Neurology Flashcards
Which body parts fall into the territory of the anterior cerebral artery in the sensory and motor cortices?
Trunk, legs, feet
Which body parts fall into the territory of the middle cerebral artery in the sensory and motor cortices?
Arms, head and neck
What fasciculi are within the dorsal column? Which is medial and which is lateral?
Medial = Gracile fasciculus Lateral = Cuneate fasciculus
What sensory/motor role does the dorsal column have?
Fine touch + proprioception (sensory)
Where does the sensory input for the gracile fasciculus of the dorsal column come from?
Lower limbs
Where does the sensory input for the cuneate fasciculus of the dorsal column come from?
Upper limb
What are the 3 neurones involved in the dorsal column tract?
Dorsal root ganglion - gracile/cuneate nucleus (medulla)
Gracile/cuneate nucleus - ventral posterolateral thalamus
ventral posterolateral thalamus - post-central gyrus
Describe the path of the first neurone in the dorsal column tract
Sensory neurones in limbs and dorsal root gangion outside spinal cord
Enters gracile and cuneate fasciculi in spinal cord
Goes up to ipsilateral gracile and cuneate nuclei in medulla oblongata
Describe the path of the second neurone in the dorsal column tract
Gracilie/cuneate nuclei - decussates in pyramids
Travels through medial lemniscus of the pons
Reaches ventral posterolateral nucleus of thalamus
Describe the path of the third neurone in the dorsal column tract
Ventral posterolateral nucleus of thalamus to post-central gyrus in brain (sensory cortex)
What role does the spinothalamic tract have?
Pain, temperature and crude touch (sensory)
Where are the 3 neurons in the spinothalamic tract?
Sensory neuron/dorsal root gangion - dorsal horn of grey matter
Dorsal horn - contralateral thalamus
Thalamus - post-central gyrus
Describe the path of the 1st neuron of the spinothalamic tract
Sensory neuron/dorsal root ganglion
Enters spinal cord through Lissauer’s fasciculus
Ascends in spinal cord by 1 or 2 segments
Synapses in dorsal horns of grey matter
Describe the path of the 2nd neuron of the spinothalamic tract
Dorsal horn of grey matter - decussates in Anterior White Commissure at same level
Ascends through spinal cord and brain stem
Synapses at thalamus (contralateral to dorsal horn)
Describe path of 3rd neuron of spinothalamic tract
Thalamus to post-central gyrus
What is the role of the corticospinal tract?
Axial and limb motor
What is the role of the lateral corticospinal tract?
Limb motor
What is the role of the anterior corticospinal tract?
Axial motor
Describe the path of the lateral corticospinal tract
Pre-central gyrus (motor cortex) - internal capsule - brainstem
75-90% corticospinal tract decussate at pyramids (lateral corticospinal)
Descend through spinal cord and synapse at anterior horn cell
Lower motor neuron from anterior horn cell to limb muscles
Describe the path of the anterior corticospinal tract
Pre-central gyrus (motor cortex) - internal capsule - brainstem
10-25% do not decussate at pyramids (anterior corticoospinal)
Descend to level in spinal cord where decussates through anterior white commissure
Synapses at contralateral anterior horn cell
Lower motor neuron from anterior horn cell to axial muscles
Give 4 nerve endings for fine touch
Pacinian corpuscle
Meisner’s corpuscle
Ruffini endings
Merkel endings
What 3 tests do you do for a mini neurological exam?
General appearance + vital signs
GCS
Lateralising signs
Pupil reflexes
What are the 3 domains of the Glasgow Coma Scale?
Best Motor Response
Best Verbal Response
Best Eye-opening Response
Which of the 3 domains of the Glasgow Coma Scale is the best prognostically?
Best Motor Response
What might you do if patient is unconscious to assess them?
Collateral history
Mini Neurological Exam
GCS
Pupils
What are the criteria for the scores on BMR of GCS?
6 = obeys commands 5 = Localises to pain 4 = Flexes to pain 3 = spastic flexion/withdrawal (don't use) 2 = Extends to pain 1 = None
What do lateralising signs tend to indicate? Give examples of lateralising signs
One hemisphere lesion
E.g. gaze paresis, inattention to one side, upper limb drift, asymmetrical flexion/extension
What causes a fixed dilated pupil?
Compression of parasympathetic fibres on outside of CN3, usually over petrous temporal bone
What shape does a subdural bleed show on imaging? Why?
Crescent-shaped
Low pressure venous blood causes slow bleed
What shape does an extradural bleed show on imaging? Why?
Convex shaped
Needs high pressure arterial blood to peel periosteal dura off cranium
What does a subarachnoid haemorrhage present with?
Sudden onset, severe headache - pressure on meninges
Nausea, vomiting, stiff neck, photophobia, restless, agitation
May have seizures
What symptom might an occipital lobe lesion cause?
contralateral visual defect
What might occur with dominant parietal lobe syndrome?
Confusion
Difficulty writing and reading (agraphia)
Difficulty with mathematics (acalculia)
Visuospatial impairment - 3D (agnosia)
Apraxia - inability to comprehend verbal commands and motor impairment
Contralateral sensory impairment
Where is Broca’s area? What is its role?
Dominant hemisphere frontal lobe (usually left)
Speech production
Where is Wernicke’s area? What is its role?
Dominant hemisphere temporal lobe (usually left)
Surrounds auditory cortex, close to lateral fissure
Comprehension of speech
What might frontal lobe lesions present with?
Bilateral lesion = personality
Unilateral lesion = hemiparesis
What might temporal lobe lesions present with?
Memory loss - verbal memory loss if dominant hemisphere, non-verbal if non-dominant
What is the general role of the basal ganglia? How might problems present?
General role = control of movement and reward
Too much control = rigidity
Too little = chorea
What is the most likely cause of sudden onset hemiparesis? Where do most of these originate?
Stroke
More are ischaemic - embolic from carotid bifurcation (75%) or from heart (25%)
What would occur if a middle cerebellar artery stroke happened? What happens instead?
MCA stroke = unconscious
Most strokes in this territory are perforator artery emboli affecting internal capsule
Where is the lesion in myelopathy?
Spinal cord
What are the features of the A fibres in peripheral nerves?
Long, fast, myelinated
Role: proprioception, vibration, fine touch
What are the features of the C fibres in peripheral nerves?
Short, slow, non-myelinated
Role: pain, crude touch, temperature
Which nerve fibres are affected first in diabetic peripheral neuropathy? What test of sensation is most sensitive for peripheral neuropathy?
A fibres - long fibres affected first
Therefore - VIBRATION test - picks up neuropathy before symptoms/numbness appears
What is the most common cause of spinal cord lesions?
Disc osteophytes from arthritis
Give a rhyme for proximal muscle weakness. What are the presenting features?
Can’t do hair, stair, chair
Weak grasp, handwriting and flapping gait - finer movements lost first
Fatigue
Where is the lesion in a radiculopathy?
Nerve root - initial segment of a nerve leaving the spinal cord
Where is the most common cervical radiculopathy? Give the dermatome and myotome distributions of these nerve roots.
C6 and C7
C6 dermatome = thumb, myotome = biceps
C7 dermatome = middle and index finger, myotome = triceps
Where are the most common lumbar radiculopathies? What are the dermatomes and myotomes affected for these nerve roots?
L5 and S1
L5 dermatome = big toe, dorsal foot
Myotome = dorsiflexion of foot (stand on heels)
S1 dermatome = ltitle toe, lateral and sole of foot
Myotome = plantar flexion foot
What features might suggest cerebellar lesion?
