neuro Flashcards
condition associated with bilateral acoustic neuromas
neurofibromatosis type 2 (NF2)
how can you distinguish between a CSF leak and mucus coming from a patient’s nose?
check glucose - CSF has glucose
syringomyelia
fluid-filled cavity develop in central canal of spinal cord
|compresses the spinal cord tracts
which part of the spinal cord is compressed first in syringomyelia?
anterior white commissure
spinothalamic tract decussates here at level of entry
which sensory fibres are carried in the spinothalamic tract?
pain and temperature
which sensory fibres are carried in the dorsal column?
light touch, vibration and proprioception
syringomyelia typical presentation
bilateral loss of pain and temperature at level of syrinx
- burns on fingers
-> ‘cape-like’ sensation loss of arms, shoulders and torso
syringomyelia associated malformation
chiari type 1 malformation
syringomyelia imaging
MRI Full Spine
MRI Brain - check for Chiari malformation
What is tuberous sclerosis
autosomal dominant condition with cutaneous, neurological and other features
cutaneous features of tuberous sclerosis? (4)
‘Ash leaf’ spots under UV light = depigmented patches
Shagreen patches = roughened skin over lumbar spine
Adenoma sebaceum (angiofibromas) - facial, butterfly distribution
Subungual fibromata
(Cafe au lait spots - more often in NF)
neurological features of tuberous sclerosis? (3)
developmental delay
intellectual impairment
infantile spasms/partial seizures
‘other’ features of tuberous sclerosis? (6)
Retinal hamartomas = dense white areas on retina
Rhabdomyomas of heart
Gliomatous changes in brain lesions
Polycystic kidneys
Renal angiomyolipomata
Lymphangioleiomyomatosis -> multiple lung cysts
What is mononeuritis multiplex?
Simultaneous/sequential involvement of individual, non-contiguous nerve trunks
This causes acute/subacute sensory and motor loss in asymmetrical pattern
What are the Motor scores of GCS?
6 = obeys 5 = localises to pain 4 = withdraws from pain 3 = abnormal flexion 2 = extending from pain 1 = none
What are the Verbal scores of GCS?
5 = orientated 4 = confused 3 = words 2 = sounds 1 = none
What are the Eye scores of GCS?
4 = spontaneous 3 = speech 2 = pain 1 = none
What is Subacute Combined Degeneration of Spinal Cord?
Degeneration of dorsal and lateral columns due to vitamin B12 deficiency
Uncommon in UK, very common in the developing world
What type of fibres are carries in the corticospinal tract?
Voluntary motor fibre
Upper limbs are medial, lower limbs are lateral
What are the symptoms of Subacute Combined Degeneration of the Spinal Cord?
Loss of proprioception and vibration (dorsal column)
Then distal parasthesia
UMN signs in legs - brisk knee reflex, absent ankle reflex
cerebral oedema secondary to a brain tumour Mx
Dexamethasone IV
normal pressure hydrocephalus management triad
wet, wobbly, wacky
urinary incontinence
gait ataxia
dementia
normal pressure hydrocephalus management
ventriculoperitoneal shunting
10% risk of complications eg. seizure, infection, haemorrhage
migraine acute management
triptan + paracetamol
migraine prophylaxis
topiramate
propranolol
What is an essential tremor?
Autosomal dominant tremor
Generally of both upper limbs
Often have family history
What makes an essential tremor better/worse?
Worse with arms outstretched
Better with alcohol/rest
What is the management of an essential tremor?
Propranolol
What is miosis?
Pupil constriction
What is ptosis?
Eyelid drooping
What does ptosis + dilated pupil indicate?
CN3 palsy
What does ptosis + constricted pupil indicate?
Horner’s syndrome
What is Weber’s syndrome?
Midbrain stroke
What are the features of Weber’s syndrome?
Ipsilateral CN3 palsy
Contralateral hemiplegia
What is a Chiari malformation?
Herniation of cerebellar tonsils through foramen magnum
What are the features of neuroleptic malignant syndrome? (6)
Pyrexia Muscle rigidity Hypertension Tachycardia Agitated delirium Confusion
What might bloods show in neuroleptic malignant syndrome?
Increased WCC, K+ and CK
Decreased Ca2+
What is the management of neuroleptic malignant syndrome?
Stop antipsychotic
Transfer to ITU
IV fluids
Dantrolene
What is Spontaneous Intracranial Hypotension?
Headache secondary to CSF leak
Generally from thoracic N root sleeve
What is spontaneous intracranial hypotension associated with?
Marfan’s
What are the symptoms of spontaneous intracranial hypotension?
