Neurology Flashcards
Investigations for suspected GBS
Lumbar puncture (cytoalbuminologic dissociation- raised protein without raised WCC) and nerve conduction studies (decreased motor nerve conduction)
Also check (for any limb weakness)- FBC, UE, LFT, CRP, ESR, bone profile (calcium), phosphate, magnesium, B12, folate (haemanitics)
Myasthenia gravis
Autoimmune disorder, resulting in antibodies to acetylcholine receptors
Myasthenia gravis features
Muscle fatigability- muscles become weaker during periods of activity and improve after rest
Diplopia and ptosis (eg, at the end of the day after watching TV and reading)
Weakness of face, neck, limb girdle muscles
Dysphagia and slowness in chewing food
NO SENSORY PROBLEMS
NB- if concurrent thymoma, may see SVC syndrome (distended neck veins)
Myasthenia gravis associations
Thymoma
Autoimmune disorders eg. SLE
Thymic hyperplasia (most common)
Investigations for MG
All the blood tests you would do for limb weakness
Electromyography- diminished response to repetitive stimulation on EMG
Acetylcholine receptor auto antibodies (or anti muscle specific tyrosine kinase antibodies)
CT thorax to exclude thymoma
Creative kinase is normal
Long term management of MG
Pyridostigmine
Prednisolone
Immunosuppressants eg. Azathioprine
NB- thymectomy in hyperplasia but not thymoma
Management of acute MG crisis
Plasmapheresis
IV immunoglobulins
NB- resp failure can develop and kill these patients. Be prepared to step up management early
Drugs that can exacerbate pre existing MG
Beta blockers
Lithium
Phenytoin
Antibiotics eg. Gentamicin, macrolide, tetracyclines
Penicilliamine
Quinidine
Myelopathy
Disorders due to compression of the spinal cord
Myelitis
Neurological disorder due to inflammation of the spinal cord
Causes/risk factors for myelopathy
Trauma
Infection (abscess)
Neoplasm
Degenerative disease eg. Spinal stenosis, ankylosing spondylitis, disc prolapse
High axial loading job and smoking (disc prolapse risk factors)
Features of myelopathy
Limbs affects (upper or lower) depends on the site of the lesion eg. Cervical or thoracic region. Usually bilateral
Pain
UMN signs (increased tone, spasticity, hyperreflexia, up going plantar reflex, weakness)
Loss of motor function
Impaired sensation eg. Numbness, ataxia, loss of proprioception or vibration sense
Impaired bladder and bowel control (Impotence too)
Features of myelitis
Same as myelopathy
Lumbar region and myelopathy
No spinal cord in lumbar region, just nerve roots, so won’t get UMN lesions when a lumbar disc herniates (but can get LMN signs eg. Cauda equine, get autonomic (bladder and bowel), sensory (parathesia), and LMN signs (absent reflexes)
Radiculopathy
Neurological disease due to compression of nerve roots
Features of cauda equine syndrome
Back pain, radicular pain
Neurological deficits below the lesion eg. Sensory abnormalities first, then motor abnormalities
Bladder, bowel, sexual dysfunction
Saddle parasthesia
General features of myosotis (PM or DM)
Bilateral proximal muscle weakness (pelvis and shoulder girdle)
Functional difficulties eg. Dressing, climbing stairs
Dysphagia
Muscle tenderness
Cutaneous features of dermatomyositis
Gottrons papules (rough red papules over the extensor surfaces of the fingers)
Mechanics hands- thickening and hyperpigmentation
Heliotrope rash (upper eyelids)
Midfacial erythema (affects nasolabial folds unlike SLE)
Photosensitive rash
Inclusion body myosotis features
Progresses slowly over many years
Same symptoms as general myosotis but affects proximal and distal muscle groups
Investigations for suspected myosotis
Neurological examination and observations
FBC UE other electrolytes
CK- raised
Antibodies
Muscle biopsy
MRI- malignancy
Difference between PM, DM, and IBM
PM and DM are associated with younger people and malignancy, they can present quickly, affect the proximal muscles
IBM progresses slowly over many years and is associated with the elderly, affects the proximal and distal muscles
Antibodies for myosotis
PM- anti jo 1
DM- anti Mi 2 & ANA & anti synthase & anti jo 1
Management of myosotis
Refer to rheumatology
Supportive measures NSAIDS etc
Treat underlying malignancy
Steroids
Immunoglobulins
Biologics
Differentiate an acute peripheral neuropathy and an acute myelopathy (LMN vs UMN)
Tone and reflexes
Facial involvement
Facial nerve palsy features
Changes to muscles of facial expression eg. Inability to smile etc.
