Peds Movement Disorders Flashcards
Four types of ataxia
- truncal
- gait
- ocular
- appendicular
Describe chorea movement
- nonpatterned
- not repetitive
- unpredictable
Athestosis is similar to chorea but occurs primarily in the ____
hands
1 cause chorea in childhood
ARF
3 Toxins responsible for chorea
stimulant meds
cocaine
lithium
Only primary cause of chorea in childhood
HD juvenile onset
When does sydenhams chorea occur?
Usually 4 months post infection so ASO is normal
MRI finding in sydenhams chorea
increased T2 signal in putamen and globus pallidus
Ab’s usually positive in Sydenhams
- streptolysin O
- DNAase B
Treatments for Sydenhams
- IVIG, steroids, penicillin
- BDZ, AEDs, Haldol, pimozide
MOA pimozide
dopamine antagonist
Dystonia definition:
-contraction of agonist and antagonist muscle groups
Two primary causes of dystonia:
- hereditary
- metabolic
Four secondary causes of dystonia:
- CP
- trauma
- stroke
- toxin
When is dystonia absent?
during sleep
MC type dystonia in childhood
-dopa responsive
What is the first symptom of dopa responsive dystonia?
-foot dystonia
Cause of idiopathic generalized torsion dystonia ?
Progression?
- AD DYT1 gene mutation
- lower limb –> full body
Glutaric academia type 1:
-error of metabolism in what amino acids?
catabolism of
- lysine
- hydroxylysine
- tryptophan
Enzyme deficient in Glutaric acidemia type 1
glutaryl coenzyme A dehydrogenase
Symptoms of Glutaric Acidemia
macrocephaly at birth followed by chorea within first year
Treatment for glutaric academia
carnitine supplements
Wilsons chromosome
13q14q
Neuropsychiatric manifestations of WD:
Dysarthria
Dystonia
Tremor
Risus sardonicus
Distinguish between spasticity and dystonia:
- spasticity felt at rest, velocity dependent
- dystonia occurs when patient cannot be completely at rest
Three classes of drugs used to treat dystonia
- muscle relaxants
- anticholinergics
- BDZ
Two muscle relaxants used for dystonia
- baclofen
- tizanidine
Anticholinergic + BDZ used for dystonia
- trihexyphenidyl
- diazepam
Common drug related causes of movement disorders
- dopa antagonists= dystonia
- amphetamines, cocaine lithium= chorea/tremore
Describe the prorgression of movement disorders assc with dopa antagonists
acute dystonic reaction –> tardive dyskinesia –> parkinsonism –> neuroleptic malignant syndrome
Treatment for acute dystonia
IV Benadryl/ Cogentin
What distinguishes transient and chronic tics?
-transient= less than 1 year
Typical age of tic onset
6 –> worsen by 10 –> lessen by 18
When should tics be absent?
during sleep
How are stereotypies treated?
SSRIs
Most common comorbid disorders in Tourettes?
- # 1 OCD, ADHD
- #2 anxiety, mood disorder
Treatment for tics if no comorbidities?
-a2 agonists (guanfacine)
What does PANDAS stand for?
Pediatric Autoimmune Neuropsych Disorders Assc with Strep
What neuro conditions can be caused by GAS?
- OCD
- tics
How to treat PANDAS?
- no abx for tic exacerbation
- abx after ARF
- standard tic meds
Congenital causes of ataxia
-cerebellar malformation
Four tumors assc with ataxia
- neuroblastoma
- ependymoma
- astrocytoma
- cerebellar hemangioblastoma in VHL
4 causes of paroxysmal ataxia:
- MSUD
- Hartnups
- Pyruvate De. Def
- Channelopathies
Hartnup Disease Defect
- AR
- amino acid transport in kidney
Symptoms of Hartnup Disease
- pellagra
- limb ataxia
Treatment for Hartnups
-High protein diet
MSUD Cause
AR
Disorder of branched chain amino acid metabolism
Treatment MSUD
- protein restriction
- thiamine supplements
Defect in Pyruvate Dehydrogenase Def
- build up of pyruvate and lactate
- def of acetyl CoA and CO2
Treatment for Pyruvate Dehydrogenase Def
- acetazolamide
- ketogenic diet
Episodic Ataxia 1 Gene?
2?
1- KCAN1
2-CACNA1A
Distinguish between type 1 and 2 ataxia?
- type 1 brief attacks
- type 2 attacks last days
Eye movement ataxia is assc with what part of the brain?
-brainstem
Limb ataxia vs truncal ataxia assc brain regions
- limbs: hemispheres
- trunk: vermis
Things to check in ataxia:
- MRI
- BAC (think moutwash)
- CSF: infection, protein
Infections assc with ataxia
- Varicella
- HSV
- EBV
- Mumps
- Toxic levels of drugs
Describe peripheral vertigo:
- episodic
- unidirectional
- hearing loss
Describe central vertigo:
- constant
- spinning variable directions
- no hearing loss
- CN deficits or cerebellar dz
