Langerhans histiocytosis involving the central nervous system
Yaniv, I.; Mor, C.; Matz, S.; Weitz, R.; Nussbaum, B.; Zaizov, R.
Harefuah 113(9): 230-233
1987
ISSN/ISBN: 0017-7768 PMID: 3501390 Document Number: 297389
Central nervous system involvement in histiocytosis usually manifests as diabetes insipidus due to hypothalamic infiltration. Late sequellae, including bulbar, cerebellar and neuropsychiatric disturbances, are usually attributed to the primary disease or to the treatment. We present a 16-month-old girl with disseminated Langerhans histiocytosis who during clinical remission developed convulsions and cortical blindness. Cranial computed tomography and magnetic resonance imaging revealed posterior, temporal and occipital involvement of the brain. Tissue examination of the involved area, using the immunoperoxidase technique with anti-S-100 protein confirmed the presence of Langerhans cells. Langerhans cell granules were identified in ultrasections of a skin biopsy. In view of the poor prognosis once the central nervous system is affected, and as we are likely to encounter more such complications due to improved therapy and prolonged survival, CNS prophylaxis is recommended early in the course of the disease.