Neuro Flashcards
How can the lesions in MS be described?
Disseminated in space and time
What is Lhermitte’s sign and in which disease is it seen?
Seen in MS. It is when electric shock-like pain occurs in trunk and limbs when the neck is flexed
What is the mean age of onset of MS?
30
What kind of symptoms occur with MS?
Sensory- pins and needles, Lhermitte’s sign, trigeninal neuralgia, reduced vibration sense
Motor: myelitis, spastic weakness
Sexual/GU: erectile dysfunction, anorgasmia, urine retention
GI: constipation, swallowing disorder
Cerebellum: ataxia, intention tremor, monotonous speech
Eye: diplopia, hemianopia, INO, optic neuritis, pupil defects
Cognitive decline
What is the pathogenesis of MS?
Autoimmune disease attacking myelinated axons
What worsens MS symptoms?
Heat/exercise
What is the lifetime risk of MS in the UK?
1:1000
How is MS diagnosed?
Clinical diagnosis:
Past episodes e.g. Brief unexplained vision loss
Attacks lasting more than 1 hr with more than 30 days between attacks
What is seen in the CSF of MS patients
Oliclonal bands of IgG in >90% of patients
What can be used to identify a demyelinating event?
Evoked potentials
What are the differential diagnoses for MS?
Spinal cord neoplasms, subacute combined degeneration of the cord, transverse myelitis, progressive multifocal leukoencephalitis
What is the treatment for MS?
Steroids (for acute attacks) DMDs (to reduce number of relapses): -interferon alpha and beta -monoclonal antibodies (alemtuzumab and Natalizumab) -azathioprine -glatiramer acetate Treatment of spasticity- baclofen, gabapentin Treatment of tremor-Botox
What is the definition of a relapse?
Appearance of new symptoms or the return of old symptoms for a period of 24 hours or more in the absence of a change in core body temperature or infection
What are the 4 types of MS?
Relapsing-remitting
Secondary progressive
Primary progressive
Progressive-relapsing
What is CJD caused by
Prions- midfielder forms of normal proteins which can transform other proteins into prion proteins
What kind of changes do prion proteins cause in the brain?
Spongiform changes- cavities and tubulivesicular structures in the brain
What are the 2 types of CJD
Variant CJD- transmitted in BSE contaminated beef
Classic CJD- occurs spordically due to mutation
What is the mean age of onset for classic CJD and variant CJD?
Classic: 68
Variant: 28
What is the mean duration of illness for classic and variant CJD?
Classic: 4-5 months
Variant: 13-14 months
What are the symptoms of CJD?
Progressive dementia Focal neuro signs Myoclonus Depression Eye signs
What is Guillain-Barré syndrome?
Acute inflammatory demyelinating neuropathy
What are the triggers for Guillain-Barré syndrome?
Campylobacter jejuni, CMV, mycoplasma, HZV, HIV, EBV, vaccinations
What is the treatment for Guillain-Barré?
Plasma exchange
IV immunoglobulin
Supportive therapy
What are the symptoms of Guillain-Barré?
Acute ascending paralysis with glove-and-stocking weakness
Progressive weakness of all 4 limbs (days- weeks)
Arreflexia
CN palsies
Paresthesiae
Autonomic dysfunction (sweating, ⬆️pulse)
What is the prognosis for Guillain-Barré?
85% make a full recovery
10% unable to walk at 1 year
Differential diagnosis for Guillain-Barré?
Acute myelopathy
Chronic inflammatory demyelinating polyneuropathy
HIV peripheral neuropathy
Spinal cord compression
Signs and symptoms of spinal cord compression
Leg weakness Sensory loss Spinal/root pain may precede this Urine/faecal incontinence LMN signs at level UMN signs below level (but tone and reflexes reduced in acute cord compression)
What are the main causes of spinal cord compression?
- Vertebral body neoplasms
- Disc and vertebral lesions (trauma, degenerative disease)
- Inflammatory (epidural abscess, TB)
- Spinal cord neoplasms
- Rarities- Paget’s disease, epidural haemorrhage , bone cysts
Differential diagnoses for cord compression
Transverse myelitis MS carcinomatous meningitis Cord vasculitis (syphillis) Trauma Guillain-Barré Dissecting aneurism
What is the most common cause of cauda equina syndrome?
