Neuro Flashcards
Brown-Sequard Syndrome - pathophysiology and features
Hemi-cord lesion
Features
- Ipsilateralloss of proprioception and vibration
- Ipsilateral UMN weakness
- Contralateral loss of pain
Cerebellar signs
DANISH
Dysdiadokokinesia
Ataxia
Nystagmus (horizontal - ipsilateral hemisphere)
Intention tremor
Speech (slurred, staccato, scanning dysarthria- words are broken up into separate syllables)
Hypotonia
Causes of cerebellar syndrome
PASTRIES
Paraneoplastic (bronchial Ca) Alcohol (B12 and thiamine deficiency) Sclerosis Tumour Rare (Friedrich's, Alaxia Telangiectasia) Iatrogenic (Phenytoin) Endo (hypothyroidism) Stroke (vertebrobasilar)
Lateral Medullary syndrome
Alternate name, pathophysiology and features
Wallenberg’s syndrome
Patho: Occulusion of one vertebral artery
Features (DANVAH):
- Dysphagia
- Ataxia
- Nystagmus
- Vertigo
- Anaesthesia (ipsilateral facial numbness, contralateral pain loss)
- Horner’s syndrome
Beck’s syndrome
Cause, Pathophys, Features
Cause: aortic aneurysm dissection or repair
Pathophys: Infarction of the spinal cord in the distribution of the anterior spinal artery (ventral 2/3rd of the spinal cord)
Features:
- Para or quadriparesis
- Loss of pain and temperature
- Preserved touch and proprioception
Scissoring gait - area affected
Bilateral UMN lesion
Differentials of Parkinsonism
Parkinson’s disease
Multiple system atrophy
Lewy body dementia
Progressive supranuclear palsy
Vertigo causes
IMBALANCE
Infection/injury (labyrinthitis, Ramsay Hunt, trauma to petrous temporal bone) Meniere's disease BPPV Aminoglycosides Lymph Arterial (stroke/TIA, migraine) Nerve (acoustic neuroma/vestibular schwannoma) Central lesions (demyelination, tumour) Epilepsy (complex partial)
Commonest cause of unilateral sensorineural hearing loss
Acoustic neuroma
5 types of tremor
RAPID
Resting (Parkinsonism)
Action/Postural (Absent at rest, worse with outstretched hands or movement)
Intention (Cerebellar)
Dystonic (idiopathic)
Features of temporal arteritis
Unilateral temple/scalp pain and tenderness
Sudden blindness
Thickened, pulseless temporal artery
Associated with polymyalgia rheumatica in 50%
Migraine triggers
Chocolate Cheese OCP Caffeine Alcohol Anxiety Travel Exercise
Migraine treatment
Always give an anti-emetic (metoclopramide) as an adjunct
Mild-moderate:
1st - NSAID or aspirin
2nd - paracetamol
3rd - paracetamol+aspirin+caffeine
Severe:
1st - triptan+anti-emetic+NSAID
2nd - Ergot alkaloid (ergotamine)
3rd - corticosteroids
Causes of subarachnoid haemorrhage
Berry aneurysm rupture (80%) Arteriovenous malformations (15%)
First investigation of SAH
CT Head
If CT negative, do an LP >12 hours after start of headache
Drug given with SAH to reduce risk of vasospasm
Nimodipine (CCB)
Started on admission to reduce risk of poor outcome and secondary ischaemia
Bamford Classification of Strokes
And criteria for each type
TACS - all 3 of:
- Homonymous hemianopia
- Unilateral motor/sensory deficit
- Higher cortical dysfunction (speech/hemispatial neglect)
PACS - 2/3 of:
- Homonymous hemianopia
- Unilateral motor/sensory deficit
- Higher cortical dysfunction (speech/hemispatial neglect)
POCS - one of:
- homonymous hemianopia with macular sparing)
- cerebellar syndrome
LACS:
- Pure motor
- Pure sensory
- Mixed sensorimotor
- Dysarthria/clumsy hand
- Ataxic hemiparesis
Millard-Gubler syndrome
Patho and features
