Although brief arm extension may represent a portion of the normal startle response for infants exposed to unexpected stimuli, unprovoked movements of this type are suggestive of infantile spasms, an age-dependent epileptiform encephalopathy additionally characterized by a hypsarrhythmic EEG tracing. The concurrent present of infantile spasms, ash-leaf birthmarks, and calcified intracranial hamartomas confirm tuberous sclerosis as the underlying cause in this instance.
The patient's spasms subsided during 8 weeks of treatment with parenteral ACTH, but resumed following cessation of therapy. Spasms have continued despite aggressive treatment with multiple anticonvulsant combinations.
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Created: July 25, 1995