Neuropsychological sequelae in patients with neurodegenerative Langerhans cell histiocytosis.
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.
Van't Hooft
Ingrid
I
Childhood Cancer Research Unit, Department of Woman and Child Health, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden.
Gavhed
Désirée
D
Laurencikas
Evaldas
E
Henter
Jan-Inge
JI
D000293:Adolescent; D017668:Age of Onset; D001921:Brain / Q000473:pathology*; D001927:Brain Diseases / Q000209:etiology* / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology / Q000473:pathology; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000473:pathology* / Q000523:psychology; D006801:Humans; D007223:Infant; D008297:Male; D009410:Nerve Degeneration / Q000209:etiology / Q000473:pathology; D009483:Neuropsychological Tests