Startle epilepsy associated with infantile hemiplegia (SEIH): video-polygraphic features and long-term outcome.
OBJECTIVE
The aim of this study was to investigate the video-polygraphic features and the long-term outcome of epilepsy in two patients with startle epilepsy associated with infantile hemiplegia (SEIH).


METHODS
Two patients (patient 1: a 14-year-old girl; patient 2: a 17 year-and-half-year-old girl), with hemiparesis and moderate mental retardation, underwent a full clinical and neurophysiological examination with video-polygraphic monitoring and recording of startle-evoked seizures. The follow-up was 9 years from epilepsy onset in patient 1, and 8 years from epilepsy onset in patient 2.


RESULTS
Firstly, video-polygraphic recordings of startle-evoked seizures, triggered by unexpected auditory stimuli, showed tonic asymmetrical postures with ictal EEG characterized by an abrupt and diffuse electrodecremental pattern or a seizure discharge predominant over the vertex and anterior regions controlateral to the posturing limbs. Electromyogram recording showed a prevalent involvement of proximal muscles with a concomitant tachycardia and apnoea. In particular, in patient 1 ictal heart rate was high, with persisting tachycardia for 60-120 s after the end of seizures. Secondly, a high seizure frequency persisted throughout the course of the disease, as seizures were medically refractory to all currently available anti-epileptic drugs.


CONCLUSIONS
The long-term outcome of epilepsy in SEIH, with constantly high seizure frequency, suggests an early surgical intervention, avoiding years with unsuccessful drug treatments and poor quality of life.
d'Orsi
G
G
Epilepsy Center, Neurological Unit, Casa Divina Provvidenza, Opera Don Uva, Bisceglie (BAT), Italy. giudorsi@yahoo.it
Demaio
V
V
Operto
F
F
Auricchio
G
G
Minervini
M G
MG
Coppola
G
G
D000161:Acoustic Stimulation / Q000009:adverse effects; D000293:Adolescent; D004569:Electroencephalography / Q000379:methods; D004827:Epilepsy / Q000150:complications* / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D006429:Hemiplegia / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D008607:Intellectual Disability / Q000209:etiology; D008137:Longitudinal Studies; D013216:Reflex, Startle / Q000502:physiology*; D014741:Video Recording / Q000379:methods