SOCIAL-ADJUSTMENT IN ADULT MALES AFFECTED WITH PROGRESSIVE MUSCULAR-DYSTROPHY

Adult male patients affected with Decker (BMD, N = 22), limb girdle (L GMD, N = 22) and facioscapulohumeral (FSHMD, N = 18) muscular dystroph y were interviewed to assess for the first time how the disease's seve rity and recurrence risk (RR) magnitude alter their social adjustment. BMD (X-linked recessive) is the severest form and confers an intermed iate RR because all daughters will be carriers, LGMD (autosomal-recess ive) is moderately severe with a low RR in the absence of consanguineo us marriage, and FSHMD (autosomal-dominant) is clinically the mildest of these three forms of MD but with the highest RR, of 50%. Results of the semistructured questionnaire [WHO (1988): Psychiatric Disability Assessment Schedule] showed no significant difference between the thre e clinical groups, but more severely handicapped patients as well as p atients belonging to lower socioeconomic levels from all clinical grou ps showed poorer social adjustment, Taken together, myopathic patients displayed intermediate social dysfunction compared to controls and sc hizophrenics studied by Jablensky [1988: WHO Psychiatric Disability As sessment Schedule]. Since the items of major dysfunction proportion am ong myopathic patients concern intimate relationships (70%), interest in working among those unemployed (67%), and social isolation (53%), e motional support and social and legal assistance should concentrate on these aspects. Interestingly, the results of this study also suggest that high RRs do not affect relationships to the opposite sex. (C) 199 8 Wiley-Liss, Inc.