Headache
Worse on standing, better with lying
What is the management of of spontaneous intracranial hypotension?
Fluids and caffeine
Epidural blood patch = 2nd line
What is the treatment of myasthenia gravis?
Neostigmine (long-acting AChE inhibitor)
What type of neuromuscular blocks are myasthenia gravis patients more sensitive to?
Non-polarising eg. Rocuronium
As fewer post-synaptic ACh receptors
What type of neuromuscular blocks are myasthenia gravis patients resistant to?
Polarising eg. Suxamethonium
Which medications may worse myasthenia gravis?
Beta-blockers
What may be found in the CSF of Multiple Sclerosis patients?
Oligoclonal bands
NOT found in blood
What should you do if a patient has GCS<8?
Get anaesthetist
Intubate and ventilate
What is the 1st line management of trigeminal neuralgia?
Carbamazepine
What is the management of Degenerative Cervical Myelopathy?
Urgent spinal surgery referral
Decompression surgery within 6m
What other neurological condition is frontotemporal dementia associated with?
MND
What are the 4 types of MND?
Amyotrophic Lateral Sclerosis
Progressive Lateral Sclerosis
Progressive Muscular Atrophy
Bulbar Palsy
What are the main features of MNDs?
Fasciculations No sensory symptoms Mixed UMN and LMN signs Doesn't affect extraocular muscles No cerebellar signs
What do MND EMGs show?
Reduced action potentials with increased amplitude
Not required for diagnosis
What are the main functions of the common peroneal nerve?
Dorsiflexion and eversion of ankle
Posterolateral leg sensation
What are the main features of L5 radiculopathy?
Foot drop
Weakness of hip abduction
Sensory loss of big toe
Who gets idiopathic intracranial HTN?
Overweight females
What is the management of idiopathic intracranial HTN?
Lose weight
Acetazolamide/Topiramate
Repeated LPs if these fail
What is Lateral Medullary Syndrome?
Posterior Inferior Cerebellar Artery stroke
AKA Wallenberg’s syndrome
What are the features of Lateral Medullary Syndrome?
Ipsilateral FACIAL pain and temperature loss
Contralateral BODY pain and temperature loss
Ataxia
Nystagmus
What is Lateral Pontine Syndrome?
Anterior Inferior Cerebellar Artery stroke
What are the features of Lateral Pontine Syndrome?
Ipsilateral FACIAL pain/temp loss Ipsilateral FACIAL paralysis and deafness Contralateral BODY pain/temp loss Ataxia Nystagmus
Where must a lesion by above to result in autonomic dysreflexia?
Above T6
What is Lhermitte’s sign?
Patient bends their neck -> tingling in hands
Indicates disease near dorsal column
Seen in MS and SCDSc
What is Uhthoff’s phenomenon?
Worsening of vision following a rise in body temperature
Seen in MS
What does a CN3 palsy with dilated pupil indicate?
Surgical cause
Need urgent head CT to rule out bleed/aneurysm
What are some features indicative of temporal lobe epilepsy?
Lip-smacking/cloth plucking
Aura
Deja vu
Post-ictal dysphasia
How is the MRC muscle power score scored?
0 = no movement 1 = trace contraction 2 = movement with gravity eliminated 3 = movement vs gravity but not resistance 4 = movement vs resistance but weakened 5 = normal
What are the MOTOR peripheral neuropathies? (6)
Guillain-Barre syndrome Porphyria Lead poisoning HSMN (Charcot-Marie-Tooth) Chronic Inflammatory Demyelinating Polyneuropathy Diphtheria
What are the SENSORY peripheral neuropathies? (6)
Diabetes Uraemia Leprosy Alcoholism B12 deficiency Amyloidosis
What are the features of a venous sinus thrombosis?
Gradual onset headache
N+V
Depends on sinus
What are the features specific to a sagittal sinus thrombosis?
Seizures
Hemiplegia
What are the features specific to a cavernous sinus thrombosis?
Periorbital oedema
Ophthalmoplegia - CN6 1st
Trigeminal N involvement -> hyperaesthesia of upper face and eye
Central retinal vein thrombosis
What are the features specific to a lateral sinus thrombosis?
CN6+7 palsy
What is the gold standard diagnostic investigation for venous sinus thrombosis?
MR venogram
What are the myotomes for upper limb reflexes?
Biceps = C5-6 Triceps = C7-8
What are the myotomes for lower limb reflexes?
Knee = L3-4 Ankle = S1-2
What is the ideal investigation if you suspect Degenerative Cervical Myelopathy?
