CNS sequelae in Langerhans cell histiocytosis: progressive spinocerebellar degeneration as a late manifestation of the disease

Central nervous system involvement in Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, is manifested mainly by diabetes insipidus reflecting local infiltration of Langerhans cells into the posterior pituitary or hypothalamus. We describe two patients with progressive spinocere...

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Published inPediatric hematology and oncology Vol. 14; no. 6; p. 577
Main Authors Cervera, A, Madero, L, García Peñas, J J, Díaz, M A, Gutiérrez-Solana, L G, Benito, A, Ruiz-Falcó, M L, Villa, M
Format Journal Article
LanguageEnglish
Published England 1997
Subjects
Adolescent
Child
Female
Histiocytosis, Langerhans-Cell - complications
Humans
Male
Spinocerebellar Degenerations - etiology
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Summary:Central nervous system involvement in Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, is manifested mainly by diabetes insipidus reflecting local infiltration of Langerhans cells into the posterior pituitary or hypothalamus. We describe two patients with progressive spinocerebellar degeneration appearing 4 and 6 years after the initial diagnosis of LCH. No correlation was found between the clinical course of the disease or its treatment and the neurological impairment. An extensive search for metabolic, toxic, neoplastic, and hereditary etiologies for progressive cerebellar degeneration was negative.
ISSN:0888-0018
DOI:10.3109/08880019709030915