Startle epilepsy with infantile hemiplegia: report of two cases improved by surgery.

PURPOSE: To study the effectiveness of surgical therapy on 2 patients with startle epilepsy with infantile brain damage (SEIBD), a rare but distinctive epileptic syndrome characterized by motor seizures resistant to antiepileptic drugs (AEDs).

METHODS

The patients with SEIBD both had hemiplegia, due to gross contralateral hemispheric lesions, and suffered from tonic postural seizures, frequently provoked by sudden unexpected somatosensory stimuli on the paretic side of the body. These attacks occasionally caused the patients to drop to the floor, and mild-to-moderate injuries had been sustained; they were resistant to all currently available AEDs. Consequently, these daily drop attacks severely restricted the patients' social lives and school participation. Both patients underwent corpus callosotomy and resection of epileptogenic premotor and supplementary motor lesions. One patient also underwent additional multiple subpial transections of the primary sensorimotor area.

RESULTS

Seizures, as well as quality of life, improved dramatically in both patients after surgery.

CONCLUSIONS

Startle epilepsy with infantile hemiplegia is a distinct epileptic syndrome characterized by structural brain damage restricted primarily to one hemisphere, large ipsilateral epileptogenic lesions involving the perisensorimotor area, refractory startle-provoked drop attacks, and a good response to epilepsy surgery.