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Neuro online course > Movement disorders + laughing > Flashcards

Movement disorders + laughing Flashcards

0
Q

What is the major neurotransmitter within the striatum and pallidum

A

GABA / inhibitory

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1
Q

What is the main output structure of the basal ganglia projecting to the thalamus?

A

GPi

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2
Q

What is GPi main role

A

It receives info from striatum via direct/indirect pathway AND its job is to INHIBIT the THALMAUS

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3
Q

What is dopamine effect on movement?

A

Dopamine facilitates movement

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4
Q

possible enzyme deficits in dopa-responsive dystonia

A
  1. tyrosine hydroxylase

2. GTP cyclohydrolase 1

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5
Q

What are the ways the striatum influences the Globus pallidus (2 paths)

A
  1. Directly to the GPi (excitatory D1) which inhibits thalamus
  2. via GPe (inhibitory D2) then STN then GPi
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6
Q

Diagnostic screening tests in Wilson’s dz?

A

screening: high 24 hr urine copper and low serum ceruloplasmin

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7
Q

child or young adult with twisted foot, gait abnormality, juvenile parkinsonism

A

may be dopa-responsive dystonia which is treatable with low dose l-dopa!

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8
Q

Red flags (3) you aren’t dealing with pure parkinsonism

A
  1. ataxia
  2. weakness
  3. spasticity
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9
Q

toxicity in Welders that may lead to parkinsonism

A

manganese

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10
Q

Top 3 dx to consider instead of Parkinson disease in the young

A
  1. Wilson’s
  2. Dopa responsive dystonia
  3. huntington’s can have no chorea and more PD sx in young
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11
Q

Autosomal dominant mutation w/ successive generations of PD with normal onset age 50-70

A

LRRK2 mutation

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12
Q

more rare cause of dominant inheritance of PD with younger onset?

A

SNCA alpha synuclein (PARK-1)

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13
Q

most common cause of young onset PD that is not present in successive families’

  • tends to have dystonia, and diurnal variations with improvement with sleep
  • response to tx?
A

Parkin gene, good l-dopa response, absence of Lewy bodies
(Autosomal recessive)

(PARK 2: wen teen goes Parkin looking for sex: Chr 6)

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14
Q

what are Lewy bodies made up of?

A

alpha synuclein
ubiquitin
neurofilament protein

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15
Q

dose of levodopa considered a fair trial?

A

at least 900-1000mg/day (300 TID)

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16
Q

3 nonmotor sx in PD that may precede motor sx

A
  1. REM behavior disorder
  2. anosmia
  3. autonomic dysfunction
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17
Q

early severe presentation of autonomic dysfunction suggests what?

A

MSA: Multiple system atrophy

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18
Q

REM behavior disorder in isolation is highly predictive of what?

A

an alpha synucleinopathy

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19
Q

Deep brain stimulation improves what (4) and has no effect on what (1)

A

DBS good for:

  1. improving dyskinesias to best dyskinesia-free ON state on meds alone
  2. reduced med requirements
  3. improved ON time
  4. reduced OFF time

Does not effect postural reflexes

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20
Q

Contraindications for Deep brain stim for PD?

A

severe psych disease, cognitive impairment, postural instability (may worsen it)

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21
Q

Most effective and best tolerated med for PD and how does it work

A

levodopa with carbidopa which prevents it from getting broken down before it gets to the brain

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22
Q

What are ropinirole, pramipexole, and apomorphine?

What is the role? Benefits / disadvantages

A

dopamine agonists
they are less effective and have more s/e than ldopa for pd BUT they have less dyskinesias particularly in young patients who are at more risk for dyskinesias with levodopa

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23
Q

5 main med categories for PD

A
  1. Levodopa
  2. DA agonist
  3. Anticholinergics
  4. MAO-B inhibitors
  5. Amantadine
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24
Q

med that is rarely used but can prolong duration of effect of levodopa

A

entacapone (COMT inhibitor)

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25
Q

major side effects of levodopa

A

dyskinesias, vivid dreams/illusions/hallucinations (esp in older cognitively impaired pts), dizziness, hypotension, somnolence

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26
Q

major s/e of Dopamine agonists

A

worse hallucinations/nausea
sleep attacks
compulsive behaviors

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27
Q

when do dyskinesias usually occur with PD med treatment / levodopa

A

at peak of ON

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28
Q

order of options in patient on multimedications for PD and who are having cognitive / psych side effects (3)

A
  1. get off anticholinergics –> amantadine –> MAOB–>DA agonist –> levodopa (keep the ones with best efficacy and least s/e)
  2. add Rivastigmine which is a cholinesterase inhibitor approved for PD dementia
  3. lastly add antipsychotic but be very careful (black box warning)
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29
Q

Best two antipsychotics in PD least likely to worsen PD

A

Quetiapine and clozapine

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30
Q

Tao or alpha synuclein?