Hyperacusis or hearing changes
Reduced taste sensation
Reduced tears and saliva (dry mouth and eyes)
Causes of unilateral facial palsy
Bell’s palsy (pregnancy is a risk factor for Bell’s)
Ramsay hunt syndrome
Acoustic neuroma/ cholesteatoma
Parotid tumours
HIV
DM
Malignant otitis externa
Facial trauma
Stroke, MS, tumour (UMN lesion- forehead is spared)
Causes of a bilateral facial nerve palsy
Sarcoidosis
GBS
Lyme disease
Bilateral acoustic neuromas
Bell’s palsy
MND (UMN or LMN)
NB- these can cause unilateral palsies too
When to give steroids in Bell’s palsy
Within 72 hours of onset
NB- if it hasn’t resolved within 3 weeks- refer to ENT
What is shingles
Herpes zoster infection, caused by reactivation of the varicella zoster virus (chicken pox)- it had remained dormant in the dorsal root of cranial nerve ganglia for many years before reactivation
Features of shingles (herpes zoster infection)
Prodrome period- burning pain over the dermatome (severe pain), fever, headache, lethargy
Rash- vesicular, macular rash over the affected dermatome, well demarcated and doesn’t cross the midline
NB- diagnosis is clinical
Management of shingles (herpes zoster infection)
Patients are infectious- need to avoid pregnant women and the immunocompromised (wait until vesicles have crusted over- 7 days after onset), cover lesions to reduce risk of spread
Analgesia (paracetamol and NSAIDs, then amitriptyline)
Antivirals within 72 hours eg. Aciclovir (reduces incidence of post heretic neuralgia)
Complications of shingles (herpes zoster infection)
Post herpetic neuralgia (resolves in 6 months)
Herpes zoster ophthalmicus
Herpes zoster oticus/Ramsay hunt syndrome- ear lesions and facial paralysis
Brocas (expressive) dysphasia
Non fluent speech (content preserved but delivered in a very laboured/halting way)
Repetition is impaired
Comprehension in tact
Inferior frontal gyrus
Global aphasia
Non fluent speech
Comprehension impaired (receptive and expressive dysphasia)
Wernickes aphasia (receptive aphasia)
Fluent speech, but sentences make no sense, there is word substitution and neologisms
Comprehension impaired
Superior temporal gyrus
Conduction aphasia
Fluent speech
Compression intact
Inability to repeat words and phrases, aware of mistakes they make
Arcuate fasiculus - the connection between Wernicke’s and Broca’s area
Features of an Arnold chiari malformation
Headache
Non communicating hydrocephalus
Syringomelia (CSF in the spinal cord)
What is autonomic dysreflexia
Clinical syndrome that occurs in people who have a spinal cord injury at or above T6
Triggered by faecal impaction or urinary retention
Features of autonomic dysreflexia
Extreme HTN, flushing and sweating at the level above the lesion, severe cases haemorrhagic stroke
Management of autonomic dysreflexia
Control precipitating stimulus
Treat life threatening HTN and or bradycardia
Causes of a brain abscess
Extension of sepsis from middle ear or sinus, trauma, surgery, penetrating head injuries, embolic events from endocarditis
Features of a brain abscess
Headache, fever, focal neurology, other features of raised ICP eg. Nausea, papilloedema, seizures
Antibiotic choice for a brain abscess
IV 3rd generation cephalosporin and metronidazole
What is the most common brain tumour
Metastatic brain cancer
Lung (most common) breast bowel skin kidney
Where do meningiomas arise from
Dura mater
Vestibular schwannoma
Previously called acoustic neuroma
Benign tumour arising from the 8th cranial nerve
Unilateral hearing loss, facial nerve palsy, tinnitus, vertigo, absent corneal reflex
Neurofibromatosis type 2- bilateral vestibular schwannomas
MRI cerebellopontine angle (galladium enhanced)
Brown sequard syndrome
Caused by LATERAL hemi section of the spinal cord
Ipsilateral weakness below the lesion
Ipsilateral loss of proprioception and vibration sensation
Contralateral loss of pain and temperature sensation
NB- think pain and temp are the worst so you would want them on the other side
Cerebellar syndrome causes
Unilateral lesions cause Ipsilateral signs
P - Posterior fossa tumour
A - Alcohol
S - Multiple sclerosis
T - Trauma
R - Rare causes
I - Inherited (e.g. Friedreich’s ataxia)
E - Epilepsy treatments eg. phenytoin
S - Stroke
NB- vermis= ataxia, hemisphere= finger to nose
Features of cerebellar syndrome
DANISH
Dysdiadochokinesia, dysmetria (past pointing)
Ataxia
Nystagmus
Intention tremor
Slurred staccato speech, scanning dysarthria
Hypotonia
Features of a common peroneal nerve injury
Foot drop
Weakness of foot dorsiflexion
Weakness of foot eversion
Features of Creutzfeldt-Jakob disease
Rapid onset dementia
Myoclonus
NB- comes from BSE (mad cow disease)
Drugs that can cause peripheral neuropathy
Amiodarone
Metronidazole
Nitrofurantoin
Isoniazid
Vincristine
Syncope and DVLA
Simple faint- no restriction
Single episode, explained and treated- 4 weeks
Single episode, unexplained- 6 months
2 or more- 12 months
Traumatic brain injury and the DVLA
6 months stop
Stroke or TIA and the DVLA
1 month (if no residual defecits, don’t need to tell DVLA)
Multiple TIAs over short time
3 months off and tell DVLA
Craniotomy and the DVLA
1 year off if meningioma, 6 months off if pituitary tumour
Trans sphenoid all surgery and the DVLA
Can drive when no residual symptoms
Narcolepsy and the DVLA
Cease driving on diagnosis, restart when control of symptoms
Chronic neurological disorders and the DVLA
Tell DVLA, carry on driving, they may assess you regularly
Investigations for encephalitis
Lumbar puncture (lymphocytosis, elevated protein) with MCS
PCR for HSV (from CSF)
MRI head
NB- LP gold standard (MRI only shows temporal changes in HSV associated disease)
Management of encephalitis
IV aciclovir
Extradural (epidural) haematoma
Middle meningeal artery
Lucid interval
Biconvex/lentiform collection (limited to suture lines)
Causes of foot drop
Common peroneal nerve lesion
Sciatic nerve lesion
UMN lesion eg. Stroke
NB- avoid kneeling, crossing legs, should heal in several months
Raised ICP headache
Triggered by cough, vaslsalva manoeuvres, sneeze, exercise, worse in morning
Visual disturbance and focal neurology may be present
Huntingtons disease
Autosomal dominant
Trinucleotide repeat of CAG
Features of HD
Chorea
Personality change
Intellectual impairment
Dystonia
Saccades
NB- management- SALT, end-of-life planning, genetic counselling, benzo’s/ AP’s (reduce movements)
Idiopathic intracranial HTN features
Headache, blurred vision, papilloedema, enlarged blind spot, sixth nerve palsy, pulsatile tinnitus
Risks for IIH
Obese, female sex, pregnancy, combined OCP (not progesterone only) steroids, tetracyclines (lymecyclime for acne, doxycycline), vitamin A and lithium
Features of an intracranial venous thrombosis
Headache (thunderclap, sudden onset)
Nausea and vomiting
Seizures, hemiplegia, cranial nerve palsies
Amyotrophic lateral sclerosis
Most common MND
LMN signs in arms and UMN signs in legs
Primary lateral sclerosis
UMN signs only
Progressive muscular atrophy
LMN signs only
Distal muscles before proximal
Best prognosis
Progressive bulbar palsy
Palsy of tongue, muscles or chewing and swallowing, and facial muscles due to loss of function of brain stem motor nuclei
Worst prognosis
General features of MND
Fasiculations
Absence of sensory signs and symptoms
Mixed UMN and LMN signs in ALS
Wasting of small hand muscles and tibial is anterior is common
Hypophonic speech
Dysphagia- liquids first then solids
Doesn’t affect external eye muscles
No cerebellar involvement
Abdominal reflexes preserved, sphincter dysfunction if present is a very late feature
Associated with FTD
Investigations for MND
Clinical diagnosis
Blood tests for electrolytes etc.