MRI spine
What is Progressive Supranuclear Palsy?
Parkinson’s Plus syndrome
Has poor response to L-dopa
What are the features of progressive supra nuclear palsy?
Parkinson’s symptoms
Dysarthria
Reduced vertical eye movements
What is retinitis pigmentosa?
Loss of rods -> night blindness
Loss of peripheral retina -> tunnel vision
What may be seen on fundoscopy in retinitis pigmentosa?
Black bone spicule-shaped pigmentation of peripheral retina
Mottling of retinal pigment epithelium
What are some features of retinitis pigmentosa?
Night blindness Tunnel vision Often start in childhood Often have FHx of early (but incomplete) blindness Vitamin A may slow progression
What is retinitis pigmentosa associated with? (4)
Refsum disease Usher syndrome Alport syndrome Abetalipoproteinaemia ( and others)
What is Guillain-Barre syndrome?
Immune-mediated demyelination post-infection
Typical campylobacter infection
What are the features of GBS?
Progressive weakness of all 4 limbs Legs progress first Proximal muscles more than distal Few sensory symptoms Diminished reflexes
What may an LP show in GBS?
Increased protein
Normal WCC
What is myasthenia gravis?
Auto-antibodies vs ACh receptor on post-synaptic membrane
What are the features of myasthenia gravis? (5)
Progressive weakness as day goes on Proximal muscle weakness Diplopia - extraocular muscle weakness Ptosis Dysphagia
What are the associations of myasthenia gravis?
Thymomas (15%) Thymic hyperplasia (50-70%) Other autoimmune diseases
What investigations should be done for someone with myasthenia gravis?
Single fibre electromyography CT thorax - exclude thymoma AChR AutoAb - 85-90% +ve Anti-muscle-specifc tyrosine kinase Ab - 40% +ve (Creatinine Kinase is normal)
What test is no longer used for those with myasthenia gravis?
Tension test
IV edrophium temporarily reduces weakness
Not done anymore
What is the management of myasthenia gravis?
Pyridostigmine (long-acting AChE inhibitor)
Prednisolone (immunosuppression)
Thymectomy
What is the management of a myasthenic crisis?
IV immunoglobulin + plasma exchange
Where are lesions causing inferior and superior quadrantanopias?
Parietal = Inferior
Temporal = Superior
(PITS)
What are the 1st line options for migraine prohpylaxis?
Topiramate
Propranolol
What migraine prophylaxis should be used in women taking COCP?
Propranolol
Topiramate can reduce COCP efficacy
What are the 2nd and 3rd line options for migraine prophylaxis?
2nd line = 10 acupuncture sessions
3rd line = Riboflavin 400mg OD
What prophylaxis should be used for predictable menstrual migraines?
Frovatriptan or zolmitriptan
What is intranuclear ophthalmoplegia?
Lesion of Medial Longitudinal Fasciciulus = tract that allows conjugate eye movements
What are the features of intranuclear ophthalmoplegia?
Ipsilateral impaired adduction of eye
Contralateral nystagmus
What is the diagnosis if someone has a painful CN3 plasy with dilated pupil?
Posterior Communicating Artery aneurysm
What are the causes of CN3 palsy? (7)
DWN ANd OUT Diabetes Weber's syndrome Neuro (MS) ANeurysm (posterior communicating artery) Other (SLE, GCA) Uncal herniation Thrombosis (cavernous sinus)
What are the features of ALS?
LMN signs in arms, UMN signs in legs
Most common MND
Familial cases involve Chr21
How could you distinguish between ALS and PLS?
PLS has UMN signs only
ALS has UMN signs in legs and LMN signs in arms
What are the features of Progressive Muscular Atrophy?
LMN signs only
Distal to proximal muscles
Best prognosis
What is Progressive Bulbar Palsy?
MND - loss of brainstem nuclei function
What are the features of Progressive Bulbar Palsy?
Palsy of tongue, muscles of mastication and facial muscles
Worst prognosis
What are the 5 most common primary tumours to metastasise to the brain?
Lung Breast Kidney Melanoma Colorectal
What is the long term management of a stroke?
Clopidogrel
Statin if cholesterol >3.5
(Aspirin STAT after bleed excluded)
What is the MoA of Ondansetron?
5-HT3 antagonist
Acts on medulla oblongata
S/Es = constipation, long QT
Which anti-seizure medication may cause peripheral neuropathy?
Penytoin
What might be raised following a true seizure but not after a non-epileptic seizure?
Prolactin
Raised 10-20mins after epileptic seizure
What are the cerebellar signs?