IPSILATERAL dysdiadochokinesia, ataxia, nystagmus, intention tremor, slurred speech, hyperreflexia (drunk)
Where are the most common sites for compression if these cranial nerves are affected together?
a) CNIII, IV, VI
b) CNVII, VIII
c) CNIX, X, XI, XII
a) cavernous sinus
b) petrous part of temporal bone
c) jugular foramen
What are features of an intracranial mass?
epilepsy
focal neurological signs
raised intracranial pressure - headache, dizziness, nausea, vomiting, blurred vision
What is the commonest cause of proximal myopathy? How might you test for proximal myopathy clinically?
Corticosteroid use e.g. transplant, chemotherapy, autoimmune
Stand on one leg and squat
If there is a lesion at a disc level, which nerve root will be affected?
Nerve root below
e.g. disc lesion at C6/C7 - present with C7 radiculopathy
What are other ways of saying “long tract signs”?
UMN, pyramidal, descending tract, corticospinal tract signs
Give some examples of long tract signs
spastic gait hypertonia, hyperreflexia clonus + babinski cross-adductors Hoffman's sign loss of fine finger movements Deltopectoral reflex
What frequency is vibration sensation measured at?
128Hz
Where should pain stimulus in Best Motor and Best Eye response for GCS be done?
to CN distribution so not to elicit spinal reflex
e.g. behind ear and mastoid bilaterally
What is the formula for cerebral perfusion pressure? What should cerebral perfusion pressure be?
CPP = mean arterial pressure - intracranial pressure
Should be 60-70mmHg
Describe the relationship between intracranial volume and intracranial pressure
Little increase in ICP with increasing volume until decompensates
Then exponential large increase in ICP with small increase in volume
How long do the majority of seizures last for?
1-2 minutes
Give the management of a seizure
Time it! Insert nasopharyngeal airway and oxygen mask, monitor O2 sats
If >10 minutes - IV midazolam
Loading dose: IV phenytoin, valproate, levotiracetam 15 min infusion with cardiac monitoring
Give post-seizure management
U+Es
Possibly CT head
Beware of peri-ictal aspirations and risk of LRTI
What drug can you use for acute management of raised ICP?
Mannitol - 100ml 10% or 50ml 20%
When might hydrocortisone cover be given?
If on long-term corticosteroids, cover with hydrocortisone to prevent adrenal crisis
When might dexamethasone be given? What cautions need to be taken?
Given peri-operatively for tumour neurosurgery
Discontinue after 2 weeks (avoid Cushings)
Monitor blood glucose - risk hyperglycaemia
Beware of steroid psychosis
What risks do anticoagulants and anti-platelets have in neurology? How would you manage these?
Increased risk intracranial haemorrhage and chronic subdural bleeds
If pathology found, stop meds, and reverse acutely
Discuss with haematologist and only re-start if agreed by neurosurgeon
Give the pathophysiology of cranial diabetes insipidus. Give management
Decreased ADH secretion - can be feature of hypopituitarism
Excessive diuresis, thirst and hypernatraemia
Give diuresis post-surgery
Fluid-balance chart, drink to thirst, consider desmopressin
Why might hyponatraemia occur in neurology?
Water moves into plasma from cells e.g. cerebral oedema, raised ICP
Brain injury releases natriuretic peptides causing “cerebral salt wasting”
SIADH
What are the effects of hyponatraemia?
potentially life-threatening >48hrs or severe
Seizures, coma, cardio-respiratory arrest
Chronic: falls, gait problems, concentration and cognitive decline, osteoporosis
How would you manage hyponatraemia?
IV crystalloid and consider mineralocorticoid
If SIADH, either fluid restriction OR sodium replacement
Careful - Too rapid Na replacement risks central pontine myelinosis
How might you manage neuropathic pain?
Trial and error with neurotropics
Gabapentin + pre-gabalin for radicular pain
Carbamazepine - trigeminal neuralgia
Topirimate - chronic refractive head pain
Give the total capacity of the CSF and of the ventricles
CSF total = 120ml
Ventricles = 20ml
Give 3 functions of CSF
Protection (cushion)
Buoyancy - reduces pressure on base of brain
Environment for brain function and neurotransmission e.g. low K
What lines the ventricles and what produces CSF?
Ependymal cells
Choroid plexus produces CSF
What foramen connects 1st and 2nd ventricles to the 3rd?
Foramen of Monro
Where is the 3rd ventricle and what connects it to the 4th ventricle?
Between left and right thalamus
Connects to 4th via CEREBRAL AQUEDUCT
Where is the 4th ventricle and how does it drain to spinal canal and subarachnoid cisterns?
4th ventricle between pons and medulla
Drains laterally via Foramina of Luschka and foramen of Magendie medially
Give some presenting features of myelopathy
Back pain
Long tract signs - UMN signs
Loss of fine finger movements
Radiating limb pain
Give features of vertebral syndrome
Lower back pain - dull, aching
Radiating pain not as far as radiculopathy
Give features of radiculopathy
Sensory loss/pain in dermatome AND/OR motor loss in myotome
Possible back pain
What difference in presentation is there between L5 radiculopathy and common peroneal nerve palsy?
L5 radiculopathy - weakness on foot inversion
common peroneal nerve palsy - weakness on foot eversion
Give the features of Brown-Sequard syndrome
Lesion in one half of spinal cord
Ipsilateral UMN weakness below lesion
Ipsilateral loss of fine touch, proprioception and vibration below lesion
Contralateral loss of pain, temp and crude touch 1-2 segments below lesion
What is paracellar syndrome and what features present?
Pituitary tumour or mass in pituitary cellar
Upward pressure on optic chiasm - bitemporal hemianopia
Hypopituitarism
What are the 2 types and relative prevalence of strokes?
Ischaemic (85%)
Haemorrhagic (15%)
(also can be due to systemic hypoperfusion)
What is the WHO definition of a stroke
Clinical syndrome consisting of RAPIDLY developing clinical signs of focal (or global in coma) disturbance of cerebral function lasting MORE THAN 24 HRS or leading to death with no apparent cause other than a VASCULAR origin
Given common symptoms of an anterior/carotid circulation stroke
Weakness (face, legs, arms)
Impaired speech/language
Amaurosis fugax
Give common symptoms of a posterior circulation stroke
Dysarthria Dysphagia Diplopia Dizziness Ataxia Diplegia
Give the 3 types of ischaemic stroke
Embolic
Thrombotic
Systemic hypoperfusion
What does the acute CT scan show after a stroke? Why might a repeat CT be done after 48hrs?
Acute - bleeds/haemorrhagic stroke
Ischaemia appears normal, but after 48hrs may see dark patches
What arteries supply anterior circulation?
Internal carotid arteries - ACA and MCA
What arteries supply posterior circulation?
Posterior cerebral artery and vertebral arteries
What arteries connect anterior and posterior circulations?
Posterior communicating arteries
What criteria defines a total anterior circulation infarct?
Contralateral weakness/sensory loss
Homonymous Hemianopia/visual inattention
Higher cortical dysfunction - dysarthria, dysphasia
What criteria defines a partial anterior circulation infarct?
2 of the following:
Contralateral weakness/sensory loss
Homonymous hemianopia/visual inattention
Higher cortical dysfunction - dysarthria, dysphasia
What are the presenting features of a posterior circulation infarct?
Cranial nerve palsy Contralateral sensory/motor deficit (pyramidal lesion) Cerebellar dysfunction Isolated homonymous hemianopia Bilateral events - reduced GCS
What is a lacunar infarct?
= occlusion of deep penetrating arteries
Why does a lacunar infarct not present with higher cortical features?
