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Central nervous system‐related permanent consequences in patients with Langerhans cell histiocytosis
Author(s) -
Mittheisz Edda,
Seidl Rainer,
Prayer Daniela,
Waldenmair Marion,
Neophytou Birgit,
Pötschger Ulrike,
Minkov Milen,
Steiner Manuel,
Prosch Helmut,
Wnorowski Martha,
Gadner Helmut,
Grois Nicole
Publication year - 2007
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.20760
Subject(s) - medicine , langerhans cell histiocytosis , diabetes insipidus , neuropsychology , central nervous system , neurodegeneration , histiocytosis , pediatrics , disease , pathology , cognition , psychiatry
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. Pediatr Blood Cancer 2007;48:50–56. © 2006 Wiley‐Liss, Inc.