The Rigid Form of Huntington's Disease

Campbell, A. M. G.; Corner, Beryl D.; Norman, R. M.; Urich, H.

doi:10.1136/jnnp.24.1.71

Cited by 50 publications

(16 citation statements)

References 13 publications

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“…In addition, careful absorption of HD sera with group A streptococcal cell walls, membranes, and purified carbohydrate, utilizing methods previously described (9), produced no diminution or significant Antineuronal Antibodies in Huntington's Chorea (0) (21,22). In both, damage to neurons and subsequent alteration of native autologous neuronal antigens might be capable of inducing synthesis of antibodies directed against damaged neuronal structures.…”

Section: Resultsmentioning

confidence: 99%

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

Husby¹,

Li²,

Davis³

et al. 1977

J. Clin. Invest.

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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.

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Section: Resultsmentioning

confidence: 99%

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

Husby¹,

Li²,

Davis³

et al. 1977

J. Clin. Invest.

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“…The SNc is thinner than controls, yet its number of neurons were originally reported to be apparently normal in all grades giving the impression of an increased density of pigmented neurons (Campbell et al 1961;Richardson 1990). However, other studies on the SNc have found cell loss (Oyanagi and Ikuta 1987;Oyanagi et al 1989) but less than that of the SNr (Ferrante et al 1989).…”

Section: Substantia Nigramentioning

confidence: 93%

“…There is a loss of neurons in the SNr (Lewy 1923;Spielmeyer 1926;Schroeder 1931;Hallervorden 1957;Campbell et al 1961;Richardson 1990). The SNc is thinner than controls, yet its number of neurons were originally reported to be apparently normal in all grades giving the impression of an increased density of pigmented neurons (Campbell et al 1961;Richardson 1990).…”

Section: Substantia Nigramentioning

confidence: 98%

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The Neuropathology of Huntington’s Disease

Waldvogel

Kim

Tippett

et al. 2014

Current Topics in Behavioral Neurosciences

205

138

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The basal ganglia are a highly interconnected set of subcortical nuclei and major atrophy in one or more regions may have major effects on other regions of the brain. Therefore, the striatum which is preferentially degenerated and receives projections from the entire cortex also affects the regions to which it targets, especially the globus pallidus and substantia nigra pars reticulata. Additionally, the cerebral cortex is itself severely affected as are many other regions of the brain, especially in more advanced cases. The cell loss in the basal ganglia and the cerebral cortex is extensive. The most important new findings in Huntington's disease pathology is the highly variable nature of the degeneration in the brain. Most interestingly, this variable pattern of pathology appears to reflect the highly variable symptomatology of cases with Huntington's disease even among cases possessing the same number of CAG repeats.

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“…The rigidity in the childhood form may be explained by the different pathological findings since the putamen is involved to a greater extent than it is in the adults. It has been shown in the adult with Huntington's disease who has rigidity as the principal manifestation, however, that destruction of the putamen is especially severe (Campbell et al, 1961).…”

Section: Disctissionmentioning

confidence: 99%