Normal motor condition on nerve conduction studies
EMG (different to nerve conduction)- see muscles that have lost nerve supply
MRI- exclude myelopathy
Management of MND
Supportive- exercise, diet, physio etc.
Medical- riluzole (ALS- 3 months)
Respiratory support- BIPAP at night
Pituitary apoplexy
Sudden enlargement of a pituitary tumour secondary to haemorrhage or infection
Sx- headache (thunderclap), neck stiffness, visual field defects, cranial nerve palsies, signs of pituitary insufficiency;
low ACTH
tiredness
postural hypotension
low FSH/LH
amenorrhoea
infertility
loss of libido
low TSH
feeling cold
constipation
low GH
if occurs during childhood then short stature
low prolactin
problems with lactation
Management- steroid replacement (and replacement of other pituitary hormones), fluid balance, surgery
Features of raised ICP
Headache, vomiting, reduced consciousness, paipilloedmea, cushings triad (wide pulse pressure (HTN), bradycardia, irregular (reduced) breathing)
This is in contrast to shock- decreased BP, tachycardia, and tachypnoea
Management of raised ICP
Treat underlying cause
Head elevation to 30 degrees
IV mannitol
Controlled hyperventilation (reduce pCO2)
Remove CSF eg. Shunt
Causes and associations of restless leg syndrome
Family history
Iron deficiency anaemia
Uraemia
DM
PD
Pregnancy
Management
Exercises eg, stretches, walking
Treat iron deficiency
Dopamine agonists eg. Ropinirole or pramipexole
Benzodiazapenes
Gabapentin
Subacute combined degeneration of the spinal cord
Vitamin B12 deficiency
Dorsal columns and lateral corticospinal tracts affected
Joint position and vibration sense lost first, then distal parasthesia
UMN signs in legs
If untreated permanent damage can persist
Von hippel lindau syndrome
Autosomal dominant predisposing to neoplasia
Cerebellar haemangiomas
Retinal “”
Renal cysts
Phaeochromocytoma
Renal carcinoma
Warning features in GBS
Autonomic dysfunction eg. Tachycardia
Respiratory muscle weakness- dyspnoea (need serial FVC measurements), if dropping, transfer to ICU)
Transverse myelitis and MS
Oligoclonal bands in CSF can suggest transverse myelitis or MS (they suggest inflammation of the spine and resulting myelopathy). If first presentation you could suggest TM, but if previous neurological symptoms then you would think MS (history is important here).
Causes of a raised CK
Myosotis eg. PM or DM
Rhabdomyolysis (post trauma or prolonged stay on floor)
AKI
MI
Statins
Strenuous exercise
NB- not PMR
Viral meningitis or encephalitis based on CSF interpretation
If CSF is thought to be contained with a virus, whether it is meningitis or encephalitis depends not only on imaging, but also clinical history and picture (encephalitis patients more sick- they may have focal neurology, behavioural changes, seizures etc.)
Delirium following herpes zoster infection
Think herpes zoster encephalitis?
Management of any mononeuropathy eg. Common peroneal nerve palsy
Supportive- avoid compressing it, avoid contact sports, splint, physio etc.
