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Startle epilepsy associated with infantile hemiplegia (SEIH): video-polygraphic features and long-term outcome.
Neuropediatrics. 2009 Apr; 40(2):97-100.N

Abstract

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).

MATERIAL AND 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.

Authors+Show Affiliations

Epilepsy Center, Neurological Unit, Casa Divina Provvidenza, Opera Don Uva, Bisceglie (BAT), Italy. giudorsi@yahoo.itNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

19809941

Citation

d'Orsi, G, et al. "Startle Epilepsy Associated With Infantile Hemiplegia (SEIH): Video-polygraphic Features and Long-term Outcome." Neuropediatrics, vol. 40, no. 2, 2009, pp. 97-100.
d'Orsi G, Demaio V, Operto F, et al. Startle epilepsy associated with infantile hemiplegia (SEIH): video-polygraphic features and long-term outcome. Neuropediatrics. 2009;40(2):97-100.
d'Orsi, G., Demaio, V., Operto, F., Auricchio, G., Minervini, M. G., & Coppola, G. (2009). Startle epilepsy associated with infantile hemiplegia (SEIH): video-polygraphic features and long-term outcome. Neuropediatrics, 40(2), 97-100. https://doi.org/10.1055/s-0029-1237722
d'Orsi G, et al. Startle Epilepsy Associated With Infantile Hemiplegia (SEIH): Video-polygraphic Features and Long-term Outcome. Neuropediatrics. 2009;40(2):97-100. PubMed PMID: 19809941.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Startle epilepsy associated with infantile hemiplegia (SEIH): video-polygraphic features and long-term outcome. AU - d'Orsi,G, AU - Demaio,V, AU - Operto,F, AU - Auricchio,G, AU - Minervini,M G, AU - Coppola,G, Y1 - 2009/10/06/ PY - 2009/10/8/entrez PY - 2009/10/8/pubmed PY - 2009/12/16/medline SP - 97 EP - 100 JF - Neuropediatrics JO - Neuropediatrics VL - 40 IS - 2 N2 - 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). MATERIAL AND 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. SN - 1439-1899 UR - https://www.unboundmedicine.com/medline/citation/19809941/Startle_epilepsy_associated_with_infantile_hemiplegia__SEIH_:_video_polygraphic_features_and_long_term_outcome_ L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-0029-1237722 DB - PRIME DP - Unbound Medicine ER -