Pontine infarct
6th and 7th nerves affected
- Diplopia
- LMN facial palsy
- Loss of corneal reflex
- Contralateral hemiplegia
Immediate medical management of ischaemic stroke
- tPA if <4.5 hours before onset of symptoms and no contraindications
- Aspirin 300mg PO
Secondary prevention of stroke
Aspirin/clopidogrel 300mg for 2 weeks
Then clopidogrel 75mg after
Warfarin instead of asp/clop if cardioembolic stroke or chronic AF
Main cause of TIA
Atherothromboembolism from carotids
Secondary prevention of TIA
Same as for stroke
Aspirin/clopidogrel 300mg for 2 weeks
Then clopidogrel 75mg after
Warfarin instead of asp/clop if cardioembolic TIA or chronic AF
Indications for carotid endarterectomy following TIA/stroke
> 70% unilateral disease
Surgery should be performed within 2 weeks
Stroke risk calculation following TIA
ABCD2 Score (/7)
Age >60 BP > 140/90 Clinical features - Unilateral weakness (2 points) - Speech disturbance Duration - >1 hour (2 points) - 10 mins - 1 hour (1 point) Diabetes
Subdural haematoma patho and main cause
Bleeding from bridging veins between cortex and sinuses
Often due to old injuries, such as deceleration injuries
Subdural haematoma on MRI head
Crescentic haematoma over one hemisphere
Possible midline shift
Indications and type of surgery for subdural haematoma
Indications for surgery:
- Bleed >10mm size
- Midline shift >5mm
- Expanding bleed
- Neurological symptoms/signs
Surgery - 2 burr hole craniostomy to remove clot and irrigate
Hemicraniectomy if swelling
Extradural haematoma on MRI head
Lemon
Eponymous signs of meningism
These signs are more commonly seen in children
Kernig’s sign - With the patient supine and the thigh flexed to a 90° right angle, attempts to straighten or extend the leg are met with resistance
Brudzinski’s sign - Flexion of the neck causes involuntary flexion of knees and hips
Medical management of bacterial meningitis
Ceftriaxone 2g BD IV
(+ampicillin 2g BD IV if >50 years)
Dexamethasone IV QDS
Rifampicin prophylaxis for housemates and partners
Contraindications of LP
Thrombocytopenia or coagulopathy Delaying antibiotics Raised ICP Unstable (cardio and respiratory symptoms) Infection at LP site Focal neurology symptoms
Encephalitis
Main causes
HSV 1 and 2
EBV
CMV
Encephalitis
Investigations
CT - bilateral temporal involvement suggests HSV
Bloods - cultures, viral PCR
LP - increased protein, lymphocytes, PCR
Encephalitis
Management
Aciclovir 10mg/kg/8h IV infusion 14 days
Causes of seizure
2/3 epilepsy is idiopathic
Congenital (NF, Tuberous Sclerosis, TORCH)
Acquired (CVA, SOL)
Other (Withdrawal alcohol/opiates/benzos, metabolic glucose/Na/Ca/urea, infection meningitis, encephalitis)
5 As of complex partial seizures
Aura
Autonomic (change in skin colour/temperature)
Awareness lost (blank stare)
Automatisms (lap smacking, fumbling, chewing)
Amnesia
Petit mal (absence seizure) Features
Abrupt onset and offset
Glazed, blank stare
Lasts <10 seconds
Normal examination
Atonic/akinetic seizure
Sudden loss of muscle tone
No LOC
Driving rules after diagnosis of epilepsy
Cannot drive for 1 year after seizure
Cannot drive HGV for 10 years
Principles of diagnosing epilepsy
- Provoked vs unprovoked
- Generalised vs focal (MRI and EEG)
- Subcategorise (eg absence, myoclonic, tonic clonic)
Anti-epileptics
Sodium valproate (avoid in pregnancy, also enzyme inhibitor) Lamotrigine Carbamazepine (enzyme inducer) Levetiracetam Phenytoin (enzyme inducer)