Only affect small volumes of SUB-cortical white matter so does not present with cortical features e.g. dysarthria, dysphasia, visual field loss
What syndromes are common in lacunar infarcts?
pure motor hemiparesis Ataxic hemiparesis "clumsy hand" + dysarthria pure hemisensory loss Mixed sensorimotor
Why do small lacunar infarctions cause large clinical syndromes?
White matter infarcts - nerve fibres narrowed down so small area has large clinical consequences
What is the acute management of a stroke?
ABCDE + bloods (including BM!)
Hx: TIME of onset, risk factors, contraindications to thrombolysis, blood pressure, NIHSS
Urgent CT head (no contrast within 1h) BEFORE thrombolysis/aspirin
Thrombolysis/thrombectomy OR Aspirin 300mg
What might be the next stage and long-term management of a stroke?
Investigate cause (full history, exam, investigations)
Screen and prevent complications (infection, sores)
Secondary prevention (lifestyle, meds, surgery)
Rehabilitation (PT/OT/SLT)
What is the NIHSS?
National Institute of Health Stroke Scale
Score 0-42
Grades severity of stroke and can use to monitor response to acute treatment
How might ischaemic regions appear on an acute CT?
May not be visible
Subtle blurring and decreased attenuation of grey-white junction due to oedema may be early sign
May see hyperdense vessel - sign of intravascular clots e.g. MCA
What type of infarcts are MRIs better at viewing?
Posterior circulation infarcts
Lacunar infarcts
Give the indications for thrombolysis post-stroke
Within 4.5 hours of symptom onset
Non-haemorrhagic stroke on CT
Give some contra-indications for thrombolysis
Haemorrhagic stroke/bleed current or previous anti-coagulation/coagulopathy pregnancy high blood pressure Aneurysm Severe liver disease, varices, portal HTN Seizures at presentation Hypo/erglycaemia Intracranial neoplasm history Rapidly improving symptoms
What is given for thrombolysis? How should you follow up this treatment?
IV Tissue Plasminogen Activator (ALTEPLASE 0.9mg/kg)
Monitor blood pressure and complications of bleeding
24hr CT to check for haemorrhagic transformation
Give the indications and methods for thrombectomy
Indications - within 6 hours of symptom onset in anterior circulation, later if basilar thrombosis.
Methods - CT angiography to remove clot, can be used alongside thrombolysis
What are the 2 parts of ischaemic cerebral tissue called and which is more likely to survive when blood supply returns?
Ischaemic core (central) surrounded by Ischaemic Penumbra (outer) Core dies 1st, but penumbra may live if blood supply returned The smaller the core, the better
What investigations might you do to investigate the cause of the stroke?
Bloods: FBC, UE, ESR, Lipids, LFTs, CRP, clotting, glucose/Hba1c
ECG + 72 hour tape (paroxysmal AF)
Carotid Doppler USS (carotid stenosis)
Echo (endocarditis/valvular disease)
MRI (confirm diagnosis, infarcts) or delayed CT if not tolerated
What appears white in a
a) T1 weighted image?
b) T2 weighted image?
a) fat
b) water
What is the best type of MRI for detecting acute infarcts?
Diffusion-weighted MRI - infarct appears WHITE
What type of MRI is matched with DW MRIs to detect infarcts?
ADC - infarct appears BLACK
Match black lesion to white lesion on DW MRI
DWI-ADC match
Give examples of MDT management post-stroke
nursing
PT, OT, SLT, dietitian, orthoptics
What lifestyle advice might you give someone after a stroke?
smoking cessation limit alcohol and drug use dietary modifications exercise driving advice
What medications might you give as secondary prevention for strokes?
Anti-platelets - Aspirin 300mg for 2 weeks, then Clopidogrel lifelong
Anticoagulation: if have AF (HASBLED vs CHADSVASC score to determine this)
Anti-hypertensives: if haemorrhagic, be careful if ischaemic - aim <130/80
Statins - aim 40% decrease in non HDL cholesterol
How might you surgically manage extracranial carotid stenosis?
If 70-99% occluded - carotid endarterectomy
If 50-69% occluded - consider CEA or carotid artery stenting
How could you manage malignant MCA syndrome?
Decompressive hemicraniotomy
How could you manage posterior circulation infarct surgically?
External ventricular drainage or Posterior fossa decompression to prevent risk of hydrocephalus
What are the different methods of management for haemorrhagic stroke?
Manage hydrocephalus
Reversal of anticoagulation
Blood pressure control
What are the indications for medical and surgical management of haemorrhagic stroke?
Small deep haemorrhages, lobar haemorrhage without hydrocephalus, rapid neurological deterioration, large haemorrhage and significant comorbidities before stroke, GCS <8, posterior fossa haemorrhage
What are the indications for reversal of anticoagulation in haemorrhagic stroke management?
Primary intracerebral haemorrhage and taking anticoagulants with elevated INR
What are the indications for rapid blood pressure lowering in acute management of haemorrhagic stroke?
Within 6 hours symptom onset
systolic BP 150-220
Consider if >6hrs or BP>220
Aim systolic 130-140 within 1h and maintain for 7 days
What are the contraindications to rapid blood pressure lowering in management of haemorrhagic strokes?
Underlying structural cause
GCS<6
Awaiting neurosurgery for haematoma
Massive haematoma with poor prognosis
What medications can be used for reversal of anticoagulation in haemorrhagic stroke management?
Warfarin - Beriplex and vit K
LMWH - protamine
DOACs - Beriplex partial
Dabigatran - Idaruciumab
What causes can mimic stroke presentations?
Seizures Tumours/abscess Migraine Metabolic Functional Myelopathy, peripheral neuropathy, cranial nerve
What might you want to determine about the distribution and pattern of muscle weakness and why?
Proximal (muscle) or distal (nerve)
Symmetrical (genetic/metabolic) or asymmetrical
Mono or poly
Cranial involvement (bulbar/ophthalmoplegia)
Variability (fatigueability/relapse-remission)
Sensory deficit?
Give clinical features of Motor neurone disease and exclusion criteria
UMN (+LMN)
No sensory deficit or sphincter disturbance
Never affects eye movements
What are the 4 types of motor neuron disease? Which is the most common?
Most common = amyotrophic lateral sclerosis (ALS)
Progressive bulbar palsy
Progressive muscular atrophy
Primary lateral sclerosis (rare)
What are the pathophysiological features of MND?
Loss of motor neurones in cortex, cranial nerve nuclei and anterior horn cells
Where are the loss of motor neurones in ALS and what symptoms appear?
Loss motor neurons in motor cortex AND anterior horn cell
UMN+LMN signs
Worse prognosis if bulbar onset, older age, reduced FVC
Where are the loss of neurons in progressive bulbar palsy?
Cranial nerves 9-12
UMN (corticobulbar) and LMN (bulbar)
Where are the loss of neurons in progressive muscular atrophy? What type of signs do you see?
Anterior horn cell loss
LMN signs only
Where is loss of neurons in primary lateral sclerosis and what signs do you get?
Loss of Betz cells in motor cortex
Mainly UMN
Pseudobulbar palsy
Spastic leg weakness
What is the classical presentation of someone with MND?
Usually >40 (median 60yrs) Stumbling spastic gait Footdrop +/- proximal myopathy Weak grip/shoulder abduction Aspiration pneumonia UMN+LMN, potential bulbar signs Frontotemporal dementia in 25%
How is MND diagnosed?
By EXCLUSION
Brain/cord MRI to exclude structural
LP to exclude inflammatory
Nerve conduction studies - detect loss of motor neurones and exclude mimicking motor neuropathies
What is the prognosis of MND?
