DIENCEPHALIC INVOLVEMENT IN GENERALIZED LIPODYSTROPHY - RATIONALE AND TREATMENT WITH NEUROLEPTIC AGENT, PIMOZIDE

Generalized lipodystrophy (GLD) possesses, as one of its manifestations, diencephalic neuroendocrinopathy. Deranged hypothalamic catecholaminergic mechanisms may lead to the hypersecretion of hypophysiotrophic hormones that are detectable in the peripheral circulation. Initial treatment of a pre-puberal GLD patient with chlorpromazine was unrewarding. Chronic treatment of the same patients with the selective dopaminergic blocking agent, pimozide, eliminated corticotropin releasing factor and luteinizing hormone releasing factor activities from the general circulation. Pimozide treatment ameliorated most of the distorted blood chemistry profile, led to return of facial subcutaneous fat, and elimination of cutaneous hyperpigmentation, fever, abdominal pain, hypertrichosis, oilness of hair, scaliness of skin and to a state of general well-being.