Lumbar disc prolapse at L4/L5 and L5/S1
Signs and symptoms of cauda equina lesion
Bladder and bowel dysfunction Mixed UMN/LMN leg weakness- ASYMMETRICAL Saddle numbness Back pain Erectile dysfunction Arreflexia
What are the causes of foot drop?
Diabetes Common peroneal nerve palsy Stroke Prolapsed disc MS
What causes leg weakness with no sensory loss?
Motor neurone disease
Polio
Parasaggital meningioma
What causes chronic spastic paraparesis?
MS Cord tumour Motor neurone disease SCDC Syringomyelia
What causes chronic flaccid paraparesis?
Peripheral neuropathy
Myopathy
What causes absent knee jerks and extensor plantars?
Motor neurone disease/myeloradiculitis
Friedrich’s ataxia
SCDC
Tabes dorsalis
What are the differential diagnoses for blackouts?
- Epilepsy
- Postural hypotension
- Syncope
- Cardiac causes
What is syringomyelia?
Fluid-filled cavity in the spinal cord
What is syringobulbia?
Fluid-filled cavity in the brainstem
What is the most common cause of syringomyelia and syringobulbia?
Blockage of CSF flow from the 4th ventricle (due to Arnold-Chiari malformation)
Other causes: SAH, post infection, masses, post trauma, post AVM rupture
What is an Arnold-Chiari malformation?
Congenital herniation of the cerebellar tonsils through the foramen magnum
What is the normal presentation of syringobulbia or syringomyelia?
Presentation in 3rd/4th decade of life with pain and sensory loss (pain+temperature only) in the upper limbs and upper trunk only
Damage to pyramidal tracts, anterior horn cells and medulla cause wasting in the hands and claw hand
Spastic paraplegia
nystagmus
Horner’s syndrome
Charcot’s joints
Syringobulbia: nystagmus, tongue atrophy, pharyngeal/palatal weakness, facial sensory loss
What is the treatment for syringomyelia and syringobulbia?
Decompression of the foramen magnum
What is Friedreich’s ataxia?
Progressive degeneration of the cerebellum and spinocerebellar tracts
–> cerebellar ataxia, dysarthria, nystagmus
Tendon reflexes absent (peripheral n. damage)
Loss of dorsal columns–> absent joint position and vibration sense
Loss of corticospinal tract–> weakness and up going plantar
Pes cavus
Optic atrophy
Death by middle age
What is motor neurone disease?
Destruction of UMNs and anterior horn cells in brain and spinal cord
What is the life expectancy for MND and what do patients die of?
3 years- die of resp failure due to bulbar palsy and pneumonia
Is MND more common in men or women? Is it familial?
Men
Most cases sporadic (not familial)
Familial cases can be due to mutations in SOD-1 (responsible for clearing up free radicals)
What are the 4 clinical patterns of MND?
- Progressive muscular atrophy
- Amyotrophic lateral sclerosis
- Progressive bulbar and pseudobulbar palsy
- Primary lateral sclerosis
What is progressive muscular atrophy?
LMN lesion of the cord
Hands and arms- weakness, wasting, fasciculation
What is amyotrophic lateral sclerosis?
Disease of lateral corticospinal tracts and anterior horn cells
Progressive spastic tetra paresis/paraparesis
LMN signs
What is progressive bulbar and pseudobulbar palsy?
Destruction of UMN (pseudobulbar palsy) and LMN (bulbar palsy) in cranial nerves
Dysarthria+dysphagia
Wasting and fasciculation of tongue
What is primary lateral sclerosis?
Affects corticospinal tract in arms, legs and face
Progressive tetra paresis
What symptom is characteristic of MND?
Fasciculation
Weakness in arms and legs
What’s the differential diagnosis for MND?
Cervical spine lesion
What is the management of MND?
Riluzole- Na channel blocker that inhibits glutamate release (slows disease progression)
Ventilator support
PEG feeding
What is spinal muscular atrophy?
Group of rare disorders
Anterior horn cells destroyed
Symmetrical wasting and weakness of limbs
What percentage of over-65s and over-80s are affected by dementia?
10% of over-65s
20% of over-80s
What are the most common dementias?
