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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