Poor, <3 years in 50% patients
How would you manage MND?
MDT approach
Riluzole - NMDA receptor antagonist, improves survival slightly
Supportive
What supportive measures might you need to do for someone with MND?
excess saliva - positioning, suction, oral care, anti-muscarinics, botulinum toxin A
dysphagia - blend food, gastrostomy
Spasticity - baclofen, exercise, orthotics
communication - SLT, alternative methods
palliative care - from diagnosis!
ventilation - if wanted, only increases prognosis few months
Give 3 branches of peripheral nerve disease
Polyneuropathy
Mononeuropathy
Mononeuritis multiplex
Give some causes of polyneuropathy
Peripheral neuropathy Guillain-Barre Syndrome Lead poisoning Charcot-Marie tooth Leprosy
Give features of peripheral neuropathy
Chronic and progressive
Starts in longer nerves (vibration, proprioception sense)
Starts in legs first, “glove and stocking”
Sensory/LMN/both signs - numbness, pain, difficulty handling small objects/fine motor loss
Trophic changes - hair loss, shiny skin, calluses, nail changes, cold, pallor
What are the common causes of peripheral neuropathy?
Commonest = DIABETES 20% = idiopathic
What are some less common causes of peripheral neuropathy?
B12 or folate deficiency Alcohol/toxins/drugs hereditary neuropathies paraneoplastic syndromes metabolic - uraemia, thyroid
How would you manage peripheral neuropathy?
Treat cause - e.g. diabetes, deficiencies, alcohol XS
PT + OT - orthotics
Splinting joints to prevent contractures in prolonged paralysis
Give features of Guillain-Barre syndrome
Subacute <6 weeks
Ascending paralysis/numbness/areflexia
Sensory loss and LMN signs
Post-infectious autoimmune response
What investigations would you do for Guillain-Barre syndrome and what would you see?
NCS - demyelination
LP - raised protein, normal or decreased WCC (cyto-albuminologic dissociation)
How would you treat Guillain-Barre syndrome?
IV Ig
Supportive measures
Monitor FVC
Give some features of Charcot-Marie tooth disease
Hereditary motor and sensory neuropathy (HMSN)
Muscle weakness/sensory loss in feet, legs and hands
Abnormal gait
High arched or very flat feet
Usually presents age 5-15
Managed: supportive, OT/PT, walking aids
Describe the distribution and presentation of mononeuritis multiplex. How might you localise the site of lesions?
Random distribution of mononeuropathies (2 or more)
Subacute presentation <6 weeks
Electromyography
Give the causes of mononeuritis multiplex
vasculitides
Connective tissue disorders e.g. SLE, RA, sarcoidosis
Leprosy
What is the commonest mononeuropathy?
Carpal tunnel - median neuropathy
What other common mononeuropathies occur in the upper limb?
Ulnar neuropathy - compression at elbow
Radial neuropathy - compression in axilla
What is the are the comonest mononeuropathies of the leg?
Common peroneal neuropathy - fibular head
Lateral cutaneous thigh neuropathy - anterolateral burning thigh pain - inguinal ligament
What are the signs of common peroneal nerve palsy?
Footdrop
Dorsal foot sensory loss
Weak ankle dorsiflexion and EVERSION
Give the pathophysiology of myasthenia gravis
Autoantibodies to nicotinic post-synaptic acetylcholine receptors at neuromuscular junction - both B and T cells implicated
Where does spasticity tend to develop when there is UMN/pyramidal weakness?
In stronger muscle groups
arms = flexors
legs = extensors
Describe spasticity
Increased tone
Velocity-dependent - faster movement = greater resistance, until gives way
Clasp-knife response
Describe spasticity
Increased tone
Velocity-dependent - faster movement = greater resistance, until gives way
Clasp-knife response
Which groups are more likely to have myasthenia gravis?
<50 = women (thymic hyperplasia), >50 = men (thymic atrophy or thymoma)
Autoimmune conditions
Give presenting features of myasthenia gravis
Slowly progressive or relapsing muscular fatigue. Fatigue increases on repetition of movement of VOLUNTARY muscles
Order of muscles affected: extraocular, bulbar, face, neck, limb girdle, trunk
Ptosis, diplopia, “peek sign” - orbicularis fatiguability
Myasthenic snarl on smiling
Voice fades when counting to 50 - dysphonia
Normal reflexes
What can exacerbate myasthenia gravis symptoms?
pregnancy hypokalaemia infection over-treatment change of climate emotion exercise gentamicin or tetracyclines or quinine or beta blockers opiates
What investigations could you do in someone presenting with myasthenic features?
Anti-AchR antibodies (90%) or MuSK Abs
Electromyography - decreasing muscle response to repeated nerve stimulation
CT thorax to exclude thymoma
Ice on eyelid test (not diagnostic)
Tensilon test - rare (inject edrophonium)
How would you treat myasthenia gravis?
Pyridostigmine (Achesterase inhibitor) for symptoms
Prednisolone + osteoporosis prophylaxis
OR azathioprine/methotrexate/mycophenolate
Thymectomy (with or without thymoma can be helpful)
What is myasthenic crisis?
Life-threatening weakness of respiratory muscles
Can be caused by infection, natural disease or overdosing cholinergic meds (pyridostigmine)
How would you manage a myasthenic crisis?
Monitor FVC, ventilatory support if needed
Plasmapheresis or IV Ig
Treat cause
Call anaesthetics and urgent neurology review
What is the pathophysiology of Lambert-Eaton myasthenic syndrome (LEMS)?
Paraneoplastic or autoimmune production of antibodies to Ca channels on pre-synaptic membrane of neuromuscular junction
What clinical features might you see in LEMS?
Gait difficulty before eye signs
Autonomic involvement (dry mouth, constipation, impotence)
Hyporeflexia and weakness, symptoms and EMG amplitude improve after exercise
What are common causes of proximal myopathy?
Steroids
Statins
Metabolic and endocrine
Myotonic dystrophy
What are less common causes of proximal myopathy?
Duchenne’s, Becker’s, muscular dystrophies
Inflammatory muscle disease (polymyositis, dermatomyositis)
Mitochondrial disorders
What are features of proximal myopathy?
Gradual onset Preserved reflexes, no fasciculation Can't do hair, stairs, chairs Muscle wasting Facial and neck weakness (sometimes) Contractures (Scoliosis) (Eye movement disorders)
How would you investigate suspected proximal myopathy?
CK (Duchenne’s),ESR, AST, LDH, CRP
EMG
(Muscle biopsy)
(genetic testing)
How would you treat proximal myopathy?
Remove cause/medications
Supportive (OT, PT, back, renal, diet)
Immunosuppressants if inflammatory
Give some features of Duchenne’s muscular dystrophy
Proximal myopathy, commonest of muscular dystrophies, X-linked recessive
Presents 4 yrs old clumsy walking, difficulty standing and respiratory failure
Pseudohypertorphy in calves
Raised serum CK
Supportive treatment and ventilatory support
Give some features of Becker’s muscular dystrophy
Proximal myopathy, less common of muscular dystrophies, X-linked recessive
Presents later and milder than Duchenne’s
Better prognosis
Give some features of Faciospaculohumeral muscular dystrophy (FSHD, Landouzy-Dejerine)
Proximal myopathy, common as Duchenne’s, autosomal dominant
Onset 12-14
Inability to puff out cheeks, or raise arms above head
What is a myotonic disorder? What is the commonest form and its 2 types?
= tonic muscle spasm, long chains central nuclei within muscle fibres
Myotonic dystrophy - DM1 and DM2
DM1 more common and severe, both types autosomal dominant
Mexiletine may help with disabling myotonia, genetic counselling
What is the MRC grading for power?
