Journal of Pediatric Neurology 2004; 02(03): 159-162
DOI: 10.1055/s-0035-1557212
Case Report
Georg Thieme Verlag KG Stuttgart – New York

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

Jayantee Kalita
1   Departments of Neurology, Post Graduate Institute of Medical Sciences, Lucknow, India
,
Mamta B. Singh
1   Departments of Neurology, Post Graduate Institute of Medical Sciences, Lucknow, India
,
Usha K. Misra
1   Departments of Neurology, Post Graduate Institute of Medical Sciences, Lucknow, India
,
Birendra K. Das
2   Departments of Nuclear Medicine Sanjay Gandhi, Post Graduate Institute of Medical Sciences, Lucknow, India
› Author Affiliations

Subject Editor:
Further Information

Publication History

19 December 2003

15 April 2004

Publication Date:
29 July 2015 (online)

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).