Abstracts

Rationale: Standard treatment of Infantile Spasm (IS) is ACTH or Vigabatrin. Other reported therapeutic options for IS include Rufinamide, Topiramate, Levetiracetam, Prednisone, Pyridoxine and ketogenic diet. In reviewing the literature, intravenous immunoglobulins (IVIg) has only been used in one series of patients with IS. Our goal is to evaluate the outcome of IVIg in IS.Methods: Pilot Prospective study using IVIg in patients with IS when clinical history suggests the possible autoimmune mechanism.Results: We present 3 cases of IS where IVIg was given at various stages from IS onset. First patient, an AAF with a history of subdural hemorrhage after a MVA at 4 months (mo) had a full recovery until she began having IS at 8 mo. IVIg was started early after Levetiracetam. Second patient, a HF with normal early development who stopped responding to verbal stimuli after receiving her 4 mo immunizations, began having IS at 6 months of age. Her IS were refractory to multiple antiepileptic drugs including ACTH. IVIg was started at 17 mo. Third patient, a WF within three weeks after 6 month immunizations began having IS. Levetiracetam, Topiramate, and IVIg were initiated rapidly after onset. All patients received IVIg 1mg/kg/dose every 2-4 weeks. Improvement of spasm frequency and severity was noted within a week of first IVIg treatment in all patients as well as their alertness and interaction with surroundings. The third patient’s EEG ultimately normalized. She eventually became seizure free and was noted at four years of age to have a normal psychomotor development.Conclusions: In our experience, IVIg is beneficial in most infants when an underlying autoimmune mechanism is suspected. The best outcome is seen if treatment is initiated early after onset of IS.