Title

Langerhans Cell Histiocytosis in an Adult With Involvement of the Calvarium Cerebral Cortex and Brainstem: Discussion of Pathophysiology and Rationale for the use of Intravenous Immune Globulin

Department

neurology

Document Type

Article

Abstract

We report a case of Langerhans cell histiocytosis in a 64-year-old male who presented with symptoms and signs of brain involvement, including seizures and hypopituitarism. The diagnosis was confirmed with a biopsy of a lytic skull lesion. The disease affecting the bone showed no sign of progression following a short course of cladribine. Signs of temporal lobe involvement led to an additional biopsy, which showed signs of nonspecific neurodegeneration and which triggered status epilepticus. Lesions noted in the brainstem were typical for the paraneoplastic inflammation reported in this condition. These lesions improved after treatment with cladribine. They remained stable while on treatment with intravenous immune globulin.

Medical Subject Headings

neurology

Publication Date

2015

Publication Title

Case Reports in Neurology

ISSN

1662680X

Volume

7

Issue

1

First Page

30

Last Page

38

Digital Object Identifier (DOI)

10.1159/000380760

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