Central nervous system-related permanent consequences in patients with Langerhans cell histiocytosis.
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.


METHODS
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.


CONCLUSIONS
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.
Children's Cancer Research Institute, Vienna, Austria.
Edda
E
Mittheisz
Rainer
R
Seidl
Daniela
D
Prayer
Marion
M
Waldenmair
Birgit
B
Neophytou
Ulrike
U
Pötschger
Milen
M
Minkov
Manuel
M
Steiner
Helmut
H
Prosch
Martha
M
Wnorowski
Helmut
H
Gadner
Nicole
N
Grois
D000293:Adolescent; D000328:Adult; D002490:Central Nervous System / Q000473:pathology; D002493:Central Nervous System Diseases / Q000209:etiology* / Q000401:mortality / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D020790:Diabetes Insipidus, Neurogenic / Q000209:etiology* / Q000401:mortality / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000401:mortality / Q000473:pathology; D006801:Humans; D007223:Infant; D008297:Male; D008991:Monitoring, Physiologic; D010900:Pituitary Diseases / Q000209:etiology* / Q000401:mortality / Q000473:pathology