Medical- analgesia if pain (usually there isn’t)
Surgical- nerve repair if not healed and causing issues months down the line
Difference between a common peroneal nerve palsy and prolapsed IV disc
Prolapsed disc- significant pain and loss of Ipsilateral ankle reflex
Common Peroneal nerve palsy- typically painless and ankle reflex is preserved (L5 Radiculopathy)
Cause of wrist drop
Compression of radial nerve
Saturday night palsy
Relapsing remitting MS
Most common
Acute attacks last 1-2 months, followed by periods of remission
Secondary progressive MS
Relapsing remitting patients who have deteriorated and developed neurological signs and symptoms between relapses
Develops in 65% of RR patients within 15 years of diagnosis
Primary progressive MS
Progressive deterioration from onset
Diagnosing MS
2 or more relapses and either objective or clinical evidence of 2 or more lesions (disseminated in time and space)
NB- MRI of head and spine will show lesions, lumbar puncture (oligoclonal bands) will also aid diagnosis
Also do neuro exam, FBC, UE, LFT, TFT, B12, folate
Features of MS
Visual- optic neuritis (RAPD can be a sign of current or old optic neuritis- swinging light test, damage to CN II), optic atrophy, Uhtoffs phonomenon (worsening of Sx following increased temperature), INO (conjugate gaze palsy)
Sensory- parastehsia, pins and needles, trigeminal neuralgia, Lhermittes syndrome (neck flexion)
Motor- increased tone and spasticity (depends on where lesions are eg. Spinal cord lesion could cause spastic paraparesis)
Cerebellar- ataxia, tremor, other cerebellar syndromes
Others- urinary incontinence, sexual dysfunction, intellectual deterioration
Management of Acute MS relapse
High dose steroids (oral or IV methylprednisolone) for 5 days to shorten the length of an acute relapse
General management of MS
Supportive- physiotherapy, lifestyle modifications, patient education, spasticity (baclofen and gabapentin), treat bladder and bowel dysfunction (bladder scan first to assess bladder emptying) eg. Catheter or stoma or oxybutinin (urinary frequency), occupational therapy, fatigue (amantidine)
Medical- natalizumab , a monoclonal antibody is better than beta interferon
Most common presenting symptom of MS
Fatigue
Febrile convulsions
Typically children between 6 months- 5 years old
Generalised tonic clinic in nature
Alcohol withdrawal seizures
Sudden stopping of alcohol in a patient with alcohol excess history
Peak-36 hours
Psychogenic non epileptic seizures
Epileptic like seizures but no electrical discharges
May have history of mental health problems or a personality disorder
Infantile spasms (West syndrome)
Flexion of head trunk and limbs
Progressive mental handicap
EEG- hypsarrhytmia
Usually secondary to a serious neurological abnormality
NB- may progress to Lennox gaustaut syndrome (atypical absences, falls, jerks etc).
Benign Rolandic epilepsy
Parastehsia (unilaterl face), usually on waking up
Juvenile myoclonic epilepsy (Janz syndrome)
Onset in teens (girls)
Generalised seizures, often in morning
Daytime absences
Sudden muscle jerks (eg. dropping things)
Good response to valproate
NB- like absence seizure epilepsy, but muscle jerks (typically in morning)
Pharmacological management of epilepsy
AED started after second seizure
Generalised seizures- sodium valproate (lamotrigine or carbamazepine 2nd line)
Focal seizures- carbamazepine/lamotrigine (valproate 2nd line)
NB- always prescribe by brand
Adverse effects of valproate
Increase appetite and weight gain
Alopecia
P450 enzyme inhibitor
Ataxia
Tremor
Hepatitis
Pancreatitis
Teratogenic
Thrombocytopenia
Adverse effects carbamazepine
AGRANULOCYTOSIS- sore throat
P450 enzyme inducer
Dizziness and ataxia
Drowsiness
SIADH
Visual disturbances
Aplastic anaemia
Adverse effects lamotrigine
Stevens johnson syndrome
Adverse effects phenytoin
P450 enzyme inducer
Dizziness and ataxia
Drowsiness
Peripheral neuropathy
Megaloblastic anaemia
Teratogenic
Localising lesions of focal seizures
Temporal lobe (head)- hallucinations, epigastric rising, emotion, lip smacking, grabbing, or plucking, dysphagia, deja vu
Frontal lobe (motor)- head of leg movements, posturing, post ictal weakness, Jacksonian march (can get secondary tonic-clonic seizure after initial Jacksonian march)
Parietal lobe (sensory)- parasthesia
Occipital lobe (visual)- floaters or flashes
When to investigate for epilepsy
After the second generalised seizure eg. EEG and MRI brain
Management of a first seizure/ suspected epilepsy
Refer to neurology/ first seizure clinic (don’t drive, bring the witness with you if possible)
Lifestyle NA agent- promote general health
Inform the DVLA
Advice eg. Shower, swimming, heights, traffic, cautious with heavy hot or electrical equipment
Adhere to AED’s
Surgery for extreme cases
Status epilepticus
Single seizure lasting longer than 5 minutes
Or
More than 2 seizures in a 5 minute period without the patient returning to normal between them
Management of status epilepticus
ABCDE (check oxygen and BM)
In community- 10mg diazepam rectally or 10mg buccal midazolam
In hospital- secure airway, IV lorazepam 4mg (repeated after 10 minutes)
If ongoing status- start a phenytoin (2nd line agents) infusion (phenobarbital if already on phenytoin)
If refractory, after 45 minutes induce general anaesthesia (thiopental sodium)
What to do when someone has a seizure in the community
Put patient in safe position eg, lay on floor (recovery position if possible)
Put something soft under head
Remove obstacles that could cause injury
Make a note of time it started
Call ambulance if longer than 5 minutes- or if this is first seizure
Spastic tetraparesis
UMN signs in all 4 limbs
Spastic paraparesis
UMN signs in 2 limbs
Cranial nerve and limb involvement
Either stroke or brain stem pathology
Causes of recurrent stroke in a young person
Thrombophilia
Emboli (infective endocarditis)
Vasculitides
Autoimmune condition (SLE)
Recurrent drug use (cocaine);
Coughing and headaches
The red flag is when the headache is precipitated by the cough (raised ICP), not made worse (migraine)
Causes of increased ICP with a normal head CT
Cerebral venous sinus thrombosis
Idiopathic intracranial hypertension
NB- need to do a CT venogram to exclude the VST first
Signs of MG
Fatiguable ptosis- keep eyes in vertical gaze and eyelids will shut
Weak orbital muscles- shut eyes and they should be easy to open
Inability to whistle
Measuring neuromuscular respiratory impairment
FVC (restrictive disease- peak flow is used for obstructive disease such as COPD and asthma)
Breath count is a surrogate marker eg. Take breath and count as many numbers as possible
Neck flexion weakness is a bad sign in GBS (C345 supply diaphragm but also control neck flexion, if this is weak, diaphragm may also be weak)
Metabolic causes of encephalopathy
Hypoxia, increased urea,increased ammonia, septic encephalopathy (delirium), blood sugars
Red flags for headache
Worse when leaning forward, coughing
Wakes patient up at night
Morning headache
Associated neurological and visual deficits
Signs of infection eg. Meningism (photophobia)
Causes of a thunderclap headache
SAH
Intracerebral bleed
Dissected vertebral or carotid arteries
Cerebral venous sinus thrombosis (previous malignancy, young, female, OCP, thrombophilia, dehydration are risk factors)
Low CSF pressure (meningeal rupture)- headache worse standing up (not lying down)
NB- thunderclap headache is immediate. If it builds up over 30-40 minutes it isn’t a thunderclap headache.
