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.
Mittheisz
Edda
E
Children's Cancer Research Institute, Vienna, Austria.
Seidl
Rainer
R
Prayer
Daniela
D
Waldenmair
Marion
M
Neophytou
Birgit
B
Pötschger
Ulrike
U
Minkov
Milen
M
Steiner
Manuel
M
Prosch
Helmut
H
Wnorowski
Martha
M
Gadner
Helmut
H
Grois
Nicole
N
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