Document Detail

Central nervous system-related permanent consequences in patients with Langerhans cell histiocytosis.
MedLine Citation:
PMID:  16470521     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Permanent consequences in Langerhans cell histiocytosis (LCH) are irreversible late sequelae related to the disease that may severely impair the quality of life of survivors. The frequency and pattern of permanent consequences affecting the central nervous system (CNS) remains to be determined. PROCEDURE: In this single center study, 25 LCH patients observed for a median time of 10 years 3 months underwent a uniform thorough follow-up program including neuropsychological testing and electrophysiological evaluation. RESULTS: Overall permanent consequences were seen in 9 of 25 patients. Intracranial abnormalities were the most frequent including diabetes insipidus (DI) in seven patients, anterior pituitary deficiencies in five patients, and neurodegenerative CNS disease in five patients. No patient had overt neurological symptoms upon neurological evaluation, but psychological testing revealed subtle deficits in short-term auditory memory (STAM) in 14 patients. Brain stem evoked potentials showed abnormalities in four of nine tested patients, all of these four had neurodegeneration on MRI. CONCLUSION: Psychoneuroendocrine sequelae were found in an unexpectedly high number of patients in this single center study. Long-term follow-up focusing on such sequelae are important in LCH survivors, in order to detect early deficits, to monitor the evolution of the disease, and to provide specific support.
Edda Mittheisz; Rainer Seidl; Daniela Prayer; Marion Waldenmair; Birgit Neophytou; Ulrike Pötschger; Milen Minkov; Manuel Steiner; Helmut Prosch; Martha Wnorowski; Helmut Gadner; Nicole Grois
Publication Detail:
Type:  Clinical Trial; Journal Article    
Journal Detail:
Title:  Pediatric blood & cancer     Volume:  48     ISSN:  1545-5009     ISO Abbreviation:  Pediatr Blood Cancer     Publication Date:  2007 Jan 
Date Detail:
Created Date:  2006-11-23     Completed Date:  2007-02-21     Revised Date:  2009-01-12    
Medline Journal Info:
Nlm Unique ID:  101186624     Medline TA:  Pediatr Blood Cancer     Country:  United States    
Other Details:
Languages:  eng     Pagination:  50-6     Citation Subset:  IM    
Copyright Information:
(c) 2006 Wiley-Liss, Inc.
Children's Cancer Research Institute, Vienna, Austria.
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MeSH Terms
Central Nervous System / pathology
Central Nervous System Diseases / etiology*,  mortality,  pathology
Child, Preschool
Diabetes Insipidus, Neurogenic / etiology*,  mortality,  pathology
Follow-Up Studies
Histiocytosis, Langerhans-Cell / complications*,  mortality,  pathology
Monitoring, Physiologic
Pituitary Diseases / etiology*,  mortality,  pathology

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