5 = normal power 4 = active movement against gravity and resistance 3 = active movement against gravity 2 = active movement with gravity eliminated 1 = flicker movement/contraction 0 = no movement/contraction
What is subacute combined degeneration of spinal cord?
B12 deficiency
Affects dorsal column first - proprioception
Which way does someone tend to fall in an ataxic gait?
towards the side of the lesion
What are the 3 broad differentials for transient loss of consciousness (TLOC)?
- syncope
- epileptic seizure
- non-epileptic (psychogenic)
What causes syncope as a whole category?
Transient global cerebral hypoperfusion
What are the 3 types of syncope?
Reflex
Cardiogenic
Orthostatic hypotension
What are the 3 types of reflex syncope?
vasovagal
situational - micturition, exercise
carotid sinus hypersensitivity
What causes vasovagal syncope? aka neurocardiogenic
Reflex bradycardia +/- peripheral vasodilation
Provoked by emotion, pain, standing too long
What are the typical features of vasovagal syncope?
Onset over seconds - not instant
Prodromal symptoms (nausea, pallor, sweating, narrowing of vision)
Brief clonic jerking possible, but no tonic clonic
Duration - 2mins
Rapid recovery
(3Ps - precipitating, prodrome, posture)
What are the typical features of situation syncope?
Clear precipitant - cough, effort/exercise, micturition onset over seconds prodromal symptoms may jerk but not tonic-clonic Duration - 2 mins Rapid recovery
What are the typical features of carotid sinus hypersensitivity?
Baroreceptors in carotid sinus hypersensitive - reflex bradycardia + vasodilation on minimal stimulation e.g. head turning or shaving
Same as vasovagal in features
What are the features of cardiogenic syncope?
Transient arrhythmias or structural stenosis causing tachycardia or bradycardia
No warning except palpitations, falls to ground
Pale, slow or absent pulse
Recovery within seconds, patient flushes, pulse speeds up
Anoxic clonic jerks may occur in prolonged LOC
May happen several times a day and in any posture
What are some other causes of blackouts outside of syncope, epilepsy and pscychogenic causes? What features might you see?
Hypoglycaemia - tremor, hunger, light-headed
Anxiety - hyperventilation, tremor, sweating, tachy, weakness, light-headed, no LOC
Drop attacks - sudden drops without LOC (narcolepsy, catoalexy, acute hydrocephalus)
Factitious blackouts - pseudoseizures, Munchausens
What is an epileptic seizure?
Clinical manifestation of abnormal electrical discharge in the brain
What is epilepsy?
Tendency to recurrent epileptic seizures
What are the features of an epileptic seizure?
Attacks when asleep or lying down
Aura, (identifiable triggers?), sudden onset
Altered breathing + cyanosis
Typical tonic-clonic movements (symmetrical)
Jerking changing frequency
Urinary incontinence
Tongue-biting - lateral
Duration <3mins
Prolonged post-ictal drowsiness/confusion - 15 mins
Amnesia
Transient focal paralysis (Todd’s palsy)
Why is TIA not a differential for TLOC?
TIA is a focal hypoperfusion whereas TLOC indicates GLOBAL hypoperfusion!
Give the features of a non-epileptic seizure
May be trigger e.g. anxious, stress No prodrome or sparingly described Jerking not synchronous, random, constant frequency Rhythmic pelvic movements, head side to side Eyes often close May bite tongue but often tip of tongue Duration >3 mins (longer than GES) May feel tired after
What is your first investigation following a TLOC?
ECG - rule out cardiac causes e.g. heart block, arrhythmias, long QT
May do ambulatory ECG/24 hr ECG to see transient arrhythmia
What other investigations might you do after ECG for TLOC?
U+E, FBC, Mg, Ca, glucose Tilt-table test (exclude cardiac cause first) Echocardiogram EEG/sleep EEG CT/MRI
What are the 3 classifications of focal seizures?
Simple - no LOC
complex - impaired consciousness, often temporal
Starts partial then evolves to bilateral generalised convulsive seizure
What are the different types of generalised seizures?
Absence seizures Tonic-clonic Myoclonic Atonic - sudden loss muscle tone, no LOC Infantile spasms
Give some features of temporal lobe focal seizures
Impaired awareness + complex motor e.g. lip smacking, chewing, swallowing, manual movements
Dysphasia, deja vu, jamais vu
Emotional disturbance
Olfactory, gustatory, auditory hallucinations, delusions
Post-ictal confusion
Give some features of frontal lobe focal seizures
Posturing or peddling movements of legs Focal motor seizure starting with thumb/face (Jacksonian march) Motor arrest Subtle behavioural disturbances Dysphasia or speech arrest Post-ictal Todd's palsy
Give some features of parietal lobe focal seizures
Sensory disturbances - tingling, numbness, pain (rare)
Motor symptoms if spreads to pre-central gyrus
Give some features of occipital lobe focal seizures
Visual phenomena - spots, line, flashes
What is amaurosis fugax?
Occlusion of retinal artery causing unilateral progressive vision loss
“like a curtain descending”
What can cause TIAs?
Carotid emboli
Cardiac emboli
Hyperviscosity - polycythaemia, SCD, myeloma
Vasculitis - rare non-embolic cause
How would you manage a TIA?
Control CVD RFs - antihypertensives, statins, diabetic medication, smoking cessation
Antiplatelets - aspirin 300mg 2 wks, then clopidogrel 75mg OD
Anticoagulation if cardiac emboli
Carotid endarterectomy in 2wks if 1st TIA and can tolerate (preferred to carotid angioplasty with stent)
Advise not to drive for 1 month
What tool gives the risk for a stroke following a TIA?
ABCD2 score
What is a TIA?
= transient ischaemic attack
ischaemic/embolic neurological event with symptoms lasting less than 24 hours
What factors are considered on ABCD2 score?
Age >60 Blood pressure >140/90 Clinical: unilateral weakness, speech disturbance without weakness Duration: symptoms 10-59 mins or >1h Diabetes
What is a high risk score on the ABCD2?
4+ = high risk, see specialist within 24h
What are the typical features of meningitis?
Rapid onset headache Fever Photophobia Stiff neck Purpuric rash Coma
What are the typical features of encephalitis?
Rapid onset headache
Fever
Odd behaviour
Fits or reduced consciousness
Give the differentials for a rapid onset headache
Subarachnoid haemorrhage Meningitis Encephalitis Post-coital headache Cluster headaches
Give the features of venous sinus thrombosis
subacute, gradual onset headache
papilloedema
Give the features of sinusitis
Dull, constant headache over frontal or maxillary sinuses with tenderness
Ethmoid or sphenoid pain is deep in midline at nose root, common with coryza
Postnasal drip
Pain worse on bending over
Lasts 1-2 weeks
Give the features of intracranial hypotension
CSF leakage e.g. iatrogenic after LP, epidural
Headaches worse on standing
How would you manage intracranial hypotension due to CSF leak post-LP?
Epidural blood patch over leak
Conservative management with IV fluids and caffeine fails
Give the features of a tension headache
Tight band
Bilateral
Non-pulsatile
Scalp muscle tenderness
Give the features of migraines
Prodrome - precedes by days (mood changes, tired)
Visual or other aura lasting 15-30 mins, followed by
(1h unilateral, throbbing headache)
Nausea, vomiting, photophobia, phonophobia
Allodynia
Give some examples of visual aura experience in migraines
Chaotic distorting Melting and jumbling of lines Dots Zigzags Scotomata (blind spots) Hemianopia
What aura might you get with migraines other than visual
Paraesthesiae spreading from fingers to face
Dysarthria, ataxia, ophthalmoplegia, hemiparesis
Dysphasia or paraphasia
What are some triggers of migraines?
