A Psychiatric 12-year Follow-up of Adult Patients with Neurofibromatosis Type 1

The impact of neurofibromatosis type 1 (NF1) on psychiatric symptoms and diagnoses, personality variables and self evaluation was studied in a 12 year-follow-up of patients with NF 1 in the city of Göteborg, Sweden. 48 living adult patients with NF1 were re-evaluated in 1990 in a 12 year long time follow up study. The patients were diagnosed according to the Diagnostic and Statistical Manual of Mental Disorders. The following scales were used; the Comprehensive Psychopathological Rating Scale (CPRS), the Karolinska Scales of Personality inventory (KSP) and the Self-Evaluation Scale (SES). A significant psychopathology was found in the NFI patients, p <0.001. One third of the patients were affected by a psychiatric disease, 21% by dysthymia. There was no significant progress in psychiatric symptoms in the follow up period. The personality profile disclosed a heightened self-esteem. The chronic stigmatizing character of NF1 may be the reason for the increased psychopathology found.