DANISH Dysdidokokinesis Ataxia Nystagmus Intention tremor Slurred speech Hypotonia (signs are ipsilateral to lesion)
Are cerebellar signs ipsilateral or contralateral to cerebellar lesions?
Ipsilateral
Who should get a thrombophilia screen following a stroke?
<55yrs old + no obvious cause
What is the ROSIER score?
Assesses likelihood presentation is due to a stroke
What are the features of Lacunar infarcts?
ONE of:
Ataxic hemiparesis
Pure sensory stroke
Unilateral weakness
What are the features of Weber’s syndrome?
Ipsilateral CN3 palsy
Contralateral weakness
What are the features of Lateral Medullary syndrome?
Ipsilateral ataxia, nystagmus, facial numbness and dysphagia
Contralateral limb sensory loss
What is the acute management of cluster headaches?
100% oxygen + subcut Sumitriptan
What is the prophylactic management of cluster headaches?
Verapamil
What is the 1st line management of trigeminal neuralgia?
Carbamazepine
What are the Red Flags for trigeminal neuralgia, which may warrant urgent referral? (8)
Age <40yrs Sensory changes Deafness/ear problems Hx of skin/oral lesions which could be perineurial Pain in ophthalmic division Pain bilaterally Optic neuritis Fix of MS
What is the most common sequelae of meningitis?
Sensorineural deafness
What is Todd’s paresis?
Post-seizure focal weakness
Follows focal seizure
When does Steven-Johnson syndrome start after starting a new medication?
Typically 2 months
Flu-like prodrome initially
What are the features of a low pressure headache post-LP?
Develop 24-48hrs post-LP
Worse on standing
More common in women with low BMI
What is the management of a low pressure headache?
Caffeine and fluids (stay hydrated)
What are the symptoms of a subdural haematoma?
Generalised headache with fluctuating GCS
What is the first investigation you order if you suspect a stroke?
Non-contrast CT head
What role do Diffusion-weighted MRIs have in stroke management?
Can help diagnose a vascular stroke
What role do T2-weighted FLAIR MRIs have in stroke management?
Can help with estimate if stroke onset is within thrombolysis window
What are the side effects of sodium valproate? (11)
Nausea Weight gain Alopecia with curly regrowth Ataxia Tremor Thrombocytopenia Pancreatitis Hepatotoxicity Hyponatraemia Hyperammonic encephalopathy TERATOGENIC
What relevance does sodium valproate have for other medications?
P450 inhibitor
When can someone drive again after having a TIA?
Must be symptom-free after 1 month
What can be used as a ‘rescue medication’ for neuropathic pain?
Tramadol
What is the new definition of TIA?
Neurological dysfunction caused by focal CNS ischaemia without infarct
Tissue-based, not time-based
What are 4 MS risk factors?
Smoking
Previous glandular fever
Genetics
Low vitamin D
What is procyclidine?
Antimuscarinic
Used to treat tremor and rigidity
Most useful in drug-induced parkinson’s
What is carbergoline associated with?
Pulmonary fibrosis
What is Hoover’s sign?
Differentiates between organic and non-organic leg weakness
Organic = feels normal leg pushing down to try and raise weak leg
What is Romberg’s test?
Differentiates between sensory and cerebellar ataxia
Stand with eyes closed, +ve if they fall
+ve result indicates sensory ataxia
Cerebellar ataxia will have +ve result with eyes open and closed
What is the characteristic gait of sensory ataxia?
High-stepping gait
What is the characteristic gait of cerebellar ataxia?
Broad-based, staggering gait
What are the acute causes of sensory ataxia? (3)
Miller-Fisher syndrome
Sensory variant of GBS
Semisynthetic penicillins
What are the subacute causes of sensory ataxia? (3)
Lyme disease
Neurosarcoidosis
Posterior spinal cord lesions
What are the chronic causes of sensory ataxia? (6)
CIDP Paraproteinaemia Diabetes Coeliac disease Vitamin E deficiency Isoniazid
What are 5 examples of posterior spinal cord lesions?
MS Cervical spondylosis Tumours Vitamin B12 deficiency Tabes dorsalis (syphilis)
What are the replacement thresholds for low phosphate during refeeding?
<0.80 = low 0.32-0.80 = oral replacement <0.32 = IV replacement
When do you need to worry about refeeding syndrome?
If not eaten for 5+ days
What is the usual replacement regime in refeeding?
10kcal/kg/day - increase to needs over 4-7 days Oral thiamine 300mg OD K+ 2-4mmol/kg/day PO4 0.3-0.6mmol/kg/day Mg 0.2-0.4mmol/kg/day
What drugs should be used for nausea in those with Parkinson’s?