CHOCOLATE
C = chocolate, H = hangovers, O = orgasms, C = cheese/caffeine, O = oral contraceptive, L = lie-ins, A = alcohol, T = travel, E = exercise
What is the diagnostic criteria for migraines with no aura?
> 5 headaches lasting 4-72hrs with nausea/vomiting/photo/phonophobia + 2 of
unilateral, pulsating, impairs routine activity
How would you manage migraines?
Avoid triggers (consider progesterone only/non-hormonal contraceptives in young women) Prophylaxis = propanolol/topiramate/amitryptiline Attack = Oral triptan + NSAID/paracetamol, anti-emetic Other = warm or cold packs to head, rebreathing into paper bag, Butterbur extracts, riboflavin supplements, acupuncture, transcutaneous nerve stimulation
Give some contraindications and side effects of triptans
CI = IHD, coronary spasm, uncontrolled HTN, recent lithium, SSRI, ergot SE = arrhythmias, angina, MI
Give features of a headache due to raised ICP
chronic, progressive headache - worse on waking, lying, bending forward or coughing
AS - vomiting, papilloedema, seizures, false localising signs, irritability
If suspect ICP, what investigation would you do first and why?
Imaging - CT/MRI FIRST before LP (risk herniation/coning)
If no abnormalities, consider idiopathic intracranial hypertension
What medications can cause medication overuse headache/analgesic rebound headache?
Combined analgesics (paracetamol + codeine/opiates)
Ergotamine
Triptans
Episodic headache becomes chronic daily headache
What are the symptoms of cluster headaches?
Rapid onset excruciating pain around orbit unilaterally
Eye watery and bloodshot, lid swelling
Facial flushing, rhinorrhoea, miosis or ptosis
Duration 15-180 mins, OD or BD, often nocturnal
Clusters last 4-12 weeks, then pain-free periods of months-2 years
How would you treat cluster headaches?
Acute attack - 100% O2 for 15 mins via non-rebreathe mask + sumatriptan at onset
Prophylaxis - avoid triggers. Consider corticosteroidds, verapamil, lithium
What are the symptoms of trigeminal neuralgia?
Intense unilateral stabbing pain lasting seconds in trigeminal nerve distribution
Often mandibular or maxillary distributions
Triggers - washing area, shaving, eating, talking, dental prostheses
How would you manage suspected trigeminal neuralgia?
MRI - to exclude secondary inflammatory or structural causes
Carbamazepine, lamotrigine, phenytoin or gabapentin
Microvascular decompression
What is the minimum GCS score and what is the maximum?
Min = 3 Max = 15
Give some common causes of coma
Drugs//toxins/opiates/ethanol Anoxia Mass lesions e.g. bleeds, SAH Infections (HSE, meningitis Infarcts of brainstem Metabolic (DKA, wernicke's) Epilepsy
Give less common causes of coma
Tumour Carbon monoxide Venous sinus occlusions Hypothermia Psychiatric catatonia Pituitary apoplexy Fat embolism
How does blood appear on CT in sub/extradural haemorrhage initially and then after some time?
High density or white initially
Then becomes hypodense after time
How would you investigate for a non-haemorrhagic ass lesion?
CT with contrast - illuminates wall of mass
How would an MCA infarct appear on a CT? What criteria would make it a malignant MCA infarct?
Reduced white-grey matter differentiation and sulcal effacement
if 2/3 artery territory infarcted = malignant
What would you expect to see on a lumbar puncture without culture in bacterial meningitis?
Raised protein, decreased glucose
What is the pathophysiology and signs for phenobarbital overdose?
GABA-A chloride channels open longer
Cerebellar signs and diplopia
Coma, respiratory depression, death
What are the signs of CO poisoning?
headache, nausea, confusion, syncope, convulsions, coma, death
low CO levels can still kill if have IHD
What symptoms and signs can amphetamines and other stimulants cause?
seizures
psychosis
ischaemic stroke, intracranial haemorrhage
angiitis (stroke, mononeuritis multiplex)
rhabdomyolosis
delirium, coma
posterior reversible encephalopathy syndrome
What neurological events can occur shortly after ingesting cocaine?
Seizures, stereotypic movements (tic-like)
Anxiety, confusion, paranoia, visual hallucinations
Stroke - usually haemorrhagic
What are the symptoms of ethanol overdose?
ataxia
nystagmus
dysarthria
coma
What are the symptoms of ethanol/alcohol withdrawal
a) within 6-8hrs?
b) within 24 hrs?
c) within 3-5 days?
a) tremor, anxiety, nausea
b) hallucinations (visual, olfactory, auditory)
c) delirium tremens - hyperactivity (motor+sensory) and altered mental state
How would you treat alcohol withdrawal?
Thiamine, then glucose, multivitamins
Benzodiazepine (Lorazepam), Chlordiazepoxide
Fluids - check hydration, K and Mg
Check clotting, monitor glucose
What is the main concern that chronic ethanol overuse can cause? What are features of this?
= Wernicke's encephalopathy Thiamine deficiency Ophthalmoparesis Ataxia Confusion 25% get Korsakoff's psychosis
What other conditions can chronic ethano overuse cause except for WE?
cerebellar degeneration - LL and trunk ataxia
peripheral neuropathy
amblyopia
central pontine myelinosis (if hyponatraemia corrected too rapidly)
dementia
tremor
What is a sub-hyaloid haemorrhage?
Intra-ocular bleed between retina and posterior layer of vitreous
What features do you see in 3rd CN palsy?
Parasympathetic palsy = fixed dilated pupil, droopy eyelid
Sympathetic = Horner’s syndrome, partial ptosis
What are the features of Horner’s syndrome?
Loss of sympathetic oculomotor function - triad = Constricted pupil (miosis) Partial ptosis Hemifacial sweating (anhidrosis) Sometimes enophthalmos
What might you see on an LP after a sub-arachnoid haemorrhage? When should a CT be done?
CT most sensitive within 24 hrs
LP = CSF xanthochromia (yellow due to bilirubin) - most sensitie within 2 weeks
What are 2 common treatments for a sub-arachnoid haemorrhage?
- surgical clipping
2. Endovascular coiling
When would maximum weakness need to occur in acute paresis?
Within 4 weeks
What features may suggest a brainstem lesion for UMN weakness?
Cranial nerve involvement
Horner’s syndrome
Internuclear ophthalmopegia
What features may suggest a spinal cord injury for UMN weakness?
Bilateral weakness
Radicular symptoms may give level
Sensory loss below level
What are some acute causes of weakness?
Vascular (haemorrhagic or embolic)
Compression (haemorrhagic usually)
Hypoglycaemia
What are some subacute causes of weakness?
UMN = mass lesion, subdural, inflammatory, infection, metabolic LMN = GBS, peripheral neuropathy, myopathy, MG, malignant meningitis
Why is it useful to check if weakness involves dorsal and spinothalamic tracts?
If it does, then lesion must be beyond arterial distribution because each tract has separate arterial blood supply
What is spinal shock?
Acute complete transverse lesion across spinal cord can cause spinal shock
Flaccid weakness below lesion but no UMN signs
What are the features of spinal cord compression?
UMN deficit below lesion Sensory impairment below lesion Possible spinal shock if acute Lhermitte's phenomenon Painless atonic bladder Urgency Impotence Respiratory paralysis if high cervical lesion
What are the 4 types of cancer that can metastasise to the spine?
