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Neuropediatrics 2001; 32(5): 236-244
DOI: 10.1055/s-2001-19117
Original Article

Georg Thieme Verlag Stuttgart · New York

Clinical Spectrum of Epileptic Spasms Associated with Cortical Malformation

K. Kobayashi1 , Y. Ohtsuka1 , S. Ohno1 , I. Ohmori1 , T. Ogino1 , H. Yoshinaga1 , A. Tanaka2 , Y. Hiraki2 , E. Oka1
  • 1 Department of Child Neurology, Okayama University Medical School, Okayama, Japan
  • 2 Department of Radiology, Okayama University Medical School, Okayama, Japan
Further Information

Publication History

Publication Date:
18 December 2001 (online)

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Abstract

The spectrum of clinico-electrical characteristics of epileptic spasms associated with cortical malformation was studied in detail. The subjects were 15 patients suffering from spasms and cortical malformation demonstrated by MRI. The types of cortical malformation causing spasms were various, including hemimegalencephaly, diffuse pachygyria, focal cortical dysplasia, and polymicrogyria. Ohtahara syndrome was diagnosed in 3 patients, and West syndrome in 8. Symptomatic localization-related epilepsy preceded West syndrome in 4 patients, and a transition from Ohtahara syndrome to West syndrome was observed in one. West syndrome was followed by symptomatic generalized epilepsy including Lennox-Gastaut syndrome in 4 patients. Nine patients showed a condition which was labeled “epilepsy with partial seizures and spasms” (EPS) and characterized by the coexistence of partial seizures and spasms, and multifocal epileptic discharges on EEG. Spasms occurred only as EPS in 5 patients. EPS appeared following Ohtahara syndrome or West syndrome in 4 patients, and showed a transition to symptomatic localization-related epilepsy in 4. However, EPS did not evolve into generalized epilepsy, and persisted until the time of last follow-up in 5 patients. Therefore, the clinico-electrical pictures of patients with spasms and cortical malformation were diverse and not always limited within those of typical generalized epilepsy.

Key words

Cortical malformation - Infantile spasms - Periodic spasms - EEG - MRI

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M. D. Katsuhiro Kobayashi

Department of Child Neurology · Okayama University Medical School

5 - 1 Shikatacho-2 chome

Okayama, 700 - 8558

Japan

Email: k_koba@md.okayama-u.ac.jp

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