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Neuro > Peds Movement Disorders > Flashcards

Peds Movement Disorders Flashcards

1
Q

Four types of ataxia

A
  • truncal
  • gait
  • ocular
  • appendicular
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2
Q

Describe chorea movement

A
  • nonpatterned
  • not repetitive
  • unpredictable
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3
Q

Athestosis is similar to chorea but occurs primarily in the ____

A

hands

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4
Q

1 cause chorea in childhood

A

ARF

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5
Q

3 Toxins responsible for chorea

A

stimulant meds
cocaine
lithium

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6
Q

Only primary cause of chorea in childhood

A

HD juvenile onset

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7
Q

When does sydenhams chorea occur?

A

Usually 4 months post infection so ASO is normal

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8
Q

MRI finding in sydenhams chorea

A

increased T2 signal in putamen and globus pallidus

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9
Q

Ab’s usually positive in Sydenhams

A
  • streptolysin O

- DNAase B

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10
Q

Treatments for Sydenhams

A
  • IVIG, steroids, penicillin

- BDZ, AEDs, Haldol, pimozide

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11
Q

MOA pimozide

A

dopamine antagonist

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12
Q

Dystonia definition:

A

-contraction of agonist and antagonist muscle groups

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13
Q

Two primary causes of dystonia:

A
  • hereditary

- metabolic

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14
Q

Four secondary causes of dystonia:

A
  • CP
  • trauma
  • stroke
  • toxin
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15
Q

When is dystonia absent?

A

during sleep

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16
Q

MC type dystonia in childhood

A

-dopa responsive

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17
Q

What is the first symptom of dopa responsive dystonia?

A

-foot dystonia

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18
Q

Cause of idiopathic generalized torsion dystonia ?

Progression?

A
  • AD DYT1 gene mutation

- lower limb –> full body

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19
Q

Glutaric academia type 1:

-error of metabolism in what amino acids?

A

catabolism of

  • lysine
  • hydroxylysine
  • tryptophan
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20
Q

Enzyme deficient in Glutaric acidemia type 1

A

glutaryl coenzyme A dehydrogenase

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21
Q

Symptoms of Glutaric Acidemia

A

macrocephaly at birth followed by chorea within first year

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22
Q

Treatment for glutaric academia

A

carnitine supplements

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23
Q

Wilsons chromosome

A

13q14q

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24
Q

Neuropsychiatric manifestations of WD:

A

Dysarthria
Dystonia
Tremor
Risus sardonicus

25
Q

Distinguish between spasticity and dystonia:

A
  • spasticity felt at rest, velocity dependent

- dystonia occurs when patient cannot be completely at rest

26
Q

Three classes of drugs used to treat dystonia

A
  • muscle relaxants
  • anticholinergics
  • BDZ
27
Q

Two muscle relaxants used for dystonia

A
  • baclofen

- tizanidine

28
Q

Anticholinergic + BDZ used for dystonia

A
  • trihexyphenidyl

- diazepam

29
Q

Common drug related causes of movement disorders

A
  • dopa antagonists= dystonia

- amphetamines, cocaine lithium= chorea/tremore

30
Q

Describe the prorgression of movement disorders assc with dopa antagonists

A

acute dystonic reaction –> tardive dyskinesia –> parkinsonism –> neuroleptic malignant syndrome

31
Q

Treatment for acute dystonia

A

IV Benadryl/ Cogentin

32
Q

What distinguishes transient and chronic tics?

A

-transient= less than 1 year

33
Q

Typical age of tic onset

A

6 –> worsen by 10 –> lessen by 18

34
Q

When should tics be absent?

A

during sleep

35
Q

How are stereotypies treated?

A

SSRIs

36
Q

Most common comorbid disorders in Tourettes?

A
  • # 1 OCD, ADHD

- #2 anxiety, mood disorder

37
Q

Treatment for tics if no comorbidities?

A

-a2 agonists (guanfacine)

38
Q

What does PANDAS stand for?

A 
Pediatric 
Autoimmune 
Neuropsych 
Disorders 
Assc with 
Strep
39
Q

What neuro conditions can be caused by GAS?

A
  • OCD

- tics

40
Q

How to treat PANDAS?

A
  • no abx for tic exacerbation
  • abx after ARF
  • standard tic meds
41
Q

Congenital causes of ataxia

A

-cerebellar malformation

42
Q

Four tumors assc with ataxia

A
  • neuroblastoma
  • ependymoma
  • astrocytoma
  • cerebellar hemangioblastoma in VHL
43
Q

4 causes of paroxysmal ataxia:

A
  • MSUD
  • Hartnups
  • Pyruvate De. Def
  • Channelopathies
44
Q

Hartnup Disease Defect

A
  • AR

- amino acid transport in kidney

45
Q

Symptoms of Hartnup Disease

A
  • pellagra

- limb ataxia

46
Q

Treatment for Hartnups

A

-High protein diet

47
Q

MSUD Cause

A

AR

Disorder of branched chain amino acid metabolism

48
Q

Treatment MSUD

A
  • protein restriction

- thiamine supplements

49
Q

Defect in Pyruvate Dehydrogenase Def

A
  • build up of pyruvate and lactate

- def of acetyl CoA and CO2

50
Q

Treatment for Pyruvate Dehydrogenase Def

A
  • acetazolamide

- ketogenic diet

51
Q

Episodic Ataxia 1 Gene?

2?

A

1- KCAN1

2-CACNA1A

52
Q

Distinguish between type 1 and 2 ataxia?

A
  • type 1 brief attacks

- type 2 attacks last days

53
Q

Eye movement ataxia is assc with what part of the brain?

A

-brainstem

54
Q

Limb ataxia vs truncal ataxia assc brain regions

A
  • limbs: hemispheres

- trunk: vermis

55
Q

Things to check in ataxia:

A
  • MRI
  • BAC (think moutwash)
  • CSF: infection, protein
56
Q

Infections assc with ataxia

A
  • Varicella
  • HSV
  • EBV
  • Mumps
  • Toxic levels of drugs
57
Q

Describe peripheral vertigo:

A
  • episodic
  • unidirectional
  • hearing loss
58
Q

Describe central vertigo:

A
  • constant
  • spinning variable directions
  • no hearing loss
  • CN deficits or cerebellar dz

Neuro (15 decks)

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