Lung
Breast
Prostate
Kidney
What are some primary tumours that could be in the spine?
Neuroma Meningioma Glioma Sarcoma Ependymoma
What are some non-compressive causes of myelopathy?
Inflammation
Infarction
MND
Syrinx (fluid-filled cavity in spinal cord)
AVM, spinocerebellar syndromes, radiation, fluorosis, sarcoidosis
What supportive treatments might you do for GBS?
Thromboprophylaxis
Dysautonomia - 2-4hrly BP, cardiac monitor
Monitor FVC, RR
Pain - opiates, amitryptiline
Rehab - splints, pressure sores, nutrition
What types of immunotherapy can you give for GBS?
IV Ig
Plasma exchange
monoclonal antibodies??
What is status epilepticus?
When a seizure lasts longer than 5 minutes or when seizures occur close together with no recovery between them
How would you manage a sub-arachnoid haemorrhage?
Re-examine neuro often - BP, pupils, GCS
Repeat CT if deteriorating
Keep hydrated - maintain perfusion
Nimodipine - prevent vasospasm, cerebral ischaemia
Surgery - Endovascular coiling or surgical clipping
Give some primary and secondary causes of orthostatic hypotension
Primary = MSA, PoTS Secondary = diabetes, medication, Parkinsons
Give some potential triggers of epileptic seizures
sleep deprivation
flashing lights
menstruation
alcohol/alcohol withdrawal
How useful are EEGs in diagnosing epilepsy?
Not very! Should be done after ECG, bloods, imaging
Some may have abnormal epileptic wave forms inter-ictally, but some none unless during seizure
Often not useful in outpatients, send home with EEG
Can provoke seizure - sleep deprivation, hyperventilation, flashing lights
Useful if not sure if in status epilepticus
What is the role of MRI/CT in patients with recurrent blackouts?
Indications: FOCAL or new onset seizures >25yrs old
MRI preferred over CT - CT if urgent e.g. bleed
None needed if GTC, MC, absence seizures
Give pathophysiology of epileptic seizures
Excitatory and inhibitory phases
Prolonged depolorisation in neurones which spreads to adjacent areas +
failure of GABA transmission
Give treatment of focal seizures
Carbamazepine
Lamotrigine
If refractory - vagal nerve stimulator
Give treatment of tonic clonic seizures
Valproate (not if young woman)
Lamotrigine
Give treatment of myoclonic seizures
Valproate
Levoteracetam
Topiramate
What does a visual acuity of 6/60 mean?
Able to see clearly at 6m, but average population can see clearly at 60m distance
Describe Marcus-Gunn pupil
= relative afferent pupillary defect (RAPD)
Pupil constricts less/dilates when light moved from unaffected eye to affected eye
Indirect and direct responses diminished when light in affected eye, but normal when light shines on unaffected eye
Efferent constriction is OK, problem is with afferen pathway - light detection
What is the role of pinhole correction?
To exclude corneal defects, myopia, hypermetropia
What are the different causes of relative afferent pupillary defect?
Optic nerve lesion or neuritis
Vascular causes
Retinal detachment or vitreous haemorrhage
Acute angle closure glaucoma
What might you experience with retinal detachment or vitreous haemorrhage?
Flashes/floaters in vision
Decrease in vision
Vitreous haemorrage associated with diabetic retinopathy, macular degeneration
What might you experience with acute angle closure glaucoma?
painful red eye, nausea, vomiting
What causes anterior ischaemic optic neuropathy? What might you see on examination?
ischaemia of posterior choroidal artery that supplies optic nerve head (optic disc)
Swollen optic disc on fundoscopy
Think GCA
What are the features of central retinal artery occlusion?
Occlusion of central retinal artery which supplies retina (branch of ophthalmic artery)
PALE optic disc
CHERRY RED SPOT on macula
What are the features of central retinal vein occlusion?
Dilated branch veins
Multiple retinal haemorrhages
Cotton wool patches
STORMY SUNSET
What are the features of optic neuritis?
Painful eye movements, blurred vision over few days, exacerbated by heat/exercise, afferent pupillary defect, dyschromatopsia
Inflammation of optic nerve at the optic nerve head (optic disc)
If behind optic disc - often normal in 2/3
Give an example of a transient painless monocular loss of vision
Amaurosis fugax
Migraines
Give an example of a permanent painless monocular loss of vision
central retinal artery occlusion
central retinal vein occlusion
Give an example of permanent painful monocular loss of vision?
Optic neuritis
Acute angle closure glaucoma
Vitreous haemorrhage
What investigation might you do to confirm optic neuritis?
Visual evoked potentials - EEG during light stimulus
Give some causes of optic neuritis
- Multiple Sclerosis!
2. Lyme, syphilis, HIV, B12 deficiency, GCA
What is the prognosis of optic neuritis?
50% develop MS
Vision improves over 6 weeks, may have ongoing dyschromatopsia or decreased acuity
Give the diagnostic criteria for multiple sclerosis
McDonald’s criteria
Inflammatory plaques of demyelination disseminated in SPACE and TIME causing focal loss with relative preservation of axons
>30 days between attacks
>1hr duration attacks
Describe the pathophysiology of multiple sclerosis
Relapse-remission part - inflammatory response causes demyelination of axons leading to relapse and remitting symptoms
Neurodegenerative part - demyelination heals poorly leading to axonal degeneration causing progressive and fixed deficits
What are the 4 types of multiple sclerosis?
- Benign
- Relapsing-remitting
- Secondary chronic progressive
- Primary progressive
What is a pseudo-relapse of MS?
flare-up of symptoms not due to MS, but due to other factors e.g. stress, infection
Give some symptoms of MS
Unilateral vision loss Fatigue, cognitive impairment Weakness (pyramidal), spastic paresis Sensory disturbance Cerebellar - ataxia, nystagmus, intention tremor, vertigo, dysarthria Bladder involvement/sexual dysfunction Lhermitte's Uhtoff's Cranial nerve/bulbar dysfunction - swallowing, facial movements, eye movements
Give some signs of MS
UMN signs - spastic paresis, brisk reflexes
Sensory loss
Cerebellar signs
Optic atrophy, RAPD
Internuclear ophthalmoplegia
CN involvement - swallowing, face and eye movements
Give the investigations and findings for MS
- MRI - T2 weighted - white matter lesions often peri-ventricular, or in brainstem, cerebellum, spinal cord
- LP - oligoclonal IgG bands
- Visual evoked potentials - delayed
- MRI with contrast - lesions in last 3 months more enhanced - to see if disseminated in time
What is Lhermitte’s sign?
radiating pain from back of neck down spine to arms and legs on flexion of neck
(plaques in spinal cord cause nerve irritation)
What is Uhtoff’s sign?
Worsening of neurological symptoms in demyelinating conditions due to heat
Give short-term management of multiple sclerosis
RULE OUT INFECTION
1g methylprednisolone IV 24 hrs or PO for 3-5 days
(don’t use more than once a year)
What are the short-term risks of high dose steroid treatment?
delirium/psychosis
immunosuppression
peptic ulcers
pancreatitis
What is the long-term conservative management for MS?
Smoking cessation
Psychological support
Regular exercise
PT/OT/SLT
What medical long-term disease-modifying management is there for MS?
Dimethylfumarate - mild/moderate relapse-remitting
Alemtuzumab or Natalizumab
(Interferon beta and glatiramer - not recommended)
What medical symptomatic control is there for MS?
Baclofen/gabapentin - spasticity
Botulinum type A injections - tremor
Amantadine - fatigue
Tolterodine - urinary frequency/urgency
Duloxetine - bladder instability, neuropathic pain
Gabapentin, pregabalin, amitryptiline - neuropathic pain
What do you see in internuclear ophthalmoplegia?
