Infantile spasms

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Infantile spasms is a form of severe infantile epilepsy that is difficult to treat and has a high morbidity. Without treatment essentially an encephalopathy develops that affects brain development in many. Various syndromes exist such as West syndrome, or tuberous sclerosis and multiple gene mutations such as in DNM1 can cause the presentation. Treatment in those with also a hypsarrhythmic (or similar) EEG is hormonal manipulation which has about a 60% success rate. Vigabatrin adds an extra 10% remission success rate.

Treatment

  • Hormonal manipulation
    • Risk of infection and steroid side effects
    • Prednisolone usually 10 mg four times a day
    • Tetracosactide depot IM 0ยท5 mg (40 IU) on alternate days
  • Vigabatrin 100 mg/kg per day
    • Faster onset of remission
    • Risk of visual field defects
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