Incontinence and UMN lesions
Lower spinal lesion- lack of sensation (don’t feel urge to go). Insensate
Upper spinal lesion- urgency incontinence
Hyper intensities
Lesions in demylinating conditions eg. MS
Hemiplegic gait
Unilateral flexed upper limb and extended lower limb (elongated)
Patients circumduct affected leg
UMN
Causes- stroke, SOL, trauma, MS
Diplegic (scissor) gait
Similar to hemiplegic gait but bilateral in nature
Circumduction of both legs and potential overlap
UMN
Causes- prolapsed disc, spinal tumour or infarct, spastic paraparesis, cerebral palsy, MS, MND (lower motor neurone signs)
Parkinsonism gait
Slow to start walking
Reduced stride length with short steps. Subsequent steps may get smaller (festinant)
Reduced arm swing
Stooped posture
Resting tremor
Impaired balance or hesitancy on turning
Causes- IPD or Parkinson plus syndromes
Ataxic gait
Cerebellar, sensory, or vestibular cause
Stance- broad based
Stability- staggering, slow, unsteady (if a unilateral cerebellar lesion- will veer towards side of lesion)
May require a walker/something to hold onto whilst walking
Turning- turning manoeuvre can be difficult
May be additional Sx;
Features associated with cerebellar ataxia
DANISH
Dysdiadokokinesia
Ataxia
Nystagmus
Intention tremor
Speech
Hypotonia
Causes- cerebellar stroke, SOL, MS, alcoholism, B12 deficiency, lithium, spinocerebellar ataxia
Features associated with sensory ataxia
Positive Rhombergs (cerebellar ataxia- unsteady whether eyes open or closed, sensory, more prominent when eyes closed)
Impaired proprioception
Impaired vibration sense
Absent cerebellar signs
Cause- peripheral neuropathy eg. DM
Features associated with vestibular ataxia
Vertigo
Nausea
Vomiting
Causes- labyrinthitis, Ménière’s disease, acoustic neuroma
Neuropathic (high-steppage) gait
Foot drop and toe dragging- to prevent this there is excessive knee and hip flexion (high steppage)
LMN
Causes- common nerve palsy, L5 radiculopathy (disc), MND
Myopathic (waddling or trendelenburg) gait
Waddling from side to side
Circumduction of legs
Positive Trendelenburgs sign- stand on one leg, pelvis drops to contra lateral side (due to weakness of abductor muscles on other side)
Cause- thyroid disease, Cushing’s syndrome, acromegaly, poly myalgia rheumatica, polymyositis, muscular dystrophy (Duchenne)
Choreiform (hyperkinetic) gait
Involuntary movements during gait eg. Oreo facial dyskinesia
Chorea in upper and lower limbs
Causes- HD, Sydenham’s chorea, cerebral palsy, Parkinson’s medications, Wilson’s disease
Antalgic gait
Stance phase reduced on affected leg giving limp appearance
Cause- OA, fracture, nerve entrapment eg. Sciatica
Cancer patient with meningism
Think malignant meningitis
Causes of Horners syndrome’
Brain stem, lung, or carotid pathologies (BLC)
Brain stem tumour, Pam coast lung tumour, carotid artery dissection or aneurysm, migraine (but if new onset in an older person- be suspicious and do a CT angiogram)
When do signs of a stroke typically appear on a CT
12 hours afterwards
Phenytoin adverse effects
Can cause heart block and arrhythmias
With any heart disease (structural or arrhythmia (AF)) don’t use IV phenytoin
What anaesthetic agent are people with MG resistant to?
