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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Bibliographic Details
Published in:Pediatric hematology and oncology 1997, Vol.14 (6), p.577-584
Main Authors: Cervera, Aurea, Madero, Luis, Peñas, Juan J. García, Diaz, Miguel Angel, Gutiérrez-Solana, Luis G., Benito, Ana, Ruiz-Falcó, Mari-Luz, Villa, Marta
Format: Article
Language:English
Subjects:
Adolescent
Biological and medical sciences
central nervous system
cerebellum
Child
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Female
Histiocytosis, Langerhans-Cell - complications
Humans
Langerhans cell histiocytosis
Male
Medical sciences
Neurology
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
1521-0669
DOI:10.3109/08880019709030915