Granular deposits of C9 and the terminal complement complex, measuring 0.3-1.2 microns, have been demonstrated immunocytochemically in association with capillary endothelial cells, predominantly within plaques and adjacent white matter, in tissue obtained at autopsy from 5/7 patients with multiple sclerosis (MS) and one individual with subacute sclerosing panencephalitis but not from 7/7 controls. This finding suggests that the evolution of focal tissue damage in MS may involve complement activation associated with passage of humoral and cellular mediators of the immune system through the blood-brain barrier.
The distribution and severity of the neuropathological changes in 57 cases of Alzheimer's disease, and 11 patients with Down's syndrome were investigated with reference to the cerebellum. A modified silver stain and a monoclonal antibody raised against amyloid beta-protein were used to identify amyloid plaques. The highest incidence of amyloid plaques in the cerebellum (93%) was found in the group of patients who developed dementia before 65 years of age. This figure dropped to 56% in those patients with dementia beginning after 75 years. In 37 of these cases the distribution of the pathological changes of the disease were also examined in the brain stem. The severity of the pathological changes in the cerebellum corresponded to the involvement of the brain stem nuclei with connections to the cerebellar cortex. The possibility that the disease process spreads to the cerebellum by involving the fibres from the brain stem is discussed with reference to previous anatomical and neurochemical studies.
We have studied the inheritance of several polymorphic Xq27/28 DNA marker loci in two three generation families with the X linked neonatal lethal form of centronuclear/myotubular myopathy