Landau-Kleffner syndrome evolving to electrical status epilepticus: a case report illustrating clinical heterogeniety

Article type: Research Article

Authors: Kalita, Jayantee | Singh, Mamta B. | Misra, Usha K. | Das, Birendra K.

Affiliations: Departments of Neurology, Post Graduate Institute of Medical Sciences Lucknow, India | Departments of Nuclear Medicine Sanjay Gandhi, Post Graduate Institute of Medical Sciences Lucknow, India

Note: [] Correspondence: Prof. Usha Kant Misra, Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences RaeBareli Road, Lucknow- 226 014, India. Fax: 91-0522-2668017. E-mail: ukmisra@sgpgi.ac.in

Abstract: A child with Landau-Kleffner syndrome who changed to continuous spike wave discharges both during sleep as well as awake is reported in this communication. A 4.5-year-old boy developed rapidly progressive mixed aphasia with relative preservation of writing and drawing skills. Electroencephalogram showed 2–2.5 Hz spike wave discharges more marked during slow wave sleep. Single photon emission tomography revealed hypoperfusion of the left frontotemporoparietal area. The patient did not respond to methylprednisolone, intravenous immunoglobulin and a number of antiepileptic drugs. At 2 year follow up there was no clinical improvement and the EEG showed continuous spike wave discharges not only during sleep but also during waking. He was aphasic but had preservation of spatial intelligence. This case report highlights the severe and resistant form of Landau-Kleffner syndrome and the heterogeniety of the condition. (J Pediatr Neurol 2004; 2(3): 159–162).

Keywords: Landau-Kleffner syndrome, aphasia, SPECT, EEG, electrical status epilepticus

Journal: Journal of Pediatric Neurology, vol. 2, no. 3, pp. 159-162, 2004