[Organization of epilepsy care for people with mental retardation. A situational analysis after the care reform]. / Organiseringen av epilepsiomsorgen for mennesker med psykisk utviklingshemning. En situasjonsanalyse etter ansvarsreformen.

The special care services for the mentally retarded in Norway were closed down by law on 1 January 1991. This implied the termination of centralized care, and integration of these individuals into the local communities. To map the epilepsy service facilities for mentally retarded patients after the care reform, questionnaires were sent to all officially responsible community physicians and to all Departments of Neurology at Norwegian hospitals. 20% of the community physicians expressed the view that mentally retarded patients did not receive adequate specialist service for their epilepsy. There were wide variations within the country. 37% of the responders stated that the community care was inadequately equipped to attend to the special needs of these patients. Firstly, there seems to be a lack of professionals able to transfer their competence concerning epilepsy. Many of the neurological departments felt that the number of referrals of patients with epilepsy and mental retardation had increased since the care reform. 50% of the responders had no opinion about whether the quality of the epilepsy service was not the same for the mentally retarded as for other patients. Many did not realize the need for special measures to provide adequate medical follow-up of this group. It is concluded that education and guidance to personnel and carers should receive high priority in the further development of a comprehensive epilepsy service. The development of multiprofessional epilepsy units at all university hospitals should be facilitated, and the appointment of specially assigned epilepsy nurses at all neurological departments is recommended.

Effectiveness of zuclopenthixol compared with haloperidol in the treatment of behavioural disturbances in learning disabled patients.

BACKGROUND: We compared the efficacy of two neuroleptics with different receptor profiles (zuclopenthixol and haloperidol) in learning disabled patients with behavioural disturbance. METHOD: A double-blind crossover study (2 x 8 weeks; n = 34), interrupted by a two-week single-blind washout period, was employed. Assessments included the Schedule for Handicaps, Behaviour, and Skills (SHBS) and Clinical Global Impression (CGI). RESULTS: The SHBS score was significantly reduced for the zuclopenthixol cohort only. End-point analysis between the two drugs also showed an enhanced effect for zuclopenthixol over haloperidol. CGI scores did not reveal significant differences between the two drugs. CONCLUSION: Zuclopenthixol may be superior to haloperidol for the treatment of behavioural disturbances in mentally retarded subjects.

[Down's syndrome. Medical care and rehabilitation]. / Downs syndrom. Medisinsk oppfølging og habilitering.

The Norwegian Government has decided to reform the care of the mentally retarded by placing the responsibility for medical and social care with the local authorities. From 1 January 1991 the mentally retarded are to be integrated into the community. Persons with Down syndrome constitute 20-30% of the mentally retarded in institutions. The syndrome is the most common single cause of mental retardation. It represent both a medical challenge and a challenge in regard to home support, work, leisure activities and habilitation. Habilitation means identifying the fundamental deficiencies, incorporating this knowledge into plans for treatment and evaluating progress in social functioning and individual capacity. This presentation emphasizes clinically important information about the syndrome and medical complications both in the early stage of life, and among youths and adults in particular. Owing to difficulties in communication, there are often diagnostic problems. However, when recognized, many of the complications can be treated effectively.

Primordial birdheaded nanism associated with progressive ataxia, early onset insulin resistant diabetes, goiter and primary gonadal insufficiency. A new syndrome.

A new syndrome in two siblings with primordial birdheaded nanism, progressive ataxia, goiter, primary gonadal insufficiency and insulin resistant diabetes mellitus is presented. Plasma concentrations of TSH, PTH, LH, FSH, ACTH, glucagon and insulin all working through cell membrane receptors were elevated. A generalized cell membrane defect was suggested to be the pathophysiological abnormality in these patients.