MND, MS, Parkinsons+ism Flashcards

1
Q

what genes can be involved in MND

A

SOD1 (chromosome 21) , C9orf72

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2
Q

onset MND

A

males around 60

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3
Q

symptoms MND

A

UMN: increased tone, hyperreflexia, spasticy weakness // LMN: fasciculations, wasting, hyporeflexia, weakness

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4
Q

what symptoms are spared in MND

A

eye muscles, cerebellar signs, abdo reflexes

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5
Q

initial symptoms MND

A

LMN signs + hand wasting

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6
Q

most common MND subtype

A

amyotrophic lateral sclerosis

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7
Q

symptoms amyotrophic lateral sclerosis

A

UMN + LMN: hand wasting, weak hands, stumbling, drooling, frontotemporal dementia

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8
Q

where are motor neurones lost in primary lateral sclerosis

A

motor neurones lost from Benz cells in motor cortex

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9
Q

symptoms primary lateral sclerosis

A

UMN signs only - mainly affects limbs, spasticity, no cognitive decline

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10
Q

which cells are lost in progressive spinal muscular atrophy

A

anterior horn cells

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11
Q

symptoms progressive spinal muscular atrophy

A

LMN signs only - affects distal muscles first, weakness, fasciculations

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12
Q

symptoms progressive bulbar palsy

A

UMN + LMN: drooling, can’t chew and swallow, can’t talk

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13
Q

invx MND

A

nerve studies normal // EMG // MRI

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14
Q

mx to slow symptoms MND

A

riluzole (anti-glutamate)

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15
Q

mx symptoms control MND

A

drooling: hyoscine // muscle cramps: baclofen, gabapentin // SOB: lorazepam

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16
Q

resp care MND

A

BIPAP at night

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17
Q

nutritional care MND

A

PEG

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18
Q

what is MS

A

autoimmine disorder –> demyelination of CNS (white tracts) disseminated in time and space

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19
Q

who normally gets MS

A

women, 20-30, higher latitudes (white people), FH: HLA DRB1

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20
Q

what cells are involved in MS

A

CD4 destruction of oligodendrocytes

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21
Q

presentation MS

A

TEAM: tingling (pins and needles, numbness, trigem/ // eyes (optic neuritis, Internuclear ophthalmoplegia) // ataxia (tremor, difficulty walking) MOTOR: weakness

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22
Q

general and autonomic symptoms MS

A

fatigue!! // urinary incontinence, sexual dysfunction, intellectual deterioration

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23
Q

symptoms optic neuritis

A

common presenting MS feature: painful eye movement + decreased colour saturation

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24
Q

symptoms Internuclear ophthalmoplegia

A

impaired CN III, IV, VI function // ipsilateral impaired adduction + contralateral nystagmus // horizontal double vision

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25
weird signs MS
Lhermittes: shock down spine + limbs when neck flexed // Uhthoffs phenomenon: worsening vision + symptoms when hot
26
what do active vs chronic plaques look like MS
active: pink, perivascular inflamm // chronic: firm and pearly grey,
27
categories of MS least --> most severe
relapse remitting // secondary progressive // primary pregrossive // progressive relapsing
28
what is relapse remitting MS
most common, acute attacks followed by periods of remission, increased disability after each attack
29
primary progressing MS
progressive deterioration no attacks
30
secondary progressive MS
starts as relapse remitting then becomes progressive disease
31
progressive - relapsing disease
worst - progressive decline superimopsed by acute attacks
32
invx MS
MRI: plaques // LP: oligoclonal IgG // delayed evoked potentials
33
antibodies MS
anti-MBP, NMO-IGG
34
mx acute relapse MS
oral --> IV steroirds for 5 days
35
mx disease modifying drugs MS
monoclonal antibodies: natalizumab, ocrelizumab // fingolumod // beta-inteferon // glatiramer acetate
36
mx fatigue MS
amantadine
37
ms spaciicty MS
baclofen or gabapentin, physio, botox
38
mx bladder dysfunction MS
USS: if residual volume catheter // if not oxybutunin
39
pathology of parkinsons diseae
degeneration of dopamine neurones is substantia nigra of basal ganglia
40
RF + genes parkinsons
men, 65, LRRK2 (dominant), parkin (recessive)
41
parkinsons triad
asymmetric bradykinesia, resting tremor, rigidity
42
Hz of parkinsons tremor
3-5Hz, improves with movement
43
bradykinesia and gait of parkinsons
slow, short suffling gait, difficulty initiating movement, reduced facial expression
44
most common pysch parkinson feature
depression
45
most common autonomic parkinson feature
postural hypotension + reduced smell
46
parkinsons (cerebellar) exam
DANISH: Dysdiadochokinesia, ataxia, nsytagmu, intention tremor, slurred speech, hypotonis
47
how does drug-induced parikinsonism present
motor symptoms bilateral // normal rigidity and no tremor
48
parkinsons invx
SPECT scan, specialist review
49
1st line mx parkinsons if motor symptoms affecting daily life
levodpoa + carbidopa
50
mx parkinsons if motor symptoms NOT affecting daily life
1st = dopamine agonist eg Ropinirole, pramipexole, apomorphine // levodopa // MAOB inhibitors eg Selegeline, rasagiline // 2nd = COMTi, amantadine
51
SE levodopa
off periods, hallucinations, dry mouth, palipatations, postural hypotension, anorexia
52
what pysch disorder does dopamine agonist therpy increase
imoulse control
53
what SE of levodopa is worse at peak dose
dyskinesia, dystonia, or chorea
54
when should levodopa be stopped
never acutely, if cannot take oral give dopamine rescue parch
55
mx parkinson tremor
Antimuscarinics/ anticholinergics eg procyclidine
56
what drugs can cause parkinsonism
antipsychotics, metoclopramide, carbon monoxide
57
most common parkinsons + syndromes
multiple systems atropy, progressive supranuclear palsy, cortico-basal degeneration, lewy body
58
symptoms progressive supranuclear palsy
vertical gaze palsy, postural instability, bradykinesia, cognitive impairment, (tremor OK)
59
symptoms multiple system atrophy
autonomic: ED, postural hypotension, bladder, ataxia + cerebellar signs
60
MRI multiple system atrophy
hot cross bun
61
cortico-basal degeneration symptoms
rigity and weakness one limb, phantom limb sensory loss, apraxia: loss of purposeful movement
62
symptoms lewy body dementia
cognitive decline + hallucinations before parkinsonism