Eyes
1 to 4
(remember glasses = 4 eyes)
None = 1
Open to pain = 2
open to voice = 3
spontaneous opening = 4
Voice
(Roman numeral V = 5)
1 to 5
None
sounds = 2
words = 3
Confused = 4
orientated = 5
Motor
1 to 6
none
abnormal extension = 2
(decerebrate posturing = head, arms and legs extend and internally rotate, rigid = brain stem damage )
abnormal flexion = 3
(arm flexes in and draws in one chest = decorticate posturing)
withdraws to pain = 4 (flexes arm away > cerebral hemisphere damage)
localises pain = 5 (hand brought above clavicle)
obeys commands = 6
lowest = 3
< 8 = coma
Investigations: coma
- depends on suspected cause
- basic blood including glucose
- ABG
- imaging = CT head, MRI head
- lumbar puncture
- EEG
- toxicology and alcohol levels
What are the features of brown sequard syndrome?
lateral hemisection of spinal cord
- Same sided weakness and proprioception/vibration loss
- Loss of pain/temp on opposite side
Define: myelopathy
syndrome for pathology involving the spinal cord
- MC cause = disc-osteophyte cord compression
Presentation: myelopathy
- loss of fine finger movements
- difficulty walking, legs feel ‘not my own’
signs:
long tract signs/upper motor neurone signs
What are the upper motor neuron signs?
- Spastic gait
- Hypertonia
- Hyper-reflexia
- Babinski
- Clonus
- Cross-adductors
- Hoffman’s sign
- Loss of fine finger movements (‘treacle hands’)
- Deltopectoral reflex
define: Venous sinus thrombosis
severe sudden onset headache
Define: normal pressure hydrocephalus
Reversible cause of dementia seen in elderly patients
- secondary to reduced CSF absorption at the arachnoid villi
- changes may be secondary to head injury, SAH or meningitis
What is the classical triad presentation in normal pressure hydrocephalus?
- urinary incontinence
- dementia and bradyphrenia
- gait abnormality (may be similar to Parkinson’s disease)
Investigations: normal pressure hydrocephalus
CT head
hydrocephalus with ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement
Management: normal pressure hydrocephalus
- ventriculoperitoneal shunting
around 10% of patients who have shunts experience significant complications such as seizures, infection and intracerebral haemorrhages
Presentation: B12 deficiency
extreme tiredness
a lack of energy
pins and needles (paraesthesia)
a sore and red tongue
mouth ulcers
muscle weakness
disturbed vision
psychological problems, which may include depression and confusion
problems with memory, understanding and judgement
What are causes of B12 or folate deficiency?
- pernicious anaemia = immune cells attack stomach preventing body from absorbing
- lack of vitamins in diet
- medication
Define: subacute combined degeneration of the cord
vitamin B12 deficiency
resulting in impairment of the dorsal columns, lateral corticospinal tracts and spinocerebellar tracts
Presentation: subacute combined degeneration of the spinal cord
- dorsal column involvement
- distal tingling/burning/sensory loss is - symmetrical = legs >arms
- impaired proprioception and vibration sense - lateral corticospinal tract involvement
- muscle weakness,
- hyperreflexia
- spasticity
- upper motor neuron signs
- typically develop in the legs first
- brisk knee reflexes
- absent ankle jerks
- extensor plantars - spinocerebellar tract involvement
- sensory ataxia → gait abnormalities
- positive Romberg’s sign
What causes foot drop?
L5 nerve lesion
Common perineal nerve lesion
Treatment ladder: neuropathic pain
- Amitryptylline
- duloxetine
- gabapentin
- pregabalin - Tramadol
‘rescue drug’ - topical capsaicin = localised neuropathic pain
What is the difference in presentation between CN3 nerve palsy and Horner’s syndrome?
CN 3
- ptosis and dilated pupil (mydriasis)
Horner’s
- ptosis + constricted pupil (miosis)
Define: wernicke’s encephalopathy
Thiamine deficiency
- seen MC in alcoholics
What is the triad for wernicke’s?
- ophthalmoplegia/nystagmus
- ataxia
- confusion
What are some other symptoms of wernicke’s?
CAN OPEN
Confusion
Ataxia
Nystagmus
Ophthamoplegia
PEripheral
Neuropathy
Presentation: Korsakoff syndrome
- anterograde amnesia
-retrograde amnesia - confabulation