GCS
Eyes (/4): 4 spontaneously open 3 open to voice 2 open to pain 1 nothing
Speech (/5): 5 spontaneous 4 confused 3 inappropriate speech 2 vocalisations 1 nothing
Motor (/6): 6 obeys commands 5 localises to pain 4 withdraws from pain 3 flexes to pain 2 extends to pain 1 nothing
Indications for CT head in trauma
NICE ‘Head injury’ guideline 2014
CT head within 1 hour:
- GCS <13 on initial assessment
- GCS <15 at 2 hours after injury
- Suspected open or depressed skull fracture
- Signs of basal skull fracture
- Post-traumatic seizure
- Focal neurological deficit
- More than one episode of vomiting since head injury
CT head within 8 hours:
- Warfarin
- LOC or amnesia + 1 of the following:
- Age >65
- Coagulopathy
- Dangerous mechanism
of injury
Causes of SOL
Vascular
- aneurysm
- AVM
- chronic subdura haematoma
Infective
- TB
- Cerebral abscess
- Cyst
Neoplasm
- Primary
- Metastasis
Granuloma
- TB
- Sarcoid
Idiopathic intracranial hypertension
Features and management
Features (similar to SOL)
- Papilloedema
- Headache
- 6th nerve palsy
- Visual disturbance
Management
- Weight loss
- Acetazolamide to decrease ICP
- Furosemide
- Lumbar-peritoneal shunt is persistent/worsening symptoms despite medical management
Cushing’s reflex - features
Hypertension
Bradycardia
Irregular breathing
Tonsillar herniation
Cause
Pathophysiology
Features
Cause: SOL/increased pressure in posterior fossa
Pathophysiology: Herniation of cerebellar tonsils through foramen magnum
Features:
- 6th nerve palsy
- Upgoing plantars
- Irregular breathing and apnoea
Transtentorial/Uncal herniation
Cause
Features
Cause: Lateral supratentorial mass compressing the temporal lobe
Features:
- 3rd nerve palsy (mydriasis and down&out)
- Contralateral hemiparesis (compression of ipsilateral corticospinal tract)
Parkinson’s-plus syndromes
Additional features
- Multiple system atrophy
- Autonomic (postural HoTN, bladder dysfunction)
- Cerebellar and pyramidal signs
- Rigidity > tremor - Progressive supranuclear palsy
- Postural instability (falls)
- Gaze palsies - Corticobasilar degeneration
- Aphasia, dysarthria, apraxia
- Alien limb phenomenon - Lewy Body Dementia
- Fluctuating cognition
- Visual hallucinations
Parkinsonism features
TRAPPS PD
Tremor (4-7Hz)
Rigidity
Akinesia
Postural instability
Postural hypotension and other autonomic dysfunction
Sleep disorders (insomnia and frequent waking)
Psychosis (visual hallucinations)
Depression/dementia
Autonomic dysfunction in Parkinson’s
Postural hypotension Constipation Urinary symptoms (urgency, frequency, nocturia, incontinence) Hypersalivation - dribbling Hyperhidrosis Erectile dysfunction
Diagnosis of Parkinson’s
Diagnosis is clinical
Investigations are not needed for diagnosis
- Can be used in <40s to exclude Wilson’s disease
Dopamine agonists used in Parkinson’s
2 examples
Ropinirole
Pramipexole
MOA-B inhibitors used in Parkinson’s
2 examples
Rasagiline
Selegiline
MS
Pathophysiology
CD4-mediated destruction of oligodendrocytes, leading to demyelination and neuronal death
Lhermitte’s sign in MS
Neck flexion produces electric shock-like sensation in the trunk and limbs
Optic neuritis
Symptoms and signs on fundoscopy
Symptoms:
- Pain on eye movement
- Rapid loss of central vision
Fundoscopy:
- Reduced acuity
- Loss of colour vision, red initially
- White optic disc