Multiple system atrophy

A

alpha synuclein

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31
Q

Tao or alpha synuclein?

Progressive Supranuclear Palsy

A

Tao

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32
Q

Corticobasal degeneration

Tao or alpha synuclein?

A

Tao

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33
Q

Tau or alpha synuclein?

PD

A

Parkinson disease - alpha synuclein

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34
Q

Tau or alpha synuclein?

Lewy Body dementia?

A

Alpha synuclein

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35
Q

Parkinsonism with very early falls?

A

supranuclear vertical palsy

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36
Q

diseases that are less likely to respond to ldopa

A

PSP and CBD

37
Q

PD + disease with alien hand or unilateral limb dystonia or apraxia

A

corticobasal degeneration

38
Q

Number of repeats with Huntingtons premutation that can pass down for anticipation/expansion (unstable)
Normal level
Level needed for disease

A

normal 40 with full prenetrance

39
Q

impaired saccade initiation and speed with need for head movements

A

huntington disease

40
Q

Possible treatment of huntingtons disease chorea

A

tetrabenazine dopamine receptor blocker but depletes all monoamines so can deplete 5HT and worsen depression

41
Q

Meds that can cause myoclonus (5)

A
  1. SSRI
  2. TCA
  3. lithium
  4. opioids
  5. stimulants
42
Q

palatal myoclonus suggestive of what?

A

brainstem lesion

43
Q

meds that may be helpful for myoclonus? (3)

A

VPA, keppra, clonazepam

44
Q

meds (4) that are known for causing action tremor

A
  1. amiodarone
  2. amitriptyline
  3. cyclosporine
  4. VPA
45
Q

MEds that may be helpful for action tremor (2)

A

nonselective BB like propanolol

primidone

46
Q

best treatment for focal / segmental dystonia like cervical dystonia?

A

botox

47
Q

best way to manage drug-induced parkinsonism

A

d/c offending agent or reduce it (may take 3 mos)
may consider adding anticholinergic or amantadine
ldopa, receptor blockers are not usually helpful

48
Q

major risk factor for Neuroleptic malignant syndrome

A

dementia with Lewy bodies (dont tolerate antipsych meds)

49
Q

3 basal ganglia toxins and their specific spot of damage

A
  1. carbon monoxide: GP
  2. methanol: putamen
  3. MPTP: parkinsonism
50
Q

Ddx abetalipoproteinemia from Freidreich’s ataxia

A

both can cause progressive ataxia BUT Friedreichs has cardiomyopathy and abetalipoproteinemia has lots of other stuff like cholesterol/TG issues, acanthocytosis, diarrhea, AND finally, retinitis pigmentosa

51
Q

Gene causing alternating hemiplegia of infants/toddlers and what is treatment
Bonus: same gene causes something else too

A

ATP1A2 chr 1q23
Tx w/ flunarizine
also causes familial hemiplegic migraine 2

52
Q

loss of reflexes and joint position sense in feet but upgoing toes, thing of what?

A

Friedreich ataxia

53
Q

Disease that looks like huntington’s disease and is also due to a CAG expansion, that causes chorea dementia, and ataxia, and in younger kids really has mostly progressive myoclonic epilepsy. What chromosome?

A

Dentatorubral-Pallidoluysian Atrophy (DRPLA) on chr 12 (not 4 like Huntingtons)

54
Q

how to diagnose dopa responsive dystonia?

A

CSF for neurotransmitters
-disease is due to BH4 synthesis problem (GTP cyclohydrolase 1 def) or Tyrosine hydroxylase deficiency that prevents tyrosine from being converted to levodopa

55
Q

episodic ataxia 1 is associated with what channel defect / gene, what clinical findings, and what duration of attack

A

K channel on chrom 12
MyoKymia
lasts Minutes

56
Q

episodic ataxia 2 is associated with what channel defect / gene, what clinical findings, and what duration of attack

A 
Calcium gene chrom 19, 
Nystagmus, responds to Acetazolamide
Attacks last Hours (19 instead of a short 12 where EA-1 is)
AND: Ca (2+: EA-2 vs K (1+: EA-1)
AND EA-2: two eyes for nystagmus
57
Q

disease with bilateral calcifications of basal ganglia and subcortical dementia, psychiatric sx, and parkinsonism

A

Fahrs disease

58
Q

who gets fragile X associated tremor/ataxia syndrome?