Suxamethonium
Cranial nerves affected in vestibular schawannomas
V VII VIII
When to intubate somebody
If GCS is less than 8
What is the mechanism of reducing ICP with hyperventilation
Reduced blood CO2 to induce cerebral vasoconstriction
Acute vs chronic subdural haematoma
Acute- hyper dense (bright)
Chronic- hypodense (dark)
Criteria for brain stem death
Deep coma of known aetiology
Reversible causes excluded
No sedation
Normal electrolytes
Management of hydrocephalus
EVD- acute
VPS- long term therapy
Obstructive (non-communicating)- remove obstructive pathology
NB- never use LP in obstructive hydrocephalus or reduced ICP as the difference in cranial and spinal pressures induced by the drainage will cause brain herniation
What ECG change may be seen during SAH
ST elevation
Management of SAH
Coil (but some need clipping via a craniotomy)
Vasospasm prevented by nimodipine (calcium channel antagonist) for 21 days
NB- hyponatraemia due to SIADH may occur
What are the 2 most important tests to do before any others when someone presents with a seizure
Blood glucose
Oxygen (ABG, sats)
Syringomelia
Fluid filled cyst in the centre of the spinal cord (anterior white commissure)
Associations- Arnold chiari malformation, trauma, tumours, idiopathic
Cape like loss of pain and temperature sensation (think shoulders)- spinothalamic tracks in anterior commissure damaged
Spastic weakness (lower limbs)
Neuropathic pain
Upgoing plantars
Autonomic features
Horner’s
Scoliosis (if chronic and untreated)
Brain and spine MRI- exclude tumour, tethered cord, chiari malformation
Can have a shunt
Co careldopa
Can only be given orally
If someone can’t take their usual levodopa, give a dopamine agonist patch
Stopping an AED
If seizure free for 2 years, done over 2-3 months
Central contusions on scan but no focal neurology
Insert an intra cranial pressure monitoring device
May develop raised ICP in a few days
EEG for absence seizures
3Hz oscillations
Tuberous sclerosis
Depigmented ash leaf spots which fluoresce under UV light
Roughened patches of skin over lumbar spine
Angiofibromas
Fibromatas beneath nails
Cafe au lait spots
Developmental delay
Epilepsy
Retinal haematomas
Hoffman sign
UMN lesion
Flick middle finger and will see flexion of thumb
Management of prolactinomas
Dopamine agonists eg. Cabergoline, bromocriptine
Management of focal seizures
1st line- lamotrigine or levetiracetam
2nd line- carbamazepine, oxcarbazepine or zonisamide
Management of tonic clinic seizures
Males- Valproate
Females- lamotrigine or levetiracetam
NB- valproate can be considered for girls under 10/those who won’t have children
Management of absence seizures
first line: ethosuximide
second line:
male: sodium valproate
female: lamotrigine or levetiracetam
NB- carbamazepine may exacerbate absence seizures
Management of neuroblastoma
Refer very urgently (within 48 hours) to see the paediatrician
Complex regional pain syndrome
Progressive/disproportionate symptoms to original injury/surgery
Allodynia
Temperature and skin colour changes
Oedema and sweating
Motor dysfunction
Budapest diagnostic criteria
Physiotherapy
Neuropathic analgesia
Pain team input
GCS Motor response
6- obeys commands
5- localised to pain
4- withdraws from pain
3- abnormal flexion to pain
2- extension to pain
1- none
GCS Verbal
5- orientated
4- confused
3- words
2- sounds
1- none
GCS Eye opening
4- spontaneous
3- to speech
2- to pain
1- none
Features of hydrocephalus
Hydrocephalus presents in infants with an increasing head circumference (splaying of the skull plates allowed by unfused sutures), bulging fontanelles, impaired upward gaze (‘sunsetting’; caused by pressure on the tectal plate), dilated scalp veins, bradycardias, seizures and coma.
Management of neuropathic pain
First-line treatment for neuropathic pain is amitriptyline (TCA), duloxetine (SSRI), gabapentin (anticonvulsant) or pregabalin (anticonvulsant. If one of these agents don’t work next line is to try one of the other remaining three drugs
NB- trigeminal neuralgia responds best to carbamazepine
NB- also incorporate physiotherapy, CBT, capsaicin cream, tramadol for short term rescue of flare ups
Features of a focal seizure
Hallucinations
Memory flashbacks
Déjà vu
Doing strange things on autopilot
Features of generalised tonic clonic seizures
There is loss of consciousness and tonic (muscle tensing) and clonic (muscle jerking) episodes. Typically the tonic phase comes before the clonic phase. There may be associated tongue biting, incontinence, groaning and irregular breathing.
Prolonged post ictal period
Types of seizure
Generalised- involve neural network on both sides of the brain. Can be motor or non-motor eg. tonic-clonic (grand mal), tonic, clonic, typical absence (petit mal), atonic (someone who drops suddenly, possible incontinent and a post-ictal period that follows). No awareness
Focal- these start in a specific area, on one side of the brain. Can be classified as being motor (e.g. Jacksonian march), non-motor (e.g. déjà vu, jamais vu; ) or having other features such as aura. Awareness can vary
Features of a myoclonic seizure
Sudden brief muscle contractions, like a sudden “jump”. The patient usually remains awake during the episode
Juvenile myoclonic epilepsy
Features of an atonic seizure
They are characterised by brief lapses in muscle tone. These don’t usually last more than 3 minute- “drop attacks”
Lennox-Gastaut syndrome
Causes of neuropathic pain
Postherpetic neuralgia from shingles is in the distribution of a dermatome and usually on the trunk
Nerve damage from surgery
Multiple sclerosis
Diabetic neuralgia typically affects the feet
Trigeminal neuralgia
Complex Regional Pain Syndrome (CRPS)
Lumbar radiculopathy
2 drugs that can be used in certain brain tumours
Bromocriptine- prolactin-secreting tumours
Ocreotide (somatostatin analogues)- growth hormone-secreting tumours (acromegaly)
NB- surgery is first line in acromegaly, whereas drugs are first line in prolactinomas
Anticipation
Huntington’s chorea displays something called genetic “anticipation”. Anticipation is a feature of trinucleotide repeat disorders. This is where successive generations have more repeats in the gene, resulting in:
Earlier age of onset
Increased severity of disease
Lambert-Eaton myasthenic syndrome
Muscle weakness similar to MG, caused by SCLC, muscle weakness improves on repeated action
NB- Voltage-gated calcium-channel antibodies
Charcot Marie Tooth disease
Inherited condition- presents in childhood (AD)
High foot arches (pes cavus)
Distal muscle wasting causing “inverted champagne bottle legs”
Weakness in the lower legs, particularly loss of ankle dorsiflexion
Weakness in the hands
Reduced tendon reflexes
Reduced muscle tone
Peripheral sensory loss
Causes of peripheral neuropathy
ABCDE
Alcohol
B12 deficiency
Cancer and Chronic Kidney Disease
Diabetes and Drugs (e.g. isoniazid, amiodarone and cisplatin)
Every vasculitis
Features of GBS
back/leg pain in the initial stages of the illness
progressive, symmetrical weakness of all the limbs.
