Antibodies to human caudate nucleus neurons in Huntington's chorea.

Abstract

A B S T R APrevalence of antibody reacting with neuronal cytoplasm was 3% in 60 normal controls and 6% among a wide variety of patients with diverse neurological disorders. However, one-third of 33 patients with Parkinson's disease showed presence of antineuronal antibody. Among patients with HD, a significant association was noted between duration of clinical disease greater than 7 yr and titers of antibody of 1:2 or greater (P < 0.001).When 115 family members of HD probands were tested, 30% of unaffected spouses showed presence of antineuronal antibody. 23.2% of first-degree relatives at risk for developing HD were also positive (P < 0.001). 10.5% of second-degree relatives showed presence of antineuronal antibody. These data may support an environmental or infectious factor somehow involved in the ultimate expression of HD.

Hypersensitivity to antigen derived from brain tissue in patients with Huntington's disease and in individuals at risk.

“…One such protein released from the brain after injury and designated S-100 is precipitated by 100% saturated ammonium sulfate (16). The antigen responsible for hypersensitivity in our study differs from those described in other reports in terms of its greater specificity for HD (17,18), its precipitation with ammonium sulfate (18), and other properties (19 …”

HLA antigens in drug‐induced parkinsonism

“…A similar hypothesis has been proposed for Huntington's disease. Antibodies to human caudate nucleus neurons have been detected in some patients with Huntington's disease (43). An autoimmune response to dopamine receptors has also been proposed as a pathophysiological model for idiopathic parkinsonism (41).…”

Hypothalmic-Mediated Immune Mechanisms and Their Relevance to the Pathoettology of Parkinson's Disease

“…In that any damage to the hypothalamus may result in derangements of either systemic or CNS immune mechanisms including modification of experimental allergic encephalitis (EAE) (Wertman et al, 1985;Cross et al, 1980), and in so much as there is a reciprocal control of immune and neuroendocrine functions (Blalock et al, 1985), then it is conceivable that hypothalamic neuroimmunologic mechanisms might account for some subsequent derangements applicable to the pathoetiology of PD. Indeed, some preliminary evidence has accumulated to indicate deranged immune responsiveness in patients with PD (Pouplard et al, 1979;Husby et al, 1977). The fact that autoantibodies directed against sympathetic ganglia, adrenal medulla, and caudate nucleus have been discovered in patients with PD (Pouplard et al, 1979), and that these areas are rich in opioids (Cuello, 1983) potentially implies a primary postencephalitic autoimmune trigger directed at hypothalamic endorphinergic targets in both "idiopathic" and postencephalitic PD.…”