Alzheimer’s (65% of all dementias)
Fronto-temporal dementia
Vascular dementia
Dementia with Lewy bodies
What is the disease progression and symptoms of Alzheimer’s?
Progresses over years Short term memory loss Disintegration of personality and intellect Difficulty with language Apraxia Agnosia Visuospatial skills
What is the pathophysiology of Alzheimer’s?
Neurofibrillary tangles
Amyloid plaques
Which investigations are done in dementia?
MMSE FBC LFTs TFTs Measure B12 And folate Brain CT
Management of Alzheimer’s?
AChE inhibitors- rivastigmine, donepezil, galantamine
What is the prognosis for Alzheimer’s?
Average survival= 8-10 years
What is the progression of vascular dementia?
Stepwise deterioration
Hx of TIAs or strokes
What is the disease course of dementia with Lewy bodies?
Fluctuating cognition with profound variation in attention and alertness
Memory loss may not occur in early stages
Impaired attention and visuospatial ability
Visual hallucinations
Parkinsonism
Depression and sleep disorders
What are the general causes of mononeuropathy?
Compression
Entrapment
Direct damage
What is carpal tunnel syndrome?
Entrapment neuropathy
Median nerve is compressed as it passes through carpal tunnel
What’s the cause of carpal tunnel?
Mostly idiopathic
Associated with: hypothyroidism, diabetes, pregnancy, obesity, RA, acromegaly
What are the features of carpal tunnel?
Pain and parenthesiae in the hand
Worse at night
Hanging the hand out of the bed and shaking it out can relieve pain
Tinnel’s sign: tapping on carpal tunnel is painful
Weakness+wasting of the bar muscles
Sensory loss- palm and palmar aspects of radial 3.5 fingers
Treatment for carpal tunnel?
Nocturnal splints
Local steroid injections
Surgical decompression
What is mononeuritis multiplex
Multiple mononeuropathy
What are the causes of mononeuritis multiplex?
Indicates systemic disease: Diabetes Leprosy Vasculitis Sarcoidosis Anyloidosis Malignancy Neurofibromatosis HIV Guillain-Barré
What are the differential diagnoses for Parkinson’s disease?
Drug-induced Parkinsonism (prochlorperazine)
Parkinson-plus syndromes (progressive supra nuclear palsy and multiple system atrophy)
Dementia with Lewy bodies
Post-encephalitic Parkinson’s
Normal pressure hydrocephalus
Vascular Parkinson’s
What kind of symptoms may occur in a poly neuropathy?
Sensory- paraesthesiae, numbness ‘glove and stocking’ distribution, pain in extremities, unsteadiness
Motor- weakness
Autonomic- postural hypotension, urinary retention, erectile dysfunction, diarrhoea, ⬇️sweating, cardiac arrhythmias, impaired pupillary responses
Name some causes of poly neuropathy
Guillain-Barré Drugs e.g. Isoniazid, phenytoin Diabetes Thyroid disease Amyloidosis Vitamin deficiency : B1, B12, B6 Alcohol, lead, arsenic Chronic inflamm. demyelinating polyn.
Which investigations should be done in a patient with polyneuropathy?
FBC ESR Serum B12 BM U&E LFTs
What is Miller Fisher syndrome
Variant of Guillain-Barré which affects ten cranial nerves to the eye
Characterised by ophthalmoplegia and ataxia
Which test is done to confirm diagnosis of Guillain- Barré?
Nerve conduction study
What is the consequence of vitamin B1 (thiamin) deficiency?
Wernicke-Korsakoff syndrome
Polyneuropathy
Cardiac failure
What causes thiamin deficiency?
Alcholism, beriberi, starvation
What is the consequence of vitamin B6 (pyridoxine) deficiency?
Sensory neuropathy
What can cause vitamin B6 deficiency?
Isoniazid therapy
What is the consequence of vitamin B12 deficiency?
Subacute combined degeneration of the cord
Distal sensory loss, absent ankle jerks, exaggerated knee jerks, up going plantars
Give an example of a hereditary sensorimotor neuropathy and explain the symptoms
Charcot-Marie-Tooth (common peroneal nerve palsy)
Progressive distal limb wasting and weakness
Loss of sensation and reflexes in legs
What is the difference in clinical
Presentation between a neuropathy and a muscle disease?