Ipsilateral eye cannot adduct while contralateral eye abducts with nystagmus
What causes INO?
Communication between CN3 and CN6 disrupted due to lesion in medial longitudinal fasciculus
What are the top 2 risk factors for stroke?
- Hypertension
2. Atrial Fibrillation
What is the CHADS-VASC Score for?
To determine stroke risk in a patient with atrial fibrillation
What are the parameters on the CHADS-VASC score?
CHF, HTN, Age, Diabetes, Stoke/TIA/VTE history, Vascular disease, Sex
What type of seizures in status epilepticus are an emergency and which type are not?
Convulsive = emergency Non-convulsive = not emergency
What are T1 and T2 in status epilepticus?
T1 = 10 mins, where unusually prolonged T2 = 30 mins, where expect impairment
How would you manage status epilepticus?
- 2 doses benzodiazepines 10 mins apart (IV/buccal midazolam, IV lorazepam/diazepam)
- If not work - valproate or levoteracetam, consider glucose + thiamine
- If not work - anaesthesia
Give mechanism of action of benzodiazepines
Increase frequency of chloride ion channel opening, increasing GABA inhibitory effect
What investigations would you do for status epilepticus?
Toxicology, FBC, U+E, CRP, BM, Calcium
CT head (quick) especially if focal neurological signs
EEG during if not sure it’s status epilepticus
What is true vertigo?
Hallucination of movement, often rotatory, of the patient and their surroundings
What might be some associated symptoms of vertigo?
Difficulty walking or standing Relief on lying, sitting still - worse on movement Nausea, vomiting Pallor, sweating Possible hearing loss, tinnitus
Give some causes of true vertigo
- vestibular neuritis
- BPPV
- Meniere’s disease
- Ototoxicity
- Acoustic neuroma
- Trauma
- HZV
- Central/brainstem causes
- Alcohol intoxication
What does BPPV stand for? What are its typical features?
Benign paroxysmal positional vertigo
- true vertigo on head MOVEMENT, stops when head still
- decreases over time due to brain adapting (self-terminating)
- Fatigueable nystagmus on Hall-pike manoeuvre is diagnostic
What manoeuvre helps to resolve BPPV?
Epley manoeuvre - clears debris from semicircular canals of ears
Give another name for acute labrynthitis. What are its typical features
= vestibular neuronitis Abrupt onset severe vertigo, nausea and vomiting Prostration No deafness or tinnitus Self-limiting (complete 3-4wks)
What causes acute labrynthitis? How would you manage?
Virus
Vascular lesion
Mx: Reassure and sedation if severe
What are the features of Meniere’s disease?
Increased pressure in endolymphatic system of inner ear
Recurrent attacks vertigo lasting >20 mins, fluctuating/permanent sensorineural hearing loss, tinnitus, aural fullness and falling to one side
How would you manage Meniere’s disease?
Bed rest and reassurance in acute attacks
If prolonged - antihistamine (cinnarizine or buccal prochlorperazine if severe)
What ototoxic drugs can cause vertigo and/or deafness?
Aminglycosides e.g. gentamicin
Loop diuretics
Cisplatin
What type of tumour is an acoustic neuroma?
Schwannoma arising from vestibular nerve
Accounts for 80% cerebellopontine angle tumours
How do acoustic neuromas present?
Unilateral hearing loss
Followed by vertigo
Can be predicted with serial MRIs, slow growth
Once progressed - CN5,6,9,10 may be affected and Ipsilateral cerebellar signs
What kind of trauma might cause vertigo?
Trauma affecting petrous temporal bone or cerebello-pontine angle, may damage CN8 causing vertigo, deafness or tinnitus
What features might you see in Herpes zoster external meatus infection?
Facial palsy, deafness, tinnitus and vertigo
What test would you do to check vertigo is not from a central/brainstem lesion?
Dolls-eye test
Move head and ask patient to keep eyes fixed on stationary object. If eyes move with head rather than stay fixed = negative - likely vestibular system and brainstem intact??
What are the features of congenital nystagmus?
Usually bidirectional, pendular (no particular beating direction)
Compensation - usually no vision effect
What is the inheritance pattern of Friedrich’s ataxia? What are its typical features?
autosomal recessive
Ataxia, sensory loss arms and legs, impaired speech
Spinocerebellar degeneration
What is the inheritance pattern of Spino-Cerebellar Ataxia? What is the commonest type and its features?
Autosomal dominant
SCA 6 is commonest in UK
Pure ataxia
What inheritance pattern is ataxia telangiectasia? What are its features?
Autosomal recessive
Progressive ataxia starting in childhood
Susceptible to cancer and oculomotor apraxia
Give some toxicities that may cause ataxia
Alcohol
Phenytoin and anti-convulsants
Lithium
Amiodarone
What is a degenerative cause of cerebellar ataxia?
MSA - either MSA P or MSA C
Early onset speech impairment and autonomic decline
What are immune-mediated causes of cerebellar ataxia?
post-viral cerebellitis - VZV in children
Paraneoplastic degeneration
Gluten ataxia (with/without coeliac)
Primary autoimmune ataxia
What might down-beat nystagmus mean?
Obstruction in foramen magnum
Give some features of Bell’s palsy
= idopathic facial nerve palsy
Abrupt onset complete unilateral facial weakness at 24-72hrs
Ipsilateral numbness or pain around the ear
Decreased taste
Hypersensitivity to sounds
How would you differentiate between LMN (Bell’s palsy) and UMN facial weakness?
UMN weakness - forehead sparing as bilateral innervation
LMN weakness - no forehead sparing, unable to wrinkle forehead
What conditions might you see a high steppage gait in?
Peripheral neuropathy
L5 radiculopathy, common peroneal nerve palsy
What gait might you see in Parkinson’s disease?
wide base, shuffling, stooped posture, asymmetrical reduced armswing
Freezing at obstacles
Apraxia - struggle to stand and start walking
What gait might you see in hemiplegia?
Arm flexed and ipsilateral leg circumduction
What gait might you see in proximal myopathy?
Waddling gait
Give the triad of extrapyramidal features of Parkinson’s disease
- Tremor
- Hypertonia/rigidity “cogwheel rigidity”
- Bradykinesia (slow movements, decrease in amplitude with repetition, festinant gait, freezing at doors and obstacles, expressionless face)
What is the cause of Parkinson’s disease?
Loss of dopaminergic neurons in substantia nigra, associated with Lewy bodies in basal ganglia, brainstem and cortex
Mostly sporadic, some genetic
Give some non-motor features of Parkinson’s
Autonomic dysfunction - postural hypotension, constipation, urinary frequency/urgency, saliva dribbling Sleep disturbance Reduced sense of smell Depression Dementia Psychosis
How is Parkinson’s disease diagnosed?
Exclude cerebellar and frontotemporal disease
Clinical diagnosis and based on response to dopaminergic treatment
If suspect alternative cause - MRI or DaTscan, PET
What is the pharmacological management for Parkinson’s disease?
Symptom control, not reversing disease so start late as tolerance increases and SE get worse with higher doses
Levodopa
Dopamine agonists to delay levodopa initiation - ropinirole, pramipexole
Apomorphine
Anticholinergics
MAO-B inhibitors
COMT inhibitors
Why should you not withdraw Levodopa suddenly?
Acute kinesia risk
Neuroleptic malignant syndrome
What is some non-pharmacological management of Parkinsons?
Deep brain stimulation (if partly dopamine-responsive)
Surgical ablation of overactive basal ganglia circuits
