Clinical neurology Flashcards
Bilateral sensory involvement LMN diseases
Polyradiculopathy
Polyneuropathies- Guillain Barre
Mononeuritis multiplex
What is Guillain-Barre?
Acute inflammatory demyelinating polyradiculoneuropathy
Often follows gastroenteritis- commonly campylobacter jejuni
Progresses over days to weeks and may lead to respiratory muscle failure
Possible causes of polyneuropathies
HINT: A DANG THERAPIST
Acquired or inherited Diabetes Amyloid Nutritional- B12 deficiency Guillain Barre Toxic Hereditary- Charcot Marie Tooth, Friedrick's ataxia Endocrine Recurring/ Renal Alcohol Pb toxicity/ porphyria Infection Systemic- SLE, sarcoid, hypothyroid, syphilis Tumours- paraneoplastic
Around 50% are idiopathic
How is Duchenne muscular dystrophy inherited?
X-linked recessive
Dystrophin absence
Signs of Duchenne muscular dystrophy
Proximal lower limb weakness from the age of 4, gradually spreads to rest of muscles over time
Calf pseudohypertrophy (from infiltration)
Eventual spread to cardiac and respiratory muscles
Gower’s manoeuvre (when asked to stand from sitting)
Acquired myopathies usual clinical features
Proximal muscle weakness, usually symmetrical and more weakness than pain
Polymyositis/dermatomyositis
Often affect the pelvic girdle more than the shoulder
Investigations into acquired myopathies
CK- in the 1000s ESR EMG to confirm spontaneous activity Biopsy Genetic analysis Antibody testing
LMN, bilateral, purely motor potential issues
Myopathy- acquired- inflammatory, endocrine, metabolic- hereditary
Anterior horn cell
NMJ- MG, MG crisis, Lambert-Eaton, botulism
Presentation of myasthenia gravis
Fluctuating weakness
Proximal, ocular and bulbar- ptosis, diplopia, dysphonia, dysarthria, dysphagia
MG facies- snarl
Fatiguability
No atrophy/ fasciculations, normal tone and reflexes as not a nerve pathology `
What is the Tensilon Test?
Short acting anticholinesterase is given IV to see if there is any improvement
What imaging is done in MG?
CT of thymus looking for thymoma
Treatment for MG
Anticholinesterase inhibitors- pyridostigmine, propantheline
Immunosuppression- especially in acute- pred, IVIg, plasmapheresis
Thymectomy
Unilateral LMN signs
Radiculopathy
Plexopathy
Mononeuropathy
Causes of plexopathy
Features reflect motor and sensory findings extending over multiple nerve roots Trauma- Erb's palsy and Klumpkeys Neuralgic amyotrophy Thoracic outlet syndrome Malignant infiltration Radiotherapy (may be years later) Compression
Radiculopathy clinical features
Shooting, electric, sharp pain radiating down the limb
Can be across multiple levels if polyradiculopathy
ALS MND stands for
Amytrophic lateral sclerosis- Motor neuron disease
ALS differentials
UMN and LMN signs cervical spondylotic radiculomyelopathy Spinal tumour Spinal conus medularis lesions Vit B12 deficiency- sub acute degeneration of the cord and peripheral neuropathy
Treatment for ALS
No cure Riluzole used to prolong life (1-2 months) Supportive- CPAP/NIPPV PEG
What is MS?
Demyelinating disorder of the CNS
Who gets MS?