- Central scotoma
Best investigation for MS
MRI, particularly gadolinium-enhancing
Lesions are typically present in periventricular white matter
Diagnosis of MS
Clinical diagnosis by demonstration of focal neurological events separated in space and time
Confirmed by MRI and potentially CSF
Devic’s syndrome
An MS variant distinguished by presence of NMO-IgG antibodies
Management of acute attack of MS
Methylprednisolone 1g IV OD for 3 days
Long-term management of MS
Interferon-beta
Glatiramer
Biologics:
- Alemtuzumab
- Natalizumab
+Symptomatic treatment
Cord compression - features
At level of the lesion:
- Radicular pain in the dermatomal distribution
- LMN signs
Below of the lesion:
- UMN weakness and sensory loss
Other features:
- Painless bladder retention (neuropathic bladder)
- Faecal incontinence
Cord compression - causes
Trauma Infection - abscess, TB Secondary malignancy - breast, thyroid, bronchial, renal, prostate Primary cord tumour Haematoma (warfarin)
Cauda equina - features
Saddle anaesthesia Urinary/faecal incontinence Back pain Radicular pain down legs Bilateral flaccid, arreflexic lower limb weakness Poor anal tone
Hoffmann reflex
Flick to the middle finger pulp causes a brief pincer flexion of the thumb and index finger
https://www.youtube.com/watch?v=MUffMh-paJc
Investigation of choice for cervical spondylosis
Cervical MRI
Cervical spondylosis - features
Motor and sensory disturbance according to the level of compression
Most commonly C7:
- Motor weakness of triceps, finger extension
- Numb middle finger
Severe UC flare
according to Truelove and Witts’ severity index
Patient has bloody stool or >6 stools per day plus at least one of:
- Temperature >37.8
- HR >90 bpm
- Anaemia (Hb >105)
- ESR >30
Nerve root affected in foot drop
L5
Can be differentiated as the cause of foot drop (rather than peroneal nerve palsy) if the patient also has weak foot inversion
Nerve foot affected with weak plantarflexion
S1
Spinal stenosis - clinical features
Spinal claudication
- Aching/heavy buttock and lower limb pain on walking
- Rapid onset
- Pain eased by leaning forward
Management of cauda equina
Emergency decompressive laminectomy within 48 hours of onset of symptoms
Most likely cause of Bell’s Palsy
HSV-1
Bell’s Palsy - clinical features
- Complete unilateral facial weakness within 24-72 hours
- Hyperacusis (stapedius palsy)
- Drooling, speech difficulty
Bell’s Palsy - diagnosis
Clinical diagnosis
Bell’s Palsy - treatment
Prednisolone
- Given within 72 hours of symptom onset
- 60mg PO for 5 days, then tapered
Ramsay Hunt syndrome - pathophysiology
VZV reactivation in the geniculate ganglion of 7th nerve
Ramsay Hunt syndrome - clinical features
- Preceding ear pain
- Unilateral complete facial weakness
- Vesicular rash in the ear
- May also affected CN8 (vertigo, hearing loss, tinnitus)
Most common cause of mononeuritis multiplex
Diabetes
Median nerve neuropathy
Nerve roots
Causes
Motor and sensory features
Nerve roots:
- C6-T1
Causes:
- Carpal tunnel
- Wrist trauma
Motor:
- LOAF muscles (flexor pollicis brevis, opponens pollicis, abductor pollicis brevis, lateral two lumbricals)
- Thenar wasting
Sensory:
- Numbness of radial 3/5 fingers and palm
- Aching pain
- Tinel’s and Phalen’s positive
Ulnar nerve neuropathy
Nerve roots
Causes
Motor and sensory features
Nerve roots:
- C7-T1
Causes:
- Elbow trauma (supracondylar fracture)
