CNS SEQUELAE IN LANGERHANS CELL HISTIOCYTOSIS - PROGRESSIVE SPINOCEREBELLAR DEGENERATION AS A LATE MANIFESTATION OF THE DISEASE

Central nervous system involvement in Langerhans cell histiocytosis (L CAT), formerly known as histiocytosis X, is manifested mainly by diabe tes insipidus reflecting local infiltration of Langerhans cells into t he posterior pituitary or hypothalamus. We describe two patients with progressive spino-cerebellar degeneration appearing 4 and 6 years afte r the initial diagnosis of LCH. No correlation was found between the c linical course of the disease or its treatment and the neurological im pairment. An extensive search for metabolic, toxic, neoplastic, and he reditary etiologies for progressive cerebellar degeneration was negati ve.