Infantile Spasms
Associated with Tuberous Sclerosis
1) Flexor spasms: Sudden flexion of neck, trunk, arms, and legs and contraction of abdominal muscles
2) Extensor spasms: Abrupt extension of the neck and trunk, with abduction or adduction of the arms or legs
3) Mixed flexor-extensor spasms: Flexion of the neck, trunk, and arms, and extension of the legs
Clinical
• Presentation < 1 year of age
• Spasms
• Developmental delay
Diagnosis
• Hypsarrhythmia EEG pattern
Management
• Corticotropin (ACTH)
• Steroids
• Benzodiazepines
Prognosis
• Dependent on development prior to the onset of seizures
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