Benign Myoclonus of Early Infancy: An Imitator of West's Syndrome

Abstract

Benign myoclonus of early infancy is a rare condition characterized by nonepileptic spasms that may resemble the epileptic spasms seen in West's syndrome. The spells in benign myoclonus of early infancy begin before age 1 year and are self-limited. The electroencephalogram (EEG) is invariably normal, and neurologic development is not affected. West's syndrome is characterized by infantile spasms that appear before 1 year of age, an abnormal EEG with hypsarrhythmia, and a poor prognosis. We describe six infants who presented for evaluation of clusters of head, trunk or extremity spasms, eye blinking, brief jerking of upper extremities or trunk, and head nodding episodes. In most, a presumptive diagnosis of West's syndrome was made prior to the referral. One infant had been placed on valproate. Routine EEG recordings or prolonged video EEG monitoring were normal both during and between episodes. After the negative evaluations, the diagnosis of benign myoclonus of early infancy was made in each infant. Subsequently, no infant was treated with anticonvulsants. Follow-up revealed complete resolution of the episodes in all children within 2 weeks to 8 months of onset. All had normal neurologic development. Based on our cases and review of the literature, the prognosis for this disorder is excellent. Care should be taken to recognize this rare entity and avoid unnecessary and potentially harmful antiepileptic therapy. (J Child Neurol 2001;16:109-112).