AS PACES Neurology Flashcards
Parkinson Plus syndromes
Progressive Supranuclear Palsy
Multiple system atrophy
Lewy Body Dementia
Corticobasilar Degeneration
Hx - Parkinsonism
Tremor, rigidity, akinesia
Autonomic (postural hypotension, urinary problems, constipation, hypersalivation)
ADL - writing, buttons, shoe laces
Sleep - insomnia, daytime sleepiness
Complications - depression / anxiety, drug SE
Cause - sudden onset, eye and balance problems, visual hallucinations, memory impairment
Parkinson’s imaging
CT/MRI to exclude vascular cause
DaTscan - Ioflupane I123 injection, binds Da neurons allowing visualisation of substantia nigra.
Managing Parkinson’s (IPD)
MDT - neurologist, PD nurse, OT, physio, GP carers
Depression screen
L-DOPA + Carbidopa Da agonists - ropinerole, pramipexole MOA-B inhibtors - rasagiline COMT inhibitors - tolcapone Anti muscarinics - procyclidine
Nausea - Domperidone
Psychosis - Quetiapine
Depression - Citalopram
Idiopathic Parkinson’s Disease - Pathophysiology
2% prevalence >65
Destruction of DA neurones in pars compacta of substantia nigra
B anyloid plaques
Hyperphosphorilated tau
Features of idiopathic parkinson’s disease
TRAPPS PD
Tremor Rigitidy (cog-wheel) Akinesia (slow initiation, difficulties with repetitive movements, micrografia, monotonous voice, mask-like face Postural instability (stooped gait) Sleep disorders (insomnia) Psychosis Depression & Dementia
L-DOPA SE mnemonic
DOPAMINE Dyskinesia On-off phenomena - motor fluctuations Psychosis ABP decrease Mouth dryness Insomnia N&V EDS (excessive daytime somnolence)
MSA
Papp-Lantos Bodies - alpha synuclein inclusions in glial cells
Autonomic dysfuntion - postural hypotension
Parkinsonism
Cerbellar ataxia
PSP
Postural instability - falls
Vertical gaze palsy
Pseudobulbar palsy - speech and swallowing patterns
Parkinsonism - symmetrical onset
Borticobasilar degeneration
Unilateral parkinsonism esp rigidity
Aphasia
Astereognosis - cortical sensory loss (alien limb phenomenon)
Lewy Body Dementia
Fluctuating cognition
Visual hallucinations
Parkinsonism
Benign Essential Tremor
AD
With movement, worse with anxiety / caffeine
Better with ETOH
Cerebellar Syndrome - mnemonic
DAISIES Demyelination Alcohol Infarct: brainstem stroke SOL: e.g. schwannoma + other CPA tumours Inherited: Wilson’s, Friedrich’s, Ataxia Telangiectasia, VHL Epilepsy medications: phenytoin System atrophy, multiple
Friedrich’s Ataxia - pathophysiology and features
AR mitochondrial disorder. Progressive degeneration of dorsal column, cerebellum, cortispinal tracts. Onset teens. Ass. HOCM and dementia
Pes Cavus, bilateral cerebellar ataxia
Leg wasting + no reflexes but planters ok
Loss of vibration and proprioception
High arched palate, optic atrophy
Spastic paraperesis - DDx
MS, cord compression/trauma, cerebral palsy
If mixed UMN & LMN signs - MND, SCDC (B12), Friedrich’s Ataxia
What is syringomyelia?
Cyst/cavity forms within spinal cord. Can expand/elongate with time, destroying spinal cord.
