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Is Cerebral Arteritis the Cause of the Landau-Kleffner Syndrome? Four Cases in Childhood with Angiographic Study

Published online by Cambridge University Press:  18 September 2015

Ignacio Pascual-Castroviejo ,
V. López Martín ,
A. Martínez Bermejo  and
A. Pérez Higueras
Ignacio Pascual-Castroviejo*
Affiliation:
Departments of Paediatric Neurology and Neuroradiology, Hospital Infantil “La Paz”, Madrid, Spain
V. López Martín*
Affiliation:
Departments of Paediatric Neurology and Neuroradiology, Hospital Infantil “La Paz”, Madrid, Spain
A. Martínez Bermejo*
Affiliation:
Departments of Paediatric Neurology and Neuroradiology, Hospital Infantil “La Paz”, Madrid, Spain
A. Pérez Higueras*
Affiliation:
Departments of Paediatric Neurology and Neuroradiology, Hospital Infantil “La Paz”, Madrid, Spain
*
Servicio de Neurología Infantil, Hospital “La Paz”, Paseo de la Castellana 261, Madrid 28046, Spain
Servicio de Neurología Infantil, Hospital “La Paz”, Paseo de la Castellana 261, Madrid 28046, Spain
Servicio de Neurología Infantil, Hospital “La Paz”, Paseo de la Castellana 261, Madrid 28046, Spain
Servicio de Neurología Infantil, Hospital “La Paz”, Paseo de la Castellana 261, Madrid 28046, Spain
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Abstract:

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Four children with Landau-Kleffner syndrome were studied over a six year period. They presented with acquired aphasia, epilepsy, and focal or generalized EEG discharges which were exacerbated during sleep. In addition, cerebral angiography demonstrated isolated arteritis of some branches of the carotid arteries in all cases. Computed tomographic and magnetic resonance images were normal. Nicardipine in a dose of 1 to 2 mg/kg/day, added to conventional anticonvulsant drugs provided effective supplementary control of seizures, of paroxysmal EEG discharges, and of language and behavioural disturbances, even several years after the onset of the disorder and in patients whose response to other medications, including steroids, had been poor. Interruption of nicardipine administration was followed by relapse of the language disorder. Repeat angiography was performed in all four patients and showed recanal-ization of obstructed vessels in two cases. Focal cerebral vasculitis may be the pathogenesis of the Landau-Kleffner syndrome and calcium channel blockers such as nicardipine may be effective and specific therapy.

Type
Articles
Information
Canadian Journal of Neurological Sciences , Volume 19 , Issue 1 , February 1992 , pp. 46 - 52
Copyright
Copyright © Canadian Neurological Sciences Federation 1992

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