Differential Diagnosis Dystonic Reaction Tourette Syndrome Benign myoclonus of infancy Breath Holding Spells Infantile Spasms OpsoclonusMyoclonus Myoclonic Epilepsy of Infancy PANDAS Sydenham Chorea
Dystonic ReactionWhich meds?Treatment: Antipsychotics, antiemetics, and antidepressants Alcohol and cocaine use increase risk. Benadryl Benztropine Benzos
Tourette Syndrome • Average age of onset 6y/o • Resolve by age 18 in half of pts • Majority of pts have comorbid conditions? • Treatment of ADHD with methylphenidate? ADHD 60% May improve tics
Benign myoclonus of infancyAka benign nonepileptic infantile spasmsMay present with myoclonus or atonia, in clusters. Begins 3-8mos old. Increase in intensity over months, remits spontaneously at 2-3y/o.Normal neuro exam, EEG, and developmentWhat time of day? during wakefulness, frequently at mealtime.
Breath Holding SpellsFamHx in 30%Autonomic dysfunctionOnset prior to 18 mos, resolve by 4y/oExcellent prognosisPallid spells are associated with bradycardia, extremely rare cases require atropine or cardiac pacing.Associated with what finding on CBC? Iron Deficiency Anemia Supplementation may lead to resolution of symptoms in 30%
Infantile SpasmsAka West syndrome (infantile spasms, arrested development, hypsarhythmia on EEG)Peak onset 2-7mos old, but up to 4yrsOnly 10% go on to achieve normal developmentTreatment? ACTH
OpsoclonusMyoclonusParaneoplastic syndrome of neuroblastomaOnly 2% of NB present with O.M.50% with O.M. have N.B.Can also result from viral infxn, post-strep Pharyngitis, metastasis, metabolic d.o., intracranial hemorrhage
Myoclonic Epilepsy of InfancyMyoclonic jerks begin 1st year of life. May be one limb or multifocal. Demonstrates spike and wave pattern on EEG.Range from benign to severe
Pediatric autoimmune neuropsychiatric disorder associated with group A streptococciObsessive-compulsive disorder and/or tic disorder Pediatric onset Abrupt onset and episodic course of symptomsTemporal relation between GAS infection and onset (3mos, multiple cases over previous year)Neurologic abnormalities, such as motor hyperactivity, choreiform movements, or tics during exacerbations
Sydenham ChoreaWaxing/waning for months then ultimate resolution of chorea. Behavioral changes may persist.40-70% will have carditis. Relapse precipitated by? Pregnancy (chorea gravidarum), oral contraceptives, or Group A Strep Infection.