Domperidone
Doesn’t cross BBB
What is the time window for thrombolysis in strokes?
<4.5hrs from onset
Combined with thrombectomy if CONFIRMED proximal circulation occlusion
What is the time window for thrombectomy in strokes?
<6hrs from onset
Must have CONFIRMED proximal circulation occlusion
When should you consider thrombectomy +/- thrombolysis for posterior circulation strokes?
If known to be well in the last 24hrs and proximal circulation occlusion
What should you do if you suspect a diagnosis of Parkinson’s disease?
Urgent neurology referral
All treatment should be initiated by specialist
What degree of stenosis is required for a carotid endarterectomy?
> 50%
How long is L-dopa usually effective for?
2yrs max
What are the side effects of L-dopa? (7)
Dyskinesia On-off effect Postural hypotension Arrhythmias N&V Psychosis Red urine of standing
What is complex regional pain syndrome?
Progressive pain in area which is disproportionate to original injury
What are some features of complex regional pain syndrome? (6)
Progressive pain Allodynia Temperature and skin changes Oedema Sweating Motor dysfunction
What scoring system is used to diagnose complex regional pain syndrome?
Budapest Diagnostic Criteria
What is the management of complex regional pain syndrome?
Early physio and pain team input
Gabapentin/amitriptyline are 1st line
Who is at an increased risk of Bell’s palsy?
Pregnant women - 3x increased risk
What are the two types of Multiple System Atrophy?
Predominantly Parkinsonian
Predominantly cerebellar
What are the features of Multiple System Atrophy?
Parkinsonism
Autonomic disturbance eg. erectile dysfunction, postural hypotension, atonic bladder
Cerebellar signs - DANISH
Who gets idiopathic intracranial hypertension?
Fat pregnant females
What are the features of idiopathic intracranial hypertension?
Headache + blurred vision
Papilloedema
Enlarged blind spot
Occasionally CN6 palsy
What is the management of idiopathic intracranial hypertension?
Weight loss = 1st line
Acetazolamide
Topiramate = 2nd line, may aid weight loss
Repeated LPs if these unsuccessful
What are the features of an acoustic neuroma?
CN8 = unilateral sensorineural hearing loss, tinnitus, vertigo CN5 = absent corneal reflex CN7 = facial palsy
What is cataplexy?
Strong emotions cause transient loss of muscle tone
Eg. laughing -> fall
2/3 people with narcolepsy have cataplexy
What is the triad of symptoms for normal pressure hydrocephalus?
Urinary incontinence
Dementia and bradyphrenia
Gait abnormalities
What would imaging show in normal pressure hydrocephalus?
Hydrocephalus with enlarged 4ht ventricle
What is the management of normal pressure hydrocephalus?
Ventriculoperitoneal shunting
What are pulling clothes, lip-smacking, aura and deja vu all features of?
Temporal lobe epilepsy
What can the acute withdrawal of L-dopa precipitate?
Neuroleptic malignant syndrome
What are the features of absence seizures?
Last few seconds, quick recovery
Child often unaware they have had one
May be precipitated by hyperventilation
Who gets absence seizures?
Onset generally 3-10yrs
90% seizure-free in teens
What is the characteristic EEG feature of absence seizure?
Bilateral symmetrical 3Hz spike and wave pattern
What is the management of absence seizures?
Sodium valproate or Ethosuximidie
What is a common feature of migraines in children but not adults?
GI symptoms
What is riluzole?
Glutamate receptor antagonist
Prolongs life by about 3m in MND
Used mainly in ALS
What symptoms does olivopontinecerebellar atrophy cause?
Parkinsonism and cerebellar signs
What symptoms does Progressive Supranuclear Palsy cause?
Parkinsonism and ophthalmoplegia
What symptoms does Lewy body dementia cause?
Parkinsonism and visual hallucinations
What are symptoms of autonomic dysreflexia?
Sever hypertension, flushing and sweating WITHOUT HR increase
Due to spinal cord injury above T6
What is Hoffman’s sign?
Flicking the distal phalanx of the patient’s middle finger causes an exaggerated thumb flexion
+ve suggests DCM or MS
Why the COCP contraindicated in those with Hx of migraine with aura?
Significantly increases risk of ischaemic stroke
If a headache is worse on coughing and lying, what does that suggest?
Raised ICP
Need head CT
What 4 medications are 1st line treatments of neuropathic pain?
Amitriptyline
Duloxetine
Gabapentin
Pregabalin