Reflexes and sensation normal in muscle disease but not neuropathy
What are polymyosotis and dermatomyositis?
Acquired muscle diseases
Polymyositis- inflammation and necrosis of skeletal muscle fibres
Dermatomyositis- when skin is involved
Symptoms of Polymyositis and dermatomyositis
Symmetrical progressive muscle weakness and wasting of proximal muscles of shoulder and pelvic girdle
Difficulty rising from a chair and raising hands above head
Dysphagia, dysphonia
Dermatomyositis- purple discolouration of eyelids, erythema over knuckles, arthralgia, dysphagia
How is myosotis treated?
Prednisolone
What condition is associated with myasthenia gravis in 70% of patients under 40?
Thymic hyperplasia
What is the clinical presentation of myasthenia gravis?
Weakness and fatiguability of ocular, bulbar and proximal limb muscles (weakness improves after rest)
Ptosis
Diplopia
Difficulty in talking, chewing, swallowing, breathing
Ask pt to sit with arms outstretched and look for slow downward drift
Which antibodies are specific for myasthenia gravis?
Anti-AChR autoantibodies
Which investigations are done in suspected myasthenia gravis?
Nerve stimulation tests- decrease in evoked potential
CT/MRI of mediastinum to look for thymoma
What is the differential diagnosis for myasthenia gravis
Thyroid ophthalmopathy Myotonic dystrophy Brainstem cranial nerve lesions Botulism Lambert-Eaton myasthenic myopathic syndrome
Treatment for myasthenia gravis
Anticholinesterases
Azathioprine
Plasmapherisis and IV Ig (for myasthenic crisis)
Thymectomy (for thymoma)
What is myasthenic crisis?
When respiratory and bulbar muscles become weak
What is myotonia? What are examples of some myotonic conditions?
Delayed muscle relaxation after contraction (e.g. Difficulty releasing grasp after shaking hands)
Examples: dystrophia myotonica, myotonia congenita
How does Duchenne muscular dystrophy present and what is the disease course?
Presents with weak proximal leg muscles- clumsy walking and difficulty standing
Progresses to other muscle groups- resp failure
Severe disability and death in late teens
What are symptoms of dystrophia myotonica?
Progressive distal muscle weakness with myotonia, ptosis, facial muscle weakness and wasting
What are the symptoms of myotonia congenita?
Mild isolated myotonia accentuates by rest and cold
What are the causes of low CSF opening pressure
CSF leak
Recent lumbar puncture
Which invesitgations are done in syringomyelia?
MRI
What are the causes of high CSF opening pressure?
Meningitis
Tumour
Intracranial haemorrhage
Benign intracranial hypertension
What does cloudy/purulent CSF
signify?
Meningitis
What does bloody CSF signify?
Traumatic tap (hit a blood vessel during lumbar puncture) Subarachnoid haemorrhage
What are the causes of raised CSF protein?
Meningitis Brain abscess Infra cerebral haemorrhage Neoplastic disease Guillain-Barré MS
CSF glucose should be …..% that of the level in the blood
40-60%
What are the signs of type 1 neurofibromatosis?
Cafe ah lair spots
Freckling in skin-folds
Nodular neurofibromas
Lisch nodules
Which dural venous sinus is the most commonly thrombosed?
Sagittal sinus (47% of cases) Transverse sinus (35% of cases)
What are the signs of sagittal sinus thrombosis?
Headache, vomiting, seizures, decreased vision, papilloedema
What are the signs of transverse sinus thrombosis?
Hedache +/- mastoid pain, focal CNS signs, seizures, papilloedema
What are the symptoms of cortical vein thrombosis?
Symptoms: Thunderclap headache, seizures.
Signs: encephalopathy, slowly evolving focal deficits (paresis, speech disorders, decreased cognition, decreased vision)
Usually occurs with sinus thrombosis
Galen thrombosis=rare cause of CVT associated with vascular malformation
What are the differential diagnosis for intracranial venous thrombosis?
SAH, meningitis, encephalitis, intracranial abscess, arterial infarction
Which investigations are done in intracranial venous thrombosis?
CT/MRI venography, LP
What is the treatment for intracranial venous thrombosis?
