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.
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.
authors: Department of Neurology, Barrow Neurological Institute, Phoenix, Ariz., USA.
authors: Christopher
authors: C
authors: Dardis
authors: Department of Neurology, Barrow Neurological Institute, Phoenix, Ariz., USA.
authors: Thandar
authors: T
authors: Aung
authors: Department of Neurology, Barrow Neurological Institute, Phoenix, Ariz., USA.
authors: William
authors: W
authors: Shapiro
authors: Department of Neuropathology, Barrow Neurological Institute, Phoenix, Ariz., USA.
authors: John
authors: J
authors: Fortune
authors: Department of Neuropathology, Barrow Neurological Institute, Phoenix, Ariz., USA.
authors: Stephen
authors: S
authors: Coons
keywords: Extrapulmonary manifestation; Langerhans cell histiocytosis; Neurodegeneration; Paraneoplastic inflammation