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Flashcards in Peds Movement Disorders Deck (58):
1

Four types of ataxia

-truncal
-gait
-ocular
-appendicular

2

Describe chorea movement

-nonpatterned
-not repetitive
-unpredictable

3

Athestosis is similar to chorea but occurs primarily in the ____

hands

4

#1 cause chorea in childhood

ARF

5

3 Toxins responsible for chorea

stimulant meds
cocaine
lithium

6

Only primary cause of chorea in childhood

HD juvenile onset

7

When does sydenhams chorea occur?

Usually 4 months post infection so ASO is normal

8

MRI finding in sydenhams chorea

increased T2 signal in putamen and globus pallidus

9

Ab's usually positive in Sydenhams

-streptolysin O
-DNAase B

10

Treatments for Sydenhams

-IVIG, steroids, penicillin
-BDZ, AEDs, Haldol, pimozide

11

MOA pimozide

dopamine antagonist

12

Dystonia definition:

-contraction of agonist and antagonist muscle groups

13

Two primary causes of dystonia:

-hereditary
-metabolic

14

Four secondary causes of dystonia:

-CP
-trauma
-stroke
-toxin

15

When is dystonia absent?

during sleep

16

MC type dystonia in childhood

-dopa responsive

17

What is the first symptom of dopa responsive dystonia?

-foot dystonia

18

Cause of idiopathic generalized torsion dystonia ?
Progression?

-AD DYT1 gene mutation
-lower limb --> full body

19

Glutaric academia type 1:
-error of metabolism in what amino acids?

catabolism of
-lysine
-hydroxylysine
-tryptophan

20

Enzyme deficient in Glutaric acidemia type 1

glutaryl coenzyme A dehydrogenase

21

Symptoms of Glutaric Acidemia

macrocephaly at birth followed by chorea within first year

22

Treatment for glutaric academia

carnitine supplements

23

Wilsons chromosome

13q14q

24

Neuropsychiatric manifestations of WD:

Dysarthria
Dystonia
Tremor
Risus sardonicus

25

Distinguish between spasticity and dystonia:

-spasticity felt at rest, velocity dependent
-dystonia occurs when patient cannot be completely at rest

26

Three classes of drugs used to treat dystonia

-muscle relaxants
-anticholinergics
-BDZ

27

Two muscle relaxants used for dystonia

-baclofen
-tizanidine

28

Anticholinergic + BDZ used for dystonia

-trihexyphenidyl
-diazepam

29

Common drug related causes of movement disorders

-dopa antagonists= dystonia
-amphetamines, cocaine lithium= chorea/tremore

30

Describe the prorgression of movement disorders assc with dopa antagonists

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

31

Treatment for acute dystonia

IV Benadryl/ Cogentin

32

What distinguishes transient and chronic tics?

-transient= less than 1 year

33

Typical age of tic onset

6 --> worsen by 10 --> lessen by 18

34

When should tics be absent?

during sleep

35

How are stereotypies treated?

SSRIs

36

Most common comorbid disorders in Tourettes?

-#1 OCD, ADHD
-#2 anxiety, mood disorder

37

Treatment for tics if no comorbidities?

-a2 agonists (guanfacine)

38

What does PANDAS stand for?

Pediatric
Autoimmune
Neuropsych
Disorders
Assc with
Strep

39

What neuro conditions can be caused by GAS?

-OCD
-tics

40

How to treat PANDAS?

-no abx for tic exacerbation
-abx after ARF
-standard tic meds

41

Congenital causes of ataxia

-cerebellar malformation

42

Four tumors assc with ataxia

-neuroblastoma
-ependymoma
-astrocytoma
-cerebellar hemangioblastoma in VHL

43

4 causes of paroxysmal ataxia:

-MSUD
-Hartnups
-Pyruvate De. Def
-Channelopathies

44

Hartnup Disease Defect

-AR
-amino acid transport in kidney

45

Symptoms of Hartnup Disease

-pellagra
-limb ataxia

46

Treatment for Hartnups

-High protein diet

47

MSUD Cause

AR
Disorder of branched chain amino acid metabolism

48

Treatment MSUD

-protein restriction
-thiamine supplements

49

Defect in Pyruvate Dehydrogenase Def

-build up of pyruvate and lactate
-def of acetyl CoA and CO2

50

Treatment for Pyruvate Dehydrogenase Def

-acetazolamide
-ketogenic diet

51

Episodic Ataxia 1 Gene?
2?

1- KCAN1
2-CACNA1A

52

Distinguish between type 1 and 2 ataxia?

-type 1 brief attacks
-type 2 attacks last days

53

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

-brainstem

54

Limb ataxia vs truncal ataxia assc brain regions

-limbs: hemispheres
-trunk: vermis

55

Things to check in ataxia:

-MRI
-BAC (think moutwash)
-CSF: infection, protein

56

Infections assc with ataxia

-Varicella
-HSV
-EBV
-Mumps
-Toxic levels of drugs

57

Describe peripheral vertigo:

-episodic
-unidirectional
-hearing loss

58

Describe central vertigo:

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