Neuropsychological sequelae in patients with neurodegenerative Langerhans cell histiocytosis.

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.

journal_name

Pediatr Blood Cancer

journal_title

Pediatric blood & cancer

authors

Van't Hooft I,Gavhed D,Laurencikas E,Henter JI

doi

10.1002/pbc.21656

subject

Has Abstract

pub_date

2008-11-01 00:00:00

pages

669-74

issue

5

eissn

1545-5009

issn

1545-5017

journal_volume

51

pub_type

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