Objective: To retrospectively examine the incidence and etiologies of asterixis in children evaluated at a tertiary care pediatric hospital.

Background: Asterixis (negative myoclonus) is associated with generalized encephalopathies or structural brain lesions in adults but is thought to be less common in children. However, relative incidence of asterixis and its varied etiologies may be different in the pediatric population.

Methods: We conducted a retrospective chart review of all inpatient and outpatient notes at a large tertiary care pediatric hospital (Boston Children’s Hospital, Boston, MA, USA) over a seven month period (9/30/2014-4/30/2015) containing the words "asterixis" or "negative myoclonus." Documentation of these findings as present or absent in the physical exam portion of the note was used to determine whether patients were formally evaluated for asterixis.

Results: One hundred fifty patients were evaluated for asterixis at over a seven month period. Of these, 30 patients were age 18 or older and thus excluded. The remaining 120 patients had a mean age of 11.7 (SD 4.3) years. Only 2/120 patients demonstrated asterixis. Patient 1: A 6 year old girl with focal-onset epilepsy without an underlying brain lesion presented to Neurology for a second opinion regarding her seizures. Some seizures manifested as negative myoclonus, predominantly of her left arm. Patient 2: A 12 year old boy presented to Oncology for treatment of relapsed Hodgkin’s lymphoma. Neurology was consulted for generalized weakness. He was found to have ifosfamide-associated generalized negative myoclonus. Neither patient was encephalopathic. Of the encounters with chief complaints of twitching/tremors/tics/spasms/fasciculations (n=8), altered mental status/encephalopathy/sleepiness (n=9), or valproate-associated hyperammonemia (n=5), none documented asterixis.

Conclusions: Asterixis is a rare finding in the pediatric population, can be present without encephalopathy or an underlying brain lesion, and should prompt comprehensive investigation for etiologies including epilepsy and medication side effects.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/incidence-and-etiologies-of-pediatric-asterixis/