Heparin
Streptokinase
What are some causes of intracranial venous thrombosis?
Pregnancy, OCP, head injury, dehydration, intracranial malignancy, extracranial malignancy (hypercoagulation), recent LP, hyperthyroidism, meningitis/TB, androgens…
When should you suspect an extradural haemorrhage?
If, after head injury, conscious level falls after a lucid interval
What is the most common cause of extradural haemorrhage?
Fracture of temporal/parietal bone causing laceration of middle meningeal artery
(can also be caused by tearing of dural venous sinuses)
What are the differential diagnoses for extradural haemorrhage?
Epilepsy, Carotid dissection, carbon monoxide poisoning
What are the signs and symptoms of an extradural haemorrhage?
Raised ICP, decreased GCS, headache, vomiting, confusion, seizures, ipsilateral pupil dilatation, bilateral limb weakness, deep and irregular breathing (brainstem compression)
What is pseudobulbar palsy caused by?
Damage to the corticobulbar tracts.
MND, MS, stroke, brainstem disorders, neurosyphilis, head injury.
What are the signs and symptoms of pseudobulbar palsy?
Donald Duck speech, paralysed tongue, exaggerated jaw jerk, dysphonic, dysphagic, facial muscles paralysed
What happens to speech in myasthenia gravis?
Soft, slurred, monotonous speech which gets worse the longer the patient talks.
When should subdural haematoma be suspected?
Fluctuating conscious level, insidious physical/intellectual slowing, sleepiness, headache, personality change, unsteadiness
Risk factors: falls (alcoholics, epileptics), anticoagulation
What are the causes of subdural haematoma?
Bleeding from bridging veins between cortex and venous sinuses
Note: does not have to be trauma!
Intracranial HYPOtension, dural metastases
What is a cause of the following presentation:
- bilateral facial palsy
- uveitis
- parotitis (swollen parotid glands)
- fever
Heerfordt’s syndrome (a presentation of sarcoidosis)
Resolves spontaneously in a few weeks
Can myasthenia gravis and Lyme disease cause bilateral facial palsies?
Yep
What is Ramsay-Hunt syndrome?
Facial palsy associated with HZV
- LMN facial palsy (forehead sparing)
- Pain around ear
- Vesicles in and around external auditory meatus
Give 3 examples of mainly motor polyneuropathies
Guillain-Barre syndrome, Lead poisoning, Charcot-Marie-Tooth syndrome
Give 3 examples of mainly sensory polyneuropathies
Diabetes mellitus, Renal failure, leprosy
What are some causes of autonomic neuropathy?
Sjogren’s syndrome, HIV, SLE, leprosy, Guillain-Barre, paraneoplastic syndrome, diabetes
What are the signs of parasympathetic neuropathy?
erectile dysfunction, nocturnal diarrhoea, urine retention, Holmes-Adie pupil
What are the signs of sympathetic neuropathy?
Ejaculatory failure, postural hypotension, decreased sweating, Horner’s syndrome
What is cervical spondylosis?
Chronic cervical disc degeneration with herniation of disc material, calcification and osteophytic outgrowths
What are the signs and symptoms of cervical spondylosis
Can cause cord compression --> progressive spastic quadraparesis with sensory loss below neck Painful neck movement Neck stiffness Crepitus (cracking) on moving neck Stabbing/dull arm and wrist pain Signs of cord compression: -Spastic leg weakness (often one leg is worse) -weakness and clumsiness in hands -heavy legs -numb hands -foot drop/poor walking -incontinence
What are the signs and symptoms of radiculopathy?
Pain (electrical sensations) in arms or fingers at level of compression
Dull reflexes
Sensory disturbance (numbness, tingling, reduced pain and temp sensation)
LMN effects in muscles innervated by affected root
UMN affected below level
Where is pain referred to in:
a) Trapped nerve at C3-C4
b) Trapped nerve at C5
c) Trapped nerve at C6
d) Trapped nerve at C7
e) Trapped nerve at C8
a) Neck and trapezius pain
b) Shoulder pain and pain in arm to below the elbow
c) Pain worse on wrist flexion
d) Pain down arm into 3rd finger
e) Pain down arm into 4th and 5th fingers
What are the motor + sensory defects associated with:
a) C5 root involvment
b) C6 root involvement
c) C7 root involvement
d) C8 root involvement
a) Weak deltoid+supraspinatus, reduced supinator jerk, numb elbow
b) Weak biceps+brachioradialis, reduced biceps jerk, numb thumb and index finger
c) Weak triceps+finger extension, reduced triceps jerks, numb middle finger
d) Weak finger flexors+small hand muscles, numb 4th and 5th fingers
What are differential diagnoses for cord compression?
