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
INO = disruption of:
medial longitudonal fasciculus
26
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)
27
Poor prognostic signs in MS
``` Older Male Motor signs at onset Frequent relapses Many lesions ```
28
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)
29
Ddx proximal myopathy
Inherited (muscular dystrophy) Inflammation (polymyositis, dermatomyositis) Endocrine (cushing's, acromegaly, thyrotoxicosis, osteomalacia, diabetic amyotrophy) Drugs (alcohol, statins, steroids) Malgnancy (paraneoplastic)
30
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
31
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
32
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 ```
33
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
34
Lambert Eaton Myaesthenic Syndrome
Abs against Voltage Gated Calcium Channels Often paraneoplastic SCLC Lower limb girdle weakness Weakness improves with repeated testing
35
DDs B/L ptosis
``` MG Myotonic dystrophy Congenital Senile B/L horner's (rare) ```
36
GBS - pathophysiology and features
Acute AI demyelinating polyneuropathy 2* to molecular mimicry from C. jejuni, mycoplasma, CMV, EBV Symmetrical ascending flaccid paralysis, paraesthesia
37
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
38
DDx VII palsy
``` Bell's 75% Vascular MS SOL CPA? - acoustic neuroma Ramsay Hunt ```
39
DDx B/L VII palsy
B/L bells, sarcoidosis, BGS, Lyme, MG, myotonic dystrophy
40
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
41
Bells palsy - prognosis
Complete - 80% full recovery, rest either delayed or some residual weakness
42
VII nerve palsy - complications
Synkiesis - blinking causes up turing of mouth | Crocodile tears - eating stimulates unilateral lacrimation (eyes)
43
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
44
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 ```
45
Visual pathway
``` Retina Optic Nerve Optic Chiasm Optic tract Lateral Geniculate Nucleus of thalamus Optic radiation (superior is temporal, inferior is parietal) Visual cortex ```
46
Causes of conductive hearing loss
Preauricular Obstruction - wax, foreign body Tympanic membrane perforation Ossicle defectos - otosclerosis, infection
47
Causes of SNHL
Defects of cochlea Congenital - alports (SNHL + haematuria) Acquired - gentamicin, meningitis, measles, vestibular schwannoma
48
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
49
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)
50
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
51
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 ```