Classically white, young women who live at the poles
Types of MS
Clinically isolated syndrome
Relapsing-remitting
Primary progressive
Secondary progressive
How can MS be diagnosed
Two events demonstrating dissemination over space and time
Can be done faster now by radiological imaging
Management of MS
Acute- corticosteroids to rapidly induce recovery
Disease modifying therapy- interferons and monoclonal antibodies
Symptomatic management- spasticity, bladder, bowel, pain, depression/low mood
MDT
Types of trigeminal autonomic cephalalgias
Cluster headaches
Paroxysmal hemicrania
Short-lasting unilateral neuralgiform headache (with conjunctival injection and tearing)
Cluster headache important other differentials
Migraine
Other types of TACs
Secondary causes of chronic headache
Hypnic headache
Primary headache causes
Migraine
Tension type
Trigeminal autonomic cephalalgias
Other- exercise/ sex-induced
Secondary headache causes
Injury
Medication overuse
Cranial or cervical vascular disorder (carotid or vertebral artery dissection)
Venous sinus thrombosis
Non-vascular- idiopathic intracranial hypertension
Painful cranial neuropathies
Presentation of venous sinus thrombosis
Recurrent presentations
Procoagulant state
Headache with signs of raised ICP
Normal CT head (possibly anti-delta sign)
Signs of idiopathic intracranial hypertension
Papilloedema- compression of the optic nerves and subsequent visual loss
Other cranial neuropathies
Raised LP opening pressure
Management of IIH
Conservative- analgesia, weight loss (ultimate treatment), life-style
Medical- Acetazolamide, topiramate, furosemide
CSF drainage/ shunting
Acetazolamide
MOA: Carbonic acid anhydrase inhibitor
S/E: haemorrhage, metabolic acidosis, nephrolithiasis, abnormal sensation
AVOID in pregnancy (especially 1st trimester) and sulphonamide reactions
Causes of cerebellar dysfunction
HINT: MAVIS
MS Alcohol Vascular Inherited Space occupying lesion
Pyramidal tracts
Originate in the cerebral cortex
Voluntary control of musculature of the face and body
Extrapyramidal tracts
Originate in the brainstem
Involuntary and autonomic control of musculature- tone, balance, posture and locomotion
What is Uhthoff’s phenomenon?
Transient worsening of neurological symptoms when the body becomes overheated
Lhermitte’s phenomenon
Electric shock like complaint down the spine from flexion of the neck due to a cervical plaque
Clinical findings of MS due to optic nerve involvement
RAPD
Central scotoma
Red desaturation
Optic disc atrophy
Causes of carotid artery dissection
Traumatic- hyperextension
Iatrogenic- previous surgery, endartectomy
Spontaneous- HTN, smoking
Connective tissue disorder- Marfan’s, Ehlers-Danlos
Signs and symptoms of a carotid artery dissection
MCA thrombotic events Headache Amaurosis fugax Limb/facial weakness Neck swelling Horner's (partial if post-ganglionic)
What is Eagle syndrome?
Rare mimic of carotid artery dissection- calcified or elongated stylohyoid ligament compresses the carotid artery
Treatment of carotid artery dissection
Conservative- modification of risk factors
Medical- aspirin
Surgical- reconstruction
Miller-Fisher syndrome
Less severe version of Guillain-Barre which causes ophthalmoplegia, areflexia, ataxia
Always self-limiting, does not require treatment
Anti-GQ1b antibodies
Tests to diagnose MND and why
TFTs- thyrotoxicosis HbA1c and BM- diabetic amyotrophy CK- polymyositis/ dermatomyositis EMG- confirms MND pattern of fibrillation and fasciculations and loss of lower motor neurons Normal nerve conduction Genetic analysis
Typical pattern of presentation of MND
initial, progression and sparing
Isolated limb weakness (reduced grip or foot drop) which progresses to truncal and bulbar involvement
Progressive bulbar palsy- dysarthria and dysphagia
Usually spares extraocular muscles and sensory and autonomic systems
What enhances active MS plaques on MRI T2
Gadolinium
Differentials for myasthenia gravis
Myasthenic crisis Lambert-Eaton Myasthenic syndrome Polymyositis Dermatomyositis Botulism
Clinical features of GB
Back/leg pain in the initial stages
Progressive, symmetrical weakness of the limbs, classically ascending from the legs first, proximal muscles affected first
Reduced or absent reflexes
Mild sensory symptoms
Autonomic dysreflexia occurs in patients that have…
A spinal cord injury (often traumatic) above the level of T6
Most commonly triggered by constipation or urinary retention
Features of multisystem atrophy
Parkinsonian features
Cerebellar signs- ataxia
Autonomic disturbance:
Postural hypotension
Erectile dysfunction
Atonic bladder
Features of a bacterial meningitis LP
Turbid appearance Elevated opening pressure Very raised WCC Raised protein Low glucose
Features of a viral meningitis LP
Clear appearance Normal opening pressure Lymphocyte predominance, raised WCC Slightly raised protein Normal glucose
What is Hoover’s sign of leg paresis used to distinguish between?