MS, nerve root neurofibroma, SCDC, bone/cord tumour, intramedullary spinal sarcoidosis
What are the management options for cervical spondylosis?
Neck collar
Surgical root decompression (laminectomy or laminoplasty)
Transforaminal steroid injection
What is the difference between laminectomy and laminoplasty?
Laminectomy= portion of bone removed Laminoplasty= screws and plates
What are the features of myopathies/muscular dystrophies
Gradual onset of symmetrical proximal weakness (also distal in myotonic dystrophy)
Specific muscle groups affected
Tendon reflexes preserved
No paraesthesiae/bladder problems
What are the symptoms and signs of fascioscapulohumeral dystrophy?
Weakness of face ('ironed out' expression)
Cannot puff out cheeks
Cannot raise arms
Winging of scapula
Foot drop
Scoliosis
Horizontal clavicals
Anterior axillary foldsWhat are the signs and symptoms of Becker’s muscular dystrophy?
Similar to Duchenne’s, but later presentation
What is the inheritance pattern of:
a) Duchenne’s
b) Becker’s
c) fascioscapulohumeral
a) X-linked recessive
b) X-linked recessive
c) autosomal dominant
What do myotonic disorders cause? What is the most common one?
Tonic muscle spasm (myotonia) Most common= dystrophia myotonica (autosomal dominant chloride channelopathy) Signs+symptoms: -hand/foot drop -weak sternocleidomastoids -myotonia -facial weakness -cataracts -male frontal baldness -diabetes -testis/ovary atrophy -cardiomyopathy
Give examples of acqured myopathies (late onset)
Part of systemic disease:
Cushing’s, hyperthyroid, malignancy, hypocalcaemia, hypercalcaemia
Give an example of an inflammatory myopathy
Inclusion body myositis:
- weakness in quads, finger flexors and pharyngeal muscle
- intranuclear inclusions seen in histology
What are the complications of type 1 neurofibromatosis? How common are they?
Occur in 30% of cases
Mild learning disability
Local effects of neurofibromas (nerve root compression, GI bleeds and obstruction, bone lesions, ⬆️BP, sarcoidosis)
Malignancy (in 5% of pts)- optic glioma, sarcomatous change in neurofibroma
What is the management of type 1 neurofibromatosis
Yearly management of BP
Can excise neurofibromas
What is the prevalence of type 1 and type 2 neurofibromatosis?
How are they inherited?
Type 1: 1 in 2500
Type 2: 1 in 35000
Both autosomal dominant
What are the characteristic signs of type 2 neurofibromatosis?
Bilateral vestibular Schwannoma (acoustic neuroma)
Schwannonatosis (multiple tender Schwannomas without the bilateral vestibular Schwannomas)
Juvenile posterior subcapsular lenticular opacity (type of cataract)
What are the differential diagnosis for NF1?
McCune-Albright syndrome
Multiple lentigenes
Urticaria pignentosa
What is the differential diagnosis for NF2?
NF1
Schwannomatosis
What neurological problems can HIV cause?
Meningitis (fungal/TB) Toxoplasmosis Dementia Encephalomyelitis Cord problems Mononeuritis multiplex Guillain-Barré syndrome
What is HTLV-1 myelopathy/tropical spastic paraplegia?
Slowly progressing spastic paraplegia
Paraesthesiae
Sensory loss
Disorders of micturation
What is the treatment of Parkinson’s disease?
L-dopa (dopamine precursor which can cross the BBB)
Entacapone (dopamine decarboxylase inhibitor- reduced SEs by stopping peripheral conversion of L-dopa)
Selegiline (MAO-B inhibitors- increases effect of L-dopa)
Bromocriptine, ropinirole cabergoline (dopamine agonists)
Benzatropine (muscarinic antagonists- reduce tremor and drooling)
How does LEMS (Lambert-Eathon myasthenic syndrome) differ from myasthenia gravis?
