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Final Prep > AS PACES Neurology > Flashcards

AS PACES Neurology Flashcards

1
Q

Parkinson Plus syndromes

A

Progressive Supranuclear Palsy
Multiple system atrophy
Lewy Body Dementia
Corticobasilar Degeneration

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2
Q

Hx - Parkinsonism

A

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

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3
Q

Parkinson’s imaging

A

CT/MRI to exclude vascular cause

DaTscan - Ioflupane I123 injection, binds Da neurons allowing visualisation of substantia nigra.

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4
Q

Managing Parkinson’s (IPD)

A

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

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5
Q

Idiopathic Parkinson’s Disease - Pathophysiology

A

2% prevalence >65

Destruction of DA neurones in pars compacta of substantia nigra
B anyloid plaques
Hyperphosphorilated tau

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6
Q

Features of idiopathic parkinson’s disease

A

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
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7
Q

L-DOPA SE mnemonic

A 
DOPAMINE
Dyskinesia
On-off phenomena - motor fluctuations
Psychosis
ABP decrease
Mouth dryness
Insomnia
N&V
EDS (excessive daytime somnolence)
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8
Q

MSA

A

Papp-Lantos Bodies - alpha synuclein inclusions in glial cells
Autonomic dysfuntion - postural hypotension
Parkinsonism
Cerbellar ataxia

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9
Q

PSP

A

Postural instability - falls
Vertical gaze palsy
Pseudobulbar palsy - speech and swallowing patterns
Parkinsonism - symmetrical onset

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10
Q

Borticobasilar degeneration

A

Unilateral parkinsonism esp rigidity
Aphasia
Astereognosis - cortical sensory loss (alien limb phenomenon)

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11
Q

Lewy Body Dementia

A

Fluctuating cognition
Visual hallucinations
Parkinsonism

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12
Q

Benign Essential Tremor

A

AD
With movement, worse with anxiety / caffeine
Better with ETOH

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13
Q

Cerebellar Syndrome - mnemonic

A 
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
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14
Q

Friedrich’s Ataxia - pathophysiology and features

A

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

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15
Q

Spastic paraperesis - DDx

A

MS, cord compression/trauma, cerebral palsy

If mixed UMN & LMN signs - MND, SCDC (B12), Friedrich’s Ataxia

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16
Q

What is syringomyelia?

A

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)

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17
Q

Causes of syringomyelia

A

Blocked CSF flow from posterior fossa - arnold chiari malformation
Masses
Cord trauma, myelitis, AVMs

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18
Q

Symptoms of syringomyelia

A 
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
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19
Q

Arnold - Chiari malformation

A

Downward displacement of cerebellum through foramen magnum, can cause obstruction to CSF flow (non communicating hydrocephalus)

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20
Q

Bamford Classification of Stroke

A

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.

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21
Q

Criteria for stroke thrombolysis

A

18-80 years <4.5 hours since onset. tPA (alteplase). CT 24h post to look for haemorrhage

22
Q

Stroke Workup

A

ECG, Bloods (FBC, U&E, glucose, lipids, clotting, ESR, ANA)
Thrombophilia screen (fVL, protein C and S, lupus anticoagulant, anti cardiolipin)
CXR
Carotid Doppler

23
Q

Secondary prevention of stroke

A

Statin
Aspirin 300mg 2/52 then clopidogrel 75mg OD
Warfarin perhaps
Carotid endarterectomy

24
Q

Uhtoff’s phenomenon

A

In MS optic neuritis, vision worse with heat (bath, meal, exercise)

25
Q

INO = disruption of:

A

medial longitudonal fasciculus

26
Q

MS - treatment

A

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)

27
Q

Poor prognostic signs in MS

A 
Older 
Male
Motor signs at onset
Frequent relapses
Many lesions
28
Q

Classification of MND

A

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)

29
Q

Ddx proximal myopathy

A

Inherited (muscular dystrophy)
Inflammation (polymyositis, dermatomyositis)
Endocrine (cushing’s, acromegaly, thyrotoxicosis, osteomalacia, diabetic amyotrophy)
Drugs (alcohol, statins, steroids)
Malgnancy (paraneoplastic)

30
Q

Charcot Marie Tooth - examination findings

A

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

31
Q

Charcot Marie Tooth - investigations and pathophysiology

A

Inherited sensorimotor neuropathy
Type 1 - commonest, demyelinating, AD mutation in peripheral myelin protein 2, decreased conduction velocity
Type 2 - axonal degeneration, AD, decreased amplitude

32
Q

Myasthenia gravis - examination

A 
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
33
Q

Myasthenia Gravis - Investigations and Management

A

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

34
Q

Lambert Eaton Myaesthenic Syndrome

A

Abs against Voltage Gated Calcium Channels
Often paraneoplastic SCLC
Lower limb girdle weakness
Weakness improves with repeated testing

35
Q

DDs B/L ptosis

A 
MG
Myotonic dystrophy
Congenital
Senile
B/L horner's (rare)
36
Q

GBS - pathophysiology and features

A

Acute AI demyelinating polyneuropathy 2* to molecular mimicry from C. jejuni, mycoplasma, CMV, EBV
Symmetrical ascending flaccid paralysis, paraesthesia

37
Q

GBS - investigations and management

A

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

38
Q

DDx VII palsy

A 
Bell's 75%
Vascular
MS
SOL
CPA? - acoustic neuroma
Ramsay Hunt
39
Q

DDx B/L VII palsy

A

B/L bells, sarcoidosis, BGS, Lyme, MG, myotonic dystrophy

40
Q

VII palsy - investigations and management

A

Glucose, HBA1c, VZV serology, anti ACh receptor,
Mx - prenisolone within 72h, acyclovir
Eye protection, artificial tears, tape to close eyes at night

41
Q

Bells palsy - prognosis

A

Complete - 80% full recovery, rest either delayed or some residual weakness

42
Q

VII nerve palsy - complications

A

Synkiesis - blinking causes up turing of mouth

Crocodile tears - eating stimulates unilateral lacrimation (eyes)

43
Q

III pals - medical or surgical?

A

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

44
Q

Causes of Marcus Gunn Pupil

A 
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
45
Q

Visual pathway

A 
Retina
Optic Nerve
Optic Chiasm
Optic tract
Lateral Geniculate Nucleus of thalamus
Optic radiation (superior is temporal, inferior is parietal)
Visual cortex
46
Q

Causes of conductive hearing loss

A

Preauricular
Obstruction - wax, foreign body
Tympanic membrane perforation
Ossicle defectos - otosclerosis, infection

47
Q

Causes of SNHL

A

Defects of cochlea
Congenital - alports (SNHL + haematuria)
Acquired - gentamicin, meningitis, measles, vestibular schwannoma

48
Q

Dysphasia

A

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

49
Q

Dysarthria

A

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)

50
Q

Myotonic dystrophy

A

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

51
Q

UMN vs LMN location

A 
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

Final Prep (19 decks)

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