-the weakness is classically ascending i.e. the legs are -affected first
-reflexes are reduced or absent
-sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs
there may be a history of gastroenteritis
respiratory muscle weakness
cranial nerve involvement
-diplopia
-bilateral facial nerve palsy
-oropharyngeal weakness is common
autonomic involvement
-urinary retention
-diarrhoea
Todds paresis
Patients with focal seizures may experience post-ictal weakness (Todd’s paresis)
A focal weakness, typically of the frontal lobe (motor strip), may occur following a focal-onset seizure
During a focal seizure, patients can either be aware or unaware of their surroundings.
Polyneuropathy vs mononeuritis multiplex
Mononeuritis multiplex- pattern of involvement is asymmetric
Polyneuropathy- symmetric
Lumbar puncture and raised intracranial pressure
If a LP is performed with raised ICP, there is a risk of coning. Thus, you wouldn’t perform one if there was evidence of papilledema or clinical signs and symptoms (pain when coughing, early morning etc.) eg. in suspected meningitis with evidence of papilledema, just start antibiotics
Management of Stevens-Johnson syndrome
Cease all provoking medications, obtain IV access and begin fluid hydration
Hoover’s sign
If a patient is genuinely making an effort, the examiner would feel the ‘normal’ limb pushing downwards against their hand as the patient tries to lift the ‘weak’ leg. Noticing this is indicative of an underlying organic cause of the paresis. If the examiner, however, fails to feel the ‘normal’ limb pushing downwards as the patient tries to raise their ‘weak’ leg, then this is suggestive of an underlying functional weakness, also known as ‘conversion disorder’.
Cranial nerve control
Contralateral
6 tests to confirm brain death
pupillary reflex, corneal reflex, oculo-vestibular reflex, cough reflex, absent response to supraorbital pressure, and no spontaneous respiratory effort
L5 radiculopathy vs CNP lesion
in L5 radiculopathy, eversion tends to be spared
2 distinct causes of hypopituitsim
Sheehan’s syndrome (due to PPH)
Pituitary apoplexy (due to tumour)
Criteria for CT following head injury
CT head immediately
GCS < 13 on initial assessment
GCS < 15 at 2 hours post-injury
suspected open or depressed skull fracture.
any sign of basal skull fracture (haemotympanum, ‘panda’ eyes, cerebrospinal fluid leakage from the ear or nose, Battle’s sign).
post-traumatic seizure.
focal neurological deficit.
more than 1 episode of vomiting
CT head scan within 8 hours of the head injury - for adults with any of the following risk factors who have experienced some loss of consciousness or amnesia since the injury:
age 65 years or older
any history of bleeding or clotting disorders (or on warfarin)
dangerous mechanism of injury (a pedestrian or cyclist struck by a motor vehicle, an occupant ejected from a motor vehicle or a fall from a height of greater than 1 metre or 5 stairs)
more than 30 minutes’ retrograde amnesia of events immediately before the head injury
NB- A patient with GCS <8 or = to 8 needs urgent neurosurgical review. Especially when an open fracture is present.
NB- suspicion of C spine injury, CT c spine
Head injury management
Raised ICP and waiting for theatre- IV mannitol
Diffuse cerebral oedema- decompressive craniotomy
No Burr holes
ICP monitoring is mandatory for GCS 3-8 and abnormal scan (recommended even if scan is normal)
Cerebral perfusion in adults- above 70
Cerebral perfusion in children- 40-70
Jacksonian march
Focal seizures starts in one area eg. fingers, then moves progressively (as the electrical activity moves across the brain)
Can lead to secondary tonic-clonic seizure
Focal seizure with impaired awareness vs absence seizures
Focal seizure with impaired awareness will have prodrome and post-ictal phase (absence seizures occur and terminate rapidly)
Management of a myoclonic seizure
males: sodium valproate
females: levetiracetam
myoleve (remember like that)
Management of tonic/atonic seizure
males: sodium valproate
females: lamotrigine
Ulnar paradox
The ulnar paradox: proximal lesions of the ulnar nerve produce a less prominent deformity than distal lesions (ie. distal lesion will worsen during recovery, before improving)
Coning
‘Coning’ refers to an uncal herniation as a result of raised intracranial pressure. In this case, as the subdural haemorrhage pushes the brain through the tentorium, uncal herniation is causing damage to the 3rd cranial nerve. This nerve is vulnerable due to its anatomical position predisposing it to external compression by the tentorium.
3rd nerve palsy/down and out gaze
Sciatica
No more gabapentin/anticonvulsants/SNRI’s- use NSAIDs and weak opioids (and exercise, surgery etc.)
SAH and LP
If a CT head scan done within 6 hours of symptom onset and reported and documented by a radiologist shows no evidence of a subarachnoid haemorrhage:
do not routinely offer a lumbar puncture
think about alternative diagnoses and seek advice from a specialist.
1.1.12If a CT head scan done more than 6 hours after symptom onset shows no evidence of a subarachnoid haemorrhage, consider a lumbar puncture.
1.1.13Allow at least 12 hours after symptom onset before doing a lumbar puncture to diagnose a subarachnoid haemorrhage.