MG- normal/brisk reflexes
LEMS- hyporeflexia
What are the symptoms of temporal lobe epilepsy?
Automatisms
Abdominal rising sensation/pain beforehand
Deja vu
Dysphasia
Impaired awareness and little memory of the event
Hallucinations
What are the symptoms of frontal lobe epilepsy?
Motor features e.g. posturing Jacksonian march Behavioural disturbances Dysphasia Todd's palsy post-seizure
What are the symptoms of parietal lobe epilepsy?
Sensory disturbances Motor symptoms (if seizure spreads to pre-central gyrus)
What kind of epilepsy do childhood febrile convulsions predispose to?
Temporal lobe epilepsy
What is a common cause of temporal lobe epilepsy?
Hippocampal sclerosis
What are the symptoms of an anterior cerebral artery infarction?
Weak/numb contralateral arm and leg
No facial involvement
Bilateral infarction–> akinetic mutism
What are the symptoms of a middle cerebral artery infarction?
Contralateral hemiparesis
Contralateral hemisensory loss
Dysphasia (dominant hemisphere lesions)
Apraxia (non-dominatnt hemisphere lesions)
What are the symptoms of a posterior cerebral artery infarction
Contralateral homonymous hemianopia
What are the symptoms of lacunar infarcts?
Pure motor/pure sensory symptoms
Ataxic hemiparesis
Dysarthria
Clumsy hand
What are the symptoms of infarction of the vertebrobasilar circulation?
hemianopia cortical blindness diplopia vertigo nystagmus ataxia dysarthria dysphasia hemiplegia unilateral/bilateral sensory symptoms
What is the clinical difference between conus medullaris/cauda equina lesion and lesions higher up in the cord?
Conus medullaris/cauda equina- weakness is flaccid and arreflexic
Lesions higher up in spinal cord: weakness is spastic and hyperreflexic
What is the most common cause of weak legs and cord compression?
Secondary malignancy (breast, lung, prostate, thyroid, kidney) in the spine
What are the symptoms of conus medullaris lesions?
Mixed UMN/LMN leg weakness Early urinary retention Constipation Back pain Sacral sensory disturbance Erectile dysfunction
What are the causes of a resting tremor? How is a resting tremor abolished?
Parkinsonism
Abolished by movement
What are the causes of postural tremor? When is it present?
Benign essential tremor (improves with alcohol)
Thyroxicosis
Anxiety
Beta-agonists
Tremor is present on maintained posture and may persist on movement
What is re-emergent tremor?
Postural tremor developing after a delay of 10 seconds (e.g. in Parkinsons)
DBS may help
What are the causes of chorea?
Huntington’s disease
Sydenham’s chorea (occurs after rheumatic fever. Spontaneous remission within weeks)
What are the causes of hemiballismus?
Vascular lesion of subthalamic nucleus (hemiballismus occurs contralateral to lesion)
What is athetosis and what are the causes?
Athetosis consists of slow, confluent, purposeless movements
Most common cause= cerebral palsy
Tics occur in which syndrome?
Tourette’s syndrome
What are the causes of myoclonus?
CJD Metabolic problems (metabolic flap- liver/kidney failure, thalamic stroke (unilateral flap), hyponatraemia, hypercapnia)
What are the treatments for benign essential myoclonus?
valproate, clonazepam, piracetam
What is the cause of tardive dyskinesia
Chronic extposure to dopamine agonists (antipsychotics, antiemetics)
What is dystonia?
Prolonged muscle contractions causing abnormal posture or repetitive movements
What is idiopathic generalized dystonia and how is it treated?
Childhood onset Starts in one leg and spreads along that side of the body Progresses over 5-10 years Autosomal dominant Treatment: Trihexyphenidyl, DBS Exclude Wilson's disease
What is the treatment for focal dystonias (e.g. writer’s cramp, blepharospasm)
Botox
What are the causes of acute dystonia? What are the signs and symptoms?
Caused by drugs:
- antipsychotics
- antiemetics (cyclizine)
Signs and symptoms:
- torticollis (head pulled back)
- oculogyric/oromandibular spasm (eyes drawn up and mouth movements
What is the treatment for acute dystonic reactions?
anti-cholinergics
What is the mortality rate after the first stroke?