Organic and non-organic paresis of the leg
Synergistic contraction of the contralateral leg if the patient is genuinely trying, no contraction suggests a functional/ non-organic cause
Management of possible TIA if the patient is anticoagulated
A&E for CT head to exclude haemorrhagic stroke- do not give aspirin 300mg until this is done
Conduction dysphasia
Classically due to a stroke affecting the arcuate fasciculus- connecting Broca’s and Wernicke’s areas
Speech is fluent and comprehension is intact but poor repetition
Clinical features of neuroleptic malignant syndrome
Rigidity, hyperthermia, autonomic instability, altered mental state- agitated delirium with confusion
Can cause AKI
Management of neuroleptic malignant syndrome
Stop causative antipsychotic
IV fluids to prevent renal failure
Dopamine agonist (bromocriptine)
Dantrolene, muscle relaxer
Stroke management
Exclude haemorrhage (CT head)
Aspirin 300mg (and then for 14 days)
Thrombolysis within 4.5 hours if no contraindications
+ thrombectomy within 6 hours
Secondary stroke prevention
Follow up in primary care at 6 months and annually
Lifestyle modification
Clopidrogrel 75mg daily
Aspirin if not tolerated and dipyridamole if both not tolerated
Duel aspirin and clopidogrel for 3 months may be initiated
Statin
Treatment for HTN
Weigh up anticoagulation risk vs benefit
Optimise co-morbidities
Triad of Wernicke’s encephalopathy
Ataxia, ophthalmoplegia (nystagmus most commonly) and confusion
Peripheral polyneuropathy may be seen
Treatment of Wernicke’s encephalopathy
Urgent replacement of thiamine
IV pabrinex- vit B/C
Causes of a third nerve palsy
Vasculitis DM Posterior communicating artery aneurysm MS Weber's syndrome- ipsilateral third nerve palsy with contralateral hemiplegia- midbrain strokes Amyloid
Features of progressive supranuclear palsy
Parkinsonian features- particularly early loss of balance when walking
More rapidly progressive
Ophthalmic and bulbar symptoms more pronounced- problems controlling eye movements and blurring of vision predominant
Features of corticobasal degeneration
Cortical and basal ganglia involvement
Parkinsonism
Often presents unilaterally and then progresses bilaterally
Apraxia, limb dystonia, cognitive features, visual-spatial impairment
Cushing’s triad and other features of raised ICP
Irregular breathing
Widened pulse pressure
Bradycardia
Papilloedema
Headache
Vomiting
Reduced consciousness
LP of bacterial meningitis
Cloudy and turbid appearance Elevated opening pressure Primarily polymorphonuclear leukocytes- neutrophils elevated Low glucose Raised protein
LP of viral meningitis
Appears clear Normal or elevated opening pressure Raised WBC- primarily lymphocytes Normal glucose Raised protein
LP of fungal meningitis
Clear or cloudy appearance Elevated opening pressure Elevated WBC Low glucose Elevated protein
LP of TB meningitis
Appears opaque Elevated opening pressure Elevated WBC Low glucose Grossly raised protein
LP of subarachnoid haemorrhage
Blood stained initially Xanthochromia after 12 hours Elevated opening pressure Elevated WBC Elevated RBC Elevated protein Normal glucose
Guillain Barre LP results
Albuminocytologic dissociation- raised protein without a raised WBC
Indications for LP
Diagnostic- MS, GB, meningitis, subarachnoid haemorrhage, encephalitis, idiopathic intracranial hypertension, normal pressure hydrocephalus
Therapeutic- IIH
Normal pressure hydrocephalus triad of signs
Instability- ataxic gait
Cognitive dysfunction
Incontinence
Contraindications for LP
Meningococcal septicaemia
Intracerebral haemorrhage
Raised ICP in a non-communicating hydrocephalus
Congenital malformations- chiari, spina bifida
Coagulopathy or anticoagulated
Meniere’s disease
Increased pressure of the endolymphatic system leading to recurrent attacks of vertigo lasting >20 minutes but <24 hours
Fluctuating sensorineural hearing loss
Aural fullness
Tinnitus
Vestibular schwannoma clinical features
Unilateral sensorineural hearing loss
Slow progression leading to CN being effected- 5,6,7,9,10
Absent unilateral corneal reflex
Bilaterally associated with NF2
BPPV clinical features
Occurs on head movement due to disruption of debris
Brief episodes of mild-intense vertigo
Dix-Hallpike manoeuvre shows fatigable nystagmus
Epley manoeuvre clears debris
Vestibular neuronitis clinical features
Recent viral infection
No deafness or tinnitus
Abrupt onset, severe, associated with nausea and vomiting
Drugs that can cause ototoxicity
Loop diuretics
Aminoglycosides ie gentamycin
Spinal cord tract changes in sub-acute degeneration of the spinal cord
Dorsal column loss causing sensory and LMN signs
Corticospinal cord tract loss causing UMN signs
Spinothalamic tracts remain intact and so there is no pain or temperature change even in extreme cases
How many beats of clonus is normal?
