Journal of the neurological sciences
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Sera from rabbits with EAN induced by sensitization with galactocerebroside (GalC-EAN) bound to the surface of Schwann cells and oligodendrocytes in rat nervous system dispersion cultures. Sera from rabbits with bovine femoral nerve-induced EAN (FN-EAN) bound to Schwann cells, oligodendroglia and occasional fibroblasts. ⋯ Absorption of the capacity to bind to oligodendroglia and Schwann cells suggests that GalC is the major, if not the only surface antigen on these two cell types to which these sera bind. The capacity of GalC-EAN, SC-EAE, and FN-EAN sera to bind to the surface of the cells responsible for myelin synthesis in both PNS and CNS correlates with the ability of these sera to cause both PNS and CNS demyelination in vitro and PNS demyelination in vivo.
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Changes in the ependyma and periventricular brain tissues of the lateral, 3rd and 4th ventricles and the cervical spinal canal were studied electron-microscopically in young rabbits on the 9th day after injecting kaolin into the cisterna magna. The ependyma of the lateral ventricle overlying the white matter was notably stretched causing increased egress of CSF and disorganisation of the normal architecture of the white matter and capillaries. The neurons and glial cells close to the white matter showed edematous changes. ⋯ The height and the arrangement of the ependymal cells, the surrounding grey matter with narrow interstitial spaces and the absorbing tanycytes seemed to be factors which were responsible for the minimal changes in these regions. The changes appeared to be reversible if the CSF pressure was relieved at this stage. The spinal canal remained unaffected in the majority of our hydrocephalic animals, which could probably be attributed to the type of animal and the degree of hydrocephalus.
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Case Reports Comparative Study
Kuru plaques in the brain of two cases with Creutzfeldt-Jakob disease. A common origin for the two diseases?
We present two patients aged 66 and 69, with a rapidly progressive disease (10 and 15 months' duration) in which the presenting symptom was instability of gait. Later dementia was also a prominent feature. One case had myoclonus. ⋯ Neurons with large vacuoles in the cytoplasm were numerous in the putamen, thalamus and anterior horns. Stress is laid upon the common findings in both CJD and Kuru (K) (clinical features, pathological data, lack of antibody response, transmissibility, change in pattern on transmission). The possibility of a common origin of the two diseases is discussed.
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A patient with insidious onset of weakness, muscle atrophy and true hypertrophy, and sustained spasms is described. The spasms, initially mistaken to be myotonic, were shown electromyographically to result from sustained activity of the muscle fibres. ⋯ Histologic examination of sural nerve biopsy revealed large vacuoles within Schwann cells containing unidentified material, as well as onion-bulb formations. The possibility of a metabolic error resulting in storage of an abnormal material within Schwann cells is assumed.