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Neuropsychological sequelae in patients with neurodegenerative Langerhans cell histiocytosis
Author(s) -
van't Hooft Ingrid,
Gavhed Désirée,
Laurencikas Evaldas,
Henter JanInge
Publication year - 2008
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.21656
Subject(s) - neuropsychology , neurodegeneration , medicine , langerhans cell histiocytosis , verbal memory , visual memory , working memory , audiology , neuropsychological test , neuropsychological assessment , pediatrics , cognition , pathology , psychiatry , disease
Abstract Background Patients with Langerhans cell histiocytosis (LCH) may develop neurodegeneration and significant CNS sequelae, affecting a significant proportion of the patients. We here aimed to investigate the neuropsychological consequences in more detail. Methods Using an extensive neuropsychological test battery, we evaluated nine LCH patients, 6–20 years old, with radiological signs indicative of neurodegeneration. Results Altogether 3/9 patients performed below 1 SD of normal for age on full IQ. Detailed analysis revealed that 4/9 had deficient performance IQ, whereas 1/9 had subnormal verbal IQ (defined as below 1 SD). Furthermore, 3/8 patients showed slow speed of performance for age. Notably, 8/9 (89%) had deficient verbal working memory and 7/8 (88%) performed below normal on visual‐spatial working memory. Conclusions The results indicate a specific, uneven neuropsychological profile in patients affected by CNS‐LCH, with a decline particularly on perceptual tasks whereas the verbal performance was not as negatively influenced. Furthermore, verbal and visual‐spatial working memory functions were below normal for age in all but one patient studied. LCH may easily be misdiagnosed, but it is important that individuals affected by CNS‐LCH are diagnosed to provide advice and support. It remains a challenge to find a treatment reducing this unfortunate neurodegeneration. Pediatr Blood Cancer 2008;51:669–674. © 2008 Wiley‐Liss, Inc.