Affects what brain areas

A

men who have premutations and may have grandsons w/ dz

Affects middle cerebellar peduncles

59
Q

mutation in Friedreich’s ataxia

A

GAA expansion on chr 9 (FRDA gene)

60
Q

When does Friedreich ataxia present compared to ataxia-telangiectasia?

A

older in FReidreichs: 13

early childhood: ataxia-telangiectasia

61
Q

what chromosome and gene in ataxia telangiectasia

A

ATM chr 11

62
Q

Disorder with extrapyramidal sx in infancy, hypotonia, and MRI w frontotemporal atrophy with bat-wing appearance

A

Glutaric acidemia type 1

63
Q

where is the lesion in hemiballismus?

A

contralat subthalamic nucleus

64
Q

Huntington Disease gene mutation chromosome

A

CAG repeat Chr 4

Catch Animal Game, Hunt FOUR Animals

65
Q

akinetic, dystonic form of huntingtons?

A

westphal variant

66
Q

how does juvenile huntington present

A

more like parkinson and dystonia and myoclonus/sz

67
Q

path finding in huntingtons

A

loss of Gaba neurons in striatum

68
Q

presentation and cause of hyperekplexia?

A

exaggerated startle in infancy / apnea

caused by glycine receptor mutation chr 5

69
Q

cerebellar ataxia, cerebellar atrophy, cataracts, ID, hypotonia, weakness, primary gonadal failure, scoliosis

A

Marinesco-Sjogren syndrome

(Maraschino cherries ridiculous mnemonic)??

70
Q

neuroacanthocytosis (RBC spiky) that is x-linked and is associated with facial tics and axonal peripheral neuropathy

A

McLeod syndrome

71
Q

Oromandibular dystonia with blepharospasm

A

Meige syndrome

72
Q

hot cross bun sign is what and seen in what

A

seen in MSA multiple system atrophy from degeneration of pontocerebellar fibers causes brainstem above 4th vent to look like it has cross septations

73
Q

antibody seen in adults with opsoclonus-myoclonus syndrome

A

Ab to Ri (ANNA-2)

74
Q

essential vs symptomatic palatal tremor

A

essential: tensor veli palatini: eases in sleep
Symptomatic: from levator veli palatini and is moved by soft palate and continues in sleep

75
Q

lesion of triangle of Guillain-Mollaret may cause what

A

palatal tremor

76
Q

eye of the tiger MRI sign

A

PKAN / iron accum in brain

77
Q

what correlates witih gait abnormalities in Parkinsons

A

loss of neurons in pedunculopontine nucleus (PPN)

78
Q

possible treatment for paroxysmal dyskinesias

A

carbamazepine or phenytoin

79
Q

PD like illness with jerky eyes and backwards falling

A

PSP

postural stability is poor

80
Q

What is the ramsay hunt syndrome thats a movement disorder

A

progressive myoclonic ataxia…

81
Q

accumulation of phytanic acid occurs in what disorder and what are the sx?

A

cerebellar ataxia, retinitis pigmentosa, polyneuropathy

82
Q

antibodies in stiff person syndrome

what pts are at risk

A

anti-GAD Abs

may be paraneoplastic SCLC or breast

83
Q

wing beating tremor, sunflower cataracts, bulbar dystonia, dysarthria, drooling

A

Wilsons

84
Q

giant panda face on MRI

A

Wilsons (midbrain)

85
Q

Wilson’s gene?

A

ATP7B on chr 13q

86
Q

serotonin syndrome vs NMS?

A

myoclonus in serotonin syndrome

rigidity in NMS

87
Q

Most common autosomal dominant inherited ataxia in US?

A

Machado-Joseph disease SCA-3 (spinocerebellar atrophy 3)

88
Q

type of ataxia: slow clumsy, wide based, constant shifting of position to maintain balance. Titubation

A

cerebellar ataxia

89
Q

type of ataxia: steppage gait, irregular striking of floor, wide based

A

sensory ataxia (loss of jp sense)

90
Q

sewaga variant dystonia?

A

dopa responsive

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