LP Timings
Suspected bacterial meningitis: an LP should be done before IV antibiotics, unless:
cannot be done within 1 hour
signs of severe sepsis or a rapidly evolving rash
significant bleeding risk
signs of raised intracranial pressure
Unilateral UMN Pathology
Intracranial – stroke, SOL → hemisensory loss
Brainstem – stroke, SOL → may be crossed signs
Spinal cord – MS, infarct/haemorrhage, SOL, disc prolapse, trauma, syringomyelia, congenital → sensory-level/segmental sensory loss
Bilateral UMN Pathology
MS
Motor neurone disease → normal sensation
Myelopathy – cord compression (e.g. due to cervical myelopathy, SOL, disc prolapse, paraspinal infection), trauma, transverse myelitis, syringomyelia, congenital → sensory-level/segmental sensory loss
Others – brainstem stroke, hereditary spastic paraplegia, cerebral palsy, HTLV-1, syphilis
Unilateral LMN Pathology
See helpful diagram
Bilateral LMN Pathology (with distal weakness)
Abnormal sensation distally i.e. sensorimotor polyneuropathy
ABCDE:
Alcohol
B12/thiamine deficiency
Charcot-Marie-Tooth, Carcinomas (paraneoplastic)
Diabetes, Drugs (e.g. TB drugs, metronidazole/nitrofurantoin, vincristine/cisplatin, amiodarone)
Every vasculitis (e.g. SLE, RA, polyarteritis nodosa) and some infections (e.g. herpes zoster, HIV, leprosy, syphilis)
Normal sensation i.e. distal motor neuropathy
Chronic inflammatory demyelinating polyneuropathy
Myotonic dystrophy
Inclusion body myositis (proximal in legs but distal in arms)
Progressive muscular atrophy
Lead poisoning
Porphyria
Acute flaccid paralysis
Guillain-Barré syndrome
Some rare infections (e.g. rabies, polio, West Nile)
Cauda equina syndrome
Spinal cord shock
Bilateral Proximal Weakness (normal sensation)
DENIM:
Dystrophies – Becker’s/Duchenne, limb girdle, facioscapulohumeral (shoulder, face and truncal weakness)
Endocrinological – Cushing’s syndrome, hyper/hypothyroidism, diabetic amyotrophy (lower limbs)
Neuromuscular – myasthenia gravis (fatigable), Lambert–Eaton myasthenic syndrome
Inflammatory – dermato-/polymyositis, inclusion body myositis (proximal in legs but distal in arms), viral myositis
Metabolic/congenital/mitochondrial myopathies
Mononeuritis Multiplex
Vasculitis – granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, polyarteritis nodosa, microscopic polyangiitis
Autoimmune – RA, SLE, cryoglobulinaemia, Sjögren’s syndrome, paraneoplastic
Infectious – Lyme disease, HIV, leprosy
Others – diabetes mellitus, amyloidosis, sarcoidosis
UMN and LMN Pathology
Motor neurone disease (no sensory deficit)
Dual pathology (e.g. cervical myelopathy + polyneuropathy)
Myeloradiculopathy
Subacute combined degeneration of the cord
UMN and LMN Pathology
Motor neurone disease (no sensory deficit)
Dual pathology (e.g. cervical myelopathy + polyneuropathy)
Myeloradiculopathy
Subacute combined degeneration of the cord
Cerebellar Pathology
MAVIS:
MS
Alcohol
Vascular – thromboembolic, haemorrhagic
Inherited – Friedreich’s ataxia, spinocerebellar ataxia, ataxia telangiectasia
SOL
Parkinsonism
Parkinson’s disease
Vascular parkinsonism
Parkinson-plus syndromes: multi-system atrophy, progressive supranuclear palsy, corticobulbar degeneration, Lewy body dementia
Other causes: antidopaminergic drugs, Wilson’s disease, communicating hydrocephalus, supratentorial tumours
Horner’s Syndrome
Central lesions
Anhidrosis of the face, arm and trunk
Stroke
Syringomyelia
Sclerosis (Multiple)
SOL
Encephalitis
Pre-ganglionic lesions
Anhidrosis of the face
Tumour (Pancoast)
Thyroidectomy
Trauma
Cervical rib
Post-ganglionic lesions
No anhidrosis
Carotid artery dissection
Carotid aneurysm
Cavernous sinus thrombosis
Cluster headache
NB- STC
Third Nerve Palsy
Pupil-Sparing;
DM
HTN
Ischaemic
Non-Pupil-Sparing (ie. dilated pupil);
Idiopathic
Tumour
Trauma
Cavernous sinus thrombosis
Posterior communicating aneurysm (pupil dilated, often associated pain)
Raised ICP
MS
UMN and LMN Signs
see helpful diagrams
Subacute Combined Degeneration of Spinal Cord
B12 deficiency
Mixed UMN and LMN signs
weakness
joint position and vibration sense lost first then distal paraesthesia
upper motor neuron signs typically develop in the legs, classically extensor plantars, brisk knee reflexes, absent ankle jerks
CMT
LMN signs
High-arched feet (pes cavus)
Hammer toes
Foot drop
Champagne bottled legs
Distal muscle weakness
Distal muscle atrophy
Hyporeflexia
GBS
LMN signs
Progressive symmetrical weakness of limbs
reflexes are reduced or absent
sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs
Cauda equina
LMN (not the CNS, its the peripheral nerves (PNS) that come off the spinal cord)
low back pain
bilateral sciatica
reduced sensation/pins-and-needles in the perianal area
decreased anal tone
urinary dysfunction
incontinence is a late sign that may indicate irreversible damage
Myelopathy
UMN (spinal cord)
Radiculopathy
LMN ie. sciatica