Motor:
- Claw hand
- Hypothenar wasting
- Froment’s positive (tests adductor pollicis - patient hold paper between and thumb and index finger, then paper is pulled out by examiner)
Sensory:
- Ulnar 1.5 fingers
Radial nerve neuropathy
Nerve roots
Causes
Motor and sensory features
Nerve roots:
- C5-T1
Causes:
- Humeral fracture
Motor:
- Finger/wrist drop
- If trauma is high up - triceps paralysis
Sensory - Snuff box
Sciatic nerve neuropathy
Nerve roots
Causes
Motor and sensory features
Nerve roots:
- L4-S3
Causes:
- Pelvic/femoral fracture
Motor:
- Hamstrings and all muscles below the knee
Sensory:
- Below knee laterally and foot
Common peroneal
Nerve roots
Causes
Motor and sensory features
Nerve roots:
- L4-S1
Causes:
- Fibular head fracture
Motor:
- Foot drop
- Weak ankle dorsiflexion and eversion (intact inversion)
Sensory:
- Below knee laterally
Tibial nerve neuropathy
Nerve roots
Motor and sensory features
Nerve roots:
- L4-S3
Motor:
- Loss of plantarflexion
- Foot inversion
- Toe flexion
Sensory:
- Sole of foot
Miller-Fisher syndrome
Variant of GBS
Triad of:
- Ataxia
- Areflexia
- Ophthalmoplegia (acute onset of external ophthalmoplegia is a cardinal feature)
GBS - management
IVIg/Plasma exchange
Support (4As)
- Airway/ventilation (may need ITU admission if FVC <1.5L)
- Analgesia
- Autonomic (inotropes)
- Antithrombotic (TEDS stockings, LMWH)
GBS - clinical features
2/3 have preceding URTI or gastroenteritis within 6 weeks before onset of neuro symptoms
Progressive symmetrical muscle weakness (flaccid paralysis)
- Lower limbs before upper extremities
- Proximal muscles before distal muscles
Paraesthesia of hands and feet
Areflexia
GBS - initial investigations
LP
- Exclude infectious causes
- CSF protein with normal cell count is the classic finding
Spirometry
- Performed every 6 hours initially
- FVC <20mL/kg indicates ITU admission
Neurophysiological evaluation
- Nerve conduction studies
Serology and stool culture
- To try to identify a cause (CMV, EBV, C jejuni)
Charcot-Marie-Tooth syndrome
Group of inherited motor and sensory neuropathies
Onset in puberty
Motor:
- High stepping gait
- Symmetrical muscle atrophy (champagne bottle legs)
- Pes cavus, toe clawing
Sensory:
- Loss of sensation in stocking distribution
Initial investigation - nerve conduction studies
Charcot-Marie-Tooth syndrome
Management
Physiotherapy and exercise
Occupational therapy
Bracing/orthopaedic surgery for foot deformities
Bulbar Palsy
Cranial nerves affected
Clinical features
CN 9-12
Lower motor signs in the tongue, talking and swallowing
Clinical features:
- Tongue fasciculations, flaccid
- Quiet/nasal speech
- Absent jaw jerk reflex and gag reflex
Bulbar palsy vs pseudobulbar palsy
Bulbar is LMN, pseudobulbar is UMN
Pseudobulbar palsy is more common
Tongue:
- Bulbar - fasciculations, flaccid
- Pseudobulbar - spastic, slow movements
Speech:
- Bulbar - quiet/nasal speech
- Pseudobulbar - slow speech (hot-potato speech)
Jaw jerk reflex:
- Bulbar - normal/absent
- Pseudobulbar - brisk
Motor neurone disease - classification
ALS
- Cortex and anterior horn
- UMN and LMN
- Asymmetrical limb involvement is the most common presentation
Progressive bulbar palsy
- CN 9-12
- LMN
Progressive muscular atrophy
- Anterior horn
- LMN only
Distal to proximal progression
Primary lateral sclerosis
- Motor cortex
- Mainly UMN
