Peds Movement Disorders

Question 1

Four types of ataxia

Answer 1

• truncal
• gait
• ocular
• appendicular

Question 2

Describe chorea movement

Answer 2

• nonpatterned
• not repetitive
• unpredictable

Question 3

Athestosis is similar to chorea but occurs primarily in the ____

Answer 3

hands

Question 4

1 cause chorea in childhood

Answer 4

ARF

Question 5

3 Toxins responsible for chorea

Answer 5

stimulant meds
cocaine
lithium

Question 6

Only primary cause of chorea in childhood

Answer 6

HD juvenile onset

Question 7

When does sydenhams chorea occur?

Answer 7

Usually 4 months post infection so ASO is normal

Question 8

MRI finding in sydenhams chorea

Answer 8

increased T2 signal in putamen and globus pallidus

Question 9

Ab’s usually positive in Sydenhams

Answer 9

• streptolysin O

- DNAase B

Question 10

Treatments for Sydenhams

Answer 10

• IVIG, steroids, penicillin

- BDZ, AEDs, Haldol, pimozide

Question 11

MOA pimozide

Answer 11

dopamine antagonist

Question 12

Dystonia definition:

Answer 12

-contraction of agonist and antagonist muscle groups

Question 13

Two primary causes of dystonia:

Answer 13

• hereditary

- metabolic

Question 14

Four secondary causes of dystonia:

Answer 14

• CP
• trauma
• stroke
• toxin

Question 15

When is dystonia absent?

Answer 15

during sleep

Question 16

MC type dystonia in childhood

Answer 16

-dopa responsive

Question 17

What is the first symptom of dopa responsive dystonia?

Answer 17

-foot dystonia

Question 18

Cause of idiopathic generalized torsion dystonia ?

Progression?

Answer 18

• AD DYT1 gene mutation

- lower limb –> full body

Question 19

Glutaric academia type 1:

-error of metabolism in what amino acids?

Answer 19

catabolism of

• lysine
• hydroxylysine
• tryptophan

Question 20

Enzyme deficient in Glutaric acidemia type 1

Answer 20

glutaryl coenzyme A dehydrogenase

Question 21

Symptoms of Glutaric Acidemia

Answer 21

macrocephaly at birth followed by chorea within first year

Question 22

Treatment for glutaric academia

Answer 22

carnitine supplements

Question 23

Wilsons chromosome

Answer 23

13q14q

Question 24

Neuropsychiatric manifestations of WD:

Answer 24

Dysarthria
Dystonia
Tremor
Risus sardonicus

Question 25

Distinguish between spasticity and dystonia:

Answer 25

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

Question 26

Three classes of drugs used to treat dystonia

Answer 26

- muscle relaxants - anticholinergics - BDZ

Question 27

Two muscle relaxants used for dystonia

Answer 27

- baclofen | - tizanidine

Question 28

Anticholinergic + BDZ used for dystonia

Answer 28

- trihexyphenidyl | - diazepam

Question 29

Common drug related causes of movement disorders

Answer 29

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

Question 30

Describe the prorgression of movement disorders assc with dopa antagonists

Answer 30

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

Question 31

Treatment for acute dystonia

Answer 31

IV Benadryl/ Cogentin

Question 32

What distinguishes transient and chronic tics?

Answer 32

-transient= less than 1 year

Question 33

Typical age of tic onset

Answer 33

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

Question 34

When should tics be absent?

Answer 34

during sleep

Question 35

How are stereotypies treated?

Answer 35

SSRIs

Question 36

Most common comorbid disorders in Tourettes?

Answer 36

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

Question 37

Treatment for tics if no comorbidities?

Answer 37

-a2 agonists (guanfacine)

Question 38

What does PANDAS stand for?

Answer 38

``` Pediatric Autoimmune Neuropsych Disorders Assc with Strep ```

Question 39

What neuro conditions can be caused by GAS?

Answer 39

- OCD | - tics

Question 40

How to treat PANDAS?

Answer 40

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

Question 41

Congenital causes of ataxia

Answer 41

-cerebellar malformation

Question 42

Four tumors assc with ataxia

Answer 42

- neuroblastoma - ependymoma - astrocytoma - cerebellar hemangioblastoma in VHL

Question 43

4 causes of paroxysmal ataxia:

Answer 43

- MSUD - Hartnups - Pyruvate De. Def - Channelopathies

Question 44

Hartnup Disease Defect

Answer 44

- AR | - amino acid transport in kidney

Question 45

Symptoms of Hartnup Disease

Answer 45

- pellagra | - limb ataxia

Question 46

Treatment for Hartnups

Answer 46

-High protein diet

Question 47

MSUD Cause

Answer 47

AR | Disorder of branched chain amino acid metabolism

Question 48

Treatment MSUD

Answer 48

- protein restriction | - thiamine supplements

Question 49

Defect in Pyruvate Dehydrogenase Def

Answer 49

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

Question 50

Treatment for Pyruvate Dehydrogenase Def

Answer 50

- acetazolamide | - ketogenic diet

Question 51

Episodic Ataxia 1 Gene? | 2?

Answer 51

1- KCAN1 | 2-CACNA1A

Question 52

Distinguish between type 1 and 2 ataxia?

Answer 52

- type 1 brief attacks | - type 2 attacks last days

Question 53

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

Answer 53

-brainstem

Question 54

Limb ataxia vs truncal ataxia assc brain regions

Answer 54

- limbs: hemispheres | - trunk: vermis

Question 55

Things to check in ataxia:

Answer 55

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

Question 56

Infections assc with ataxia

Answer 56

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

Question 57

Describe peripheral vertigo:

Answer 57

- episodic - unidirectional - hearing loss

Question 58

Describe central vertigo:

Answer 58

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