What is the incidence of stroke?
12%
1/1000 per year incidence
What are the 4 main causes of stroke?
Small vessel occlusion or thrombosis in situ
Cardiac emboli
Atherothromboembolism (e.g. from carotids)
CNS bleeds (SAH, carotid artery dissection)
Which genetic diseases predispose to stroke?
CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leucocencephalopathy)
Antiphospholipid syndrome (predisposes to clots)
What is a watershed stroke?
A stroke that occurs in the zone between 2 cerebrovascular territories
What are the modifiable risk factors for stroke?
Hypertension, smoking, diabetes, heart disease (valvular, ischaemic, AF), peripheral vascular disease, past TIA, carotid bruit, OCP, increased lipids, alcohol, increased clotting, syphilis
What are the signs of a haemorrhagic stroke?
Meningism, coma within hours, severe headache, decreased GCS
What is the plan of action in the first hour after a stroke?
- Pulse, BP, ECG (do they have AF?, Cannot thrombolyse if the blood pressure is too high). Stop HRT if patient is on it.
- Blood glucose- aim for 4-11 mmol/L
- Urgent CT/MRI: Diffusion weighted MRI shows acute infarct. CT shows haemorrhage
- Thrombolysis (if symptoms begain under 4.5 hours ago)
- Nil by mouth- hydrate by IV
- Antiplatelet agents if haemorrhagic stroke is excluded (aspirin)
What are the contraindications for thrombolysis?
Major infarct or haemorrhage on CT Mild/non-disabling deficit Recent birth, surgery, trauma or artery/vein puncture Past CNS bleed AVM/aneurysm Severe liver disease, varices or portal hypertension Seizures Anticoagulants or INR above 1.7 Platelets below 100 BP> 220/130
What is the differential diagnosis for stroke?
Head injury Transient global amnesia Epilepsy (Todd's paresis) Space-occupying lesion Migraine (N.B. migraines can cause stroke) Demyelination Hypo/hyperglycaemia Infections Electrolyte disturbance Hypertensive encephalopathy Wernicke's encephalopathy Posterior reversible encephalopathy syndrome (PRES) Cerebral reversible vasoconstrictive syndrome (CRVS)
Which drug is used for thrombolysis?
Alteplase (tissue plasminogen activator)
How are ADC and DWI images used to identify stroke?
ADC- dark= new stroke
light= old stroke
DWI- dark=old stroke
light=new stroke
If bright on both ADC and DWI–> think demyelination or tumour
What is malignant MCA syndrome?
MCA occlusion
Massive oedema
Midline shift
What are the primary preventative measures for stroke (i.e. preventing a stroke in the first place)?
Control risk factors
Folate supplements
Lifelong anticoagulation for AF, prosthetic valves, rheumatic heart valves
What are the secondary preventative measures for stroke (i.e. preventing further strokes)
Control risk factors
Antiplatelet agents- clopidogrel
Anticoagulation after stroke from AF- warfarin
Which tests and investigations should be done after a stoke?
Tests for HTN- retinopathy, nephrpathy, enlarged heart on CXR
ECG- to look for AF, valvular lesions after endocarditis etc.
Carotid doppler- to look for carotid artery stenosis
Hypo/hyperglycaemia
Hyperlipidaemia
ESR- to look for vasculitis
Clotting screen- to look for thrombophilia
Hyperviscosity- polycythaemia, SCD
Thrombocytopenia
Genetic tests- CADASIL
AF increases chance of stroke by how much?
4.5% per year
What is used for prevention of stroke in patients with AF?
Warfarin or dabigatran
What are the cardiac causes of stroke?
Non-valvular atrial fibrillation
External cardioversion
Prosthetic valves
Acute MI (emboli arise in 10% of patients in the next 6-12 months)
Paradoxical systemic emboli
Cardiac surgery
Valve vegetations from infective endocarditis
what is the death rate in stroke?
20-25%
What is lateral medullary syndrome?
Most common brainstem vascular syndrome Caused by occlusion of posterior inferior cerebellar a. Sudden vomiting and vertigo Ipsilateral: Horner's syndrome Facial numbness Cerebellar signs Palatal paralysis Diminished gag reflex