- Spastic leg weakness and pseudobulbar palsy
ALS - clinical features
UMN signs:
- Weakness
- Spasticity
- Hyperreflexia
- Upgoing plantar
LMN signs:
- Weakness
- Atrophy
- Fasciculations
Never:
- Sensory
- Sphincter
- Eye movement
ALS - role of investigations
ALS is a clinical diagnosis
Investigations are used to exclude differentials and detect LMN disease in limbs that are clinical unaffected
Exclude differentials
- Imaging (area of spine with suspected lesions, brain if bulbar signs)
- Nerve conduction and blood tests if peripheral nerve signs
Asses limb involvement is asymptomatic limbs
- EMG
Duchenne muscular dystrophy - inheritance
X-linked recessive
30% are spontaneous
Becker muscular dystrophy s Duchenne
Becker presents later, is less severe and has better prognosis
Both are X-linked recessive
Myasthenia gravis - initial investigations
Antibodies:
- Anti-AChR
- Muscle-specific tyrosine kinase (MuSK) antibodies
Serial FVC and NIF - if suspected MG crisis
EMG
CT chest - performed at diagnosis to detect thymoma (15%) or thymic hyperplasia (75%)
Lambert Eaton vs Mysasthenia Gravis
Lambert Eaton:
- Leg weakness before ocular involvement
- Autonomic dysfunction (dry mouth is most common/metallic taste)
- Areflexia
Neurofibromatosis type 1
Inheritance
Clinical features
Von Recklinghausen’s disease
Inheritance: Autosomal dominant
Clinical features:
- Cafe-au-lait spots
- Freckling (axillary)
- Neurofibromas
- Lisch nodules (small hamartomatous nodules in the iris)
- Neoplasia (CNS, phaeo, AML)
- Epilepsy
- Renal artery stenosis
Cafe-au-lait spots
Causes
Neurofibromatosis (I and II)
McCune-Albright
Multiple Lentigenes
Urticaria Pigmentosa
Neurofibromatosis type 2
Inheritance
Clinical features
Inheritance:
- Autosomal dominant (50% de novo)
Clinical features:
- Bilateral acoustic schwannomas are characteristic (first sensorineural hearing loss, the tinnitus, then vertigo)
- Cafe-au-lait - typically fewer than in NF1
- Bilateral cataracts
Syringomyelia
Pathophysiology
Clinical features
Tubular cavity in the central canal of the cervical cord
Clinical features:
- Sensory loss (loss of pain/temperature, preserved touch/proprioception/vibration)
- Hand wasting (claw hand)
- Absent upper limb reflexes
- Charcot joints (shoulder, elbow)
Friedrich’s ataxia
Clinical features
Mitochondrial disease
- Pes cavus and scoliosis
- Bilateral cerebellar signs
- Leg wasting and areflexia
- Optic atrophy
- HOCM
- DM - hyperglycaemia
Cluster headache
Principles of management
100% oxygen
Subcutaneous triptan
Verapamil is used for prophylaxis
Migraine
Principles of management
Acute management:
- Triptan + NSAID/paracetamol
Prophylaxis:
- Topiramate or propranolol
Epilepsy
Recommended diet
Ketogenic diet
Venous sinus thrombosis
Gold standard investigation
MR Venogram
Degenerative cervical myelopathy
Treatment
Cervical decompressive surgery
Restless leg syndrome
Management
Dopamine agonists, eg ropinirole
Reflexes and roots
Ankle S1-2
Knee L3-4
Biceps C5-6
Triceps C7-8
Trigeminal neuralgia
First-line treatment
Carbamazepine
Cavernous sinus syndrome
Clinical features
Clinical features:
- Signs of pain
- Ophthalmoplegia
- Proptosis
- Trigeminal nerve lesion
- Horner’s syndrome
Subacute combined degeneration of the cord
Clinical features
Typically develops over months
- Ataxic gait (dorsal columns)
- Mixed UMN and LMN signs (lateral motor tracts and peripheral nerves)