3 or less
Management of cluster headaches
Keep calm, 100% high flow oxygen for 15 mins and sumatriptans at onset
Education to avoid triggers- could be alcohol
Consideration of corticosteroids in the short term or verapamil prophylaxis
Treatment of trigeminal neuralgia
Patient education
Carbamazepine prophylaxis or lamotrigine/ phenytoin/ gabapentin
Surgery can be used if medication fails- microvascular decompression
Triggers of migraines
Chocolate Hangovers Orgasms Cheese/ caffeine Oral contraceptives Lie-ins Alcohol Travel Exercise
Differentials for migraine headaches
Trigeminal autonomic cephalalgias (including cluster) Tension headache Cervical spondylosis Raised ICP TIA
Management of migraines
Patient education about triggers
Medical prophylaxis- propranolol, topiramate
During an acute attack- oral triptan combined with ibuprofen or paracetamol. Anti-emetics. Warm or cold compress, rebreathing into a paper bag. 10 sessions of acupuncture
What is a Stokes-Adams attack?
Transient arrhythmia eg bradycardia due to incomplete heart block resulting in decreased cardiac output nad LOC
Falls to the ground with no warning except palpitations, injuries are common as a result of this (screen for these!!)
Pale with a slow or absent pulse
Recovery is within seconds but if prolonged LOC there may be anoxic clonic jerks
Blackouts differentialas
Vasovagal syncope- reflex bradycardia and peripheral dilation (situation syncope if triggered specifically) Carotid sinus syncope Epilepsy Stokes-Adams attacks Hypoglycaemia Postural hypotension Anxiety- no LOC Factitious
Differentials for unilateral foot drop
DM Early MND MS Stroke Common peroneal palsy Prolapsed disc
Spastic gait description
Stiff
Circumduction of the legs +/- scuffing of the toes
UMN lesions
Acute management of stroke
A-E assessment
CT head to rule out haemorrhage
NBM until swallow screen
300mg aspirin for 14 days
Consider thrombolysis as soon as haemorrhage is ruled out (within 4.5 hours of onset)- best results within 90 minutes- Alteplase
Thrombectomy within 6 hours if large artery occlusion in the proximal anterior circulation
Secondary prevention of stroke
Control risk factors- treat HTN, hyperlipidaemia, DM, cardiac disease, AF, smoking
14 days of 300mg aspirin
Switch to long-term clopidogrel monotherapy 75mg OD
MDT approach- PT, OT, SALT, specialist nurse, stroke team
Rehabilitation on a stroke ward and into the community
Tests
24 hour ECG
HTN- retinopathy, neuropathy, cardiomegaly
Carotid artery stenosis- carotid doppler US +/- CT angiography
Pharmacological treatment of cognitive decline
Acetylcholinesterase inhibitors- donepezil, rivastigmine, galantamine- recommended by NICE for dementia
Memantine- NMDA antagonist- late stage AD
Antipsychotics acutely if extreme agitation or psychosis
Vitamin supplementation
Todd’s palsy/paresis
Weakness following a focal seizure in the motor cortex
Extrapyramidal triad of Parkinson’s disease
Resting tremor (unilateral presentation, gets better on voluntary movement)- often pill rolling
Hypertonia- cogwheel rigidity
Bradykinesia
What is Devic’s syndrome/ NMO
Neuromyelitis optica
MS variant with transverse myelitis, optic atrophy
Anti-aquaporin 4 antibodies
MND with only LMN signs
Progressive muscular atrophy- anterior horn cell lesion so only LMN
MND definition
Cluster of neurodegenerative diseases characterised by selective loss of neurons in the motor cortex, cranial nerve nuclei and anterior horn cells
UMN and LMN with bulbar inclusion
No sensory or sphincteric disturbance
NEVER affects eye movements
Signs of neurofibromatosis type 1
Cafe-au-lait spots, adults have more than 5, do NOT predispose to skin cancer
Dermal neurofibromas and nodular neurofibromas, increase in number with age
Lisch nodules on the iris
Mild learning disability common
Malignancy in 5%
Autosomal dominant
Signs of neurofibromatosis type 2
Bilateral vestibular schwannoma are characteristic
Usually become symptomatic around 20 years old
Meningiomas
Autosomal dominant
