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Neuropediatrics 1980; 11(1): 57-71
DOI: 10.1055/s-2008-1071375
© Georg Thieme Verlag KG Stuttgart · New York

CHRONIC PROGRESSIVE ENCEPHALITIS IN CHILDREN WITH X-LINKED HYPOGAMMAGLOBULINEMIA

G.  Lyon , C.  Griscelli , E.  Fernandez-Alvarez , J.  Prats-Vinas , P.  Lebon
  • Laboratoire de Neurologie du Dévelopment, Université Catholique de Louvain, Bruxelles, and Laboratoire d'Immunologie Cellulaire, Hôpital des Enfants Malades, Paris
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Publication History

Publication Date:
19 March 2008 (online)

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Abstract

This report is on six cases of a chronic relentlessly progressive encephalitis occurring in boys with congenital hypogammaglobulinemia presumably of the x-linked type, which are thought to represent a separate neurological entity. Intellectual deterioration, dysarthria, spasticity, ataxia, optic atrophy and an increase of lymphocytes in the cerebrospinal fluid, were the main clinical signs. The pathological picture was that of a viral encephalitis, but all virological investigations on brain biopsies and CSF were negative. The significance of intra-cisternal tubuloreticular inclusions in brain endothelial cells, similarities with chronic rubella encephalitis, and the role of the immunological deficiency are discussed. Sofar, the cause of this new type of encephalitis remains obscure.

Key words

Chronic encephalitis - subacute encephalitis - hypogammaglobulinemia

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