NEUROPSYCHOLOGICAL DEFICITS IN ADULTS WITH NEUROFIBROMATOSIS TYPE-1

Objectives - To examine neuropsychological deficits in adults with Neu rofibromatosis type 1 (NF1) with and without an affective mental disor der (i.e. dysthymia). Material and methods - Seventy adult NF1 patient s were followed-up after a 12-year period. Of 65 patients assessed ear lier with the Comprehensive Psychopathological Rating Scale (CPRS), 19 patients had died. The remaining patients were assessed by the CPRS w ith the exception of 4 patients, and were psychiatrically diagnosed ac cording to DSM III-R. Twelve patients were excluded because of advance d age, and psychiatric illness other than affective disorder. The rema ining 30 NF1 patients (7 with affective disorder and 23 psychiatricall y healthy), and a control group of 23 normal adults were assessed on a variety of neuropsychological tests. Results - Results indicated NF1- related deficits in inductive reasoning, visuoconstructive skill, visu al and tactual memory, logical abstraction, coordination, and mental f lexibility, although basic motor speed and vocabulary were not affecte d by NF1. An affective disorder exacerbated the neuropsychological def icits associated with NF1 only with regard to tests assessing motor fu nctions. Conclusions - NF1 results in a relatively global cognitive im pairment among adults, and additional depressive symptoms appear to sl ow down basic motor processes. It was speculated that the NF1-related cognitive deficits may partly result from white-matter lesions in subc ortical brain areas, due to proliferation of glial tissue, aberrant my elination or hamartomas.