Commonly in C spine
Symptoms classic for years but can worsen in increased pressure (sneeze, cough)
Causes of syringomyelia
Blocked CSF flow from posterior fossa - arnold chiari malformation
Masses
Cord trauma, myelitis, AVMs
Symptoms of syringomyelia
Loss of pain and temperature sensation (burn scars). Touch, proprioception, vibration all OK. Usually upper limbs and chest in "cape" distribution Wasting/weakness of hands and claw hand Areflexia in upper limb Charcot joints - shoulder and elbow UMN in lower limbs Horner's syndrome
Arnold - Chiari malformation
Downward displacement of cerebellum through foramen magnum, can cause obstruction to CSF flow (non communicating hydrocephalus)
Bamford Classification of Stroke
TACS - total anterior circulation stroke. (MCA & ACA territory) Hemiparesis ± hemisensory deficit, homonymous hemianopia, higher cortical dysfunction (aphasia, neglect, apraxia)
PACS - 2/3 TACS criteria, e.g. homonymous hemianopia and higher cortical dysfunction
POCS - vertebrobasilar territory (cerebellar syndrome, homonymous hemianopia)
LACS - lacunal infarct around basal ganglia, internal capsula, thalamus, pons.
Criteria for stroke thrombolysis
18-80 years <4.5 hours since onset. tPA (alteplase). CT 24h post to look for haemorrhage
Stroke Workup
ECG, Bloods (FBC, U&E, glucose, lipids, clotting, ESR, ANA)
Thrombophilia screen (fVL, protein C and S, lupus anticoagulant, anti cardiolipin)
CXR
Carotid Doppler
Secondary prevention of stroke
Statin
Aspirin 300mg 2/52 then clopidogrel 75mg OD
Warfarin perhaps
Carotid endarterectomy
Uhtoff’s phenomenon
In MS optic neuritis, vision worse with heat (bath, meal, exercise)
INO = disruption of:
medial longitudonal fasciculus
MS - treatment
Acute - methylprednisolone 1g/24h IV OD for 3 days (no long term benefit, reduces duration and severity of attack
Relapse - DMARDS (INFB decreases relapse by 30%) and biologics (natalizumab anti VLA4 and Alemtuzumab antiCD52)
Poor prognostic signs in MS
Older Male Motor signs at onset Frequent relapses Many lesions
Classification of MND
50% Amyotrphic Lateral Sclerosis (UMN + LMN + fasciculations)
10% Progressive Bulbar Palsy (C9-C12)
10% Progressive Muscular Atrophy (LMN signs only)
30% Primary Lateral Sclerosis (mainely UMN, spastic leg weakenss, pseudobulbar palsy, no cognitive decline)
Ddx proximal myopathy
Inherited (muscular dystrophy)
Inflammation (polymyositis, dermatomyositis)
Endocrine (cushing’s, acromegaly, thyrotoxicosis, osteomalacia, diabetic amyotrophy)
Drugs (alcohol, statins, steroids)
Malgnancy (paraneoplastic)
Charcot Marie Tooth - examination findings
Pes cavus, symmetrical distal muscle wasting (claw hand, champagne bottle leg), thick nerves (esp common peroneal around fibula)
High stepping gait + foot drop
Weak foot and toe dorsiflexion
Absent ankle jerks
Variable loss of sensation in stocking distribution
Charcot Marie Tooth - investigations and pathophysiology
Inherited sensorimotor neuropathy
Type 1 - commonest, demyelinating, AD mutation in peripheral myelin protein 2, decreased conduction velocity
Type 2 - axonal degeneration, AD, decreased amplitude
Myasthenia gravis - examination
Inspect - thymectomy scar Eyes - B/L ptosis, worse on sustained upward gaze, complex opthalmoplegia Face - snarling smile Voice - nasal, ask count back 50-->1 Limbs - fatiguability Completion - respiratory muscle function
Myasthenia Gravis - Investigations and Management
Abs - Anti AChR
EMG - fatiguability
Tensilon test - improvement with edrophonium (anticholinesterase)
TFTs (Grave’s 5%
CT mediastinum (thymoma 10%)
Acute –> plasmapheresis / IVIG, monitor FVC and consider ventilation
Chronic –> Immunosupression with steroids and azathioprine, thymectomy
Lambert Eaton Myaesthenic Syndrome
Abs against Voltage Gated Calcium Channels
Often paraneoplastic SCLC
Lower limb girdle weakness
Weakness improves with repeated testing
DDs B/L ptosis
MG Myotonic dystrophy Congenital Senile B/L horner's (rare)
GBS - pathophysiology and features
Acute AI demyelinating polyneuropathy 2* to molecular mimicry from C. jejuni, mycoplasma, CMV, EBV
Symmetrical ascending flaccid paralysis, paraesthesia
GBS - investigations and management
Infection - stool MC+S, anti ganglioside Abs, high CSF protein on LP, nerve conduction
Support - airway and breathing (ITU?), analgesia, antithrombotic (TEDS, LMWH), autonomic (ionotropes)
Immunosupression - IVIG, plasma exchange
Physiotherapy
85% complete recovery, 10% unable to walk at 1 year, 5% mort
DDx VII palsy
Bell's 75% Vascular MS SOL CPA? - acoustic neuroma Ramsay Hunt
DDx B/L VII palsy
B/L bells, sarcoidosis, BGS, Lyme, MG, myotonic dystrophy
VII palsy - investigations and management
Glucose, HBA1c, VZV serology, anti ACh receptor,
Mx - prenisolone within 72h, acyclovir
Eye protection, artificial tears, tape to close eyes at night
Bells palsy - prognosis
Complete - 80% full recovery, rest either delayed or some residual weakness
VII nerve palsy - complications
Synkiesis - blinking causes up turing of mouth
Crocodile tears - eating stimulates unilateral lacrimation (eyes)
III pals - medical or surgical?
Parasympathetic fibres originate in Edinger Westphal Nucleus and run on periphery of oculomotor nerve. Receive rich blood supply from external pial vessels. These fibres are affected late in ischaemic causes (medical) but early in compression (surgical).
Therefore is pupil dilation = surgical but if not then medical
Causes of Marcus Gunn Pupil
RAPD MS Glaucoms Leber's hereditary optic neuropathy (mitochondrial disease) Ethambutol, lead, B12 def SOL (compression) - optic glioma, pituitary adenoma, paget's Vascular - DM Infection - VZV, TB Papilloedema
Visual pathway
Retina Optic Nerve Optic Chiasm Optic tract Lateral Geniculate Nucleus of thalamus Optic radiation (superior is temporal, inferior is parietal) Visual cortex
Causes of conductive hearing loss
Preauricular
Obstruction - wax, foreign body
Tympanic membrane perforation
Ossicle defectos - otosclerosis, infection
Causes of SNHL
Defects of cochlea
Congenital - alports (SNHL + haematuria)
Acquired - gentamicin, meningitis, measles, vestibular schwannoma
Dysphasia
Impairment of language
Expressive - Broca’s area (frontal lobe), non-fluent but comprehension intact
Receptive - Wernicke’s (temporal lobe), fluent but meaningless, no comprehension
Conductive - arcuate fasciculus connecting Broca’s to Wernicke’s, comprehension ok, unable to repeat words or phrases
Dysarthria
Impaired articulation
Pseudobulbar - spastic dysarthria with B/L UMN lesions (CVA, MS, MND)
Bulbar - U/L LMN weakness (brainstem infarct, MND, GBC
Cerebellar (slurred, drunk speech)
Myotonic dystrophy
Myopathic facies, frontal balding, cataracts, decreased power in face and bulbar muscles
Wasting of face, neck and distal arms, distal weakness
Absent reflexes
Hand grip myotonia and percussion myotonia
AD inheritance
UMN vs LMN location
Pre central gyrus motor cortex Corona radiata Internal capsule Midbrain Pons Medulla --> DECUSSATES Cervical CST Synapses at anterior horn cell
Anterior horn cell Inter-vertebral foramen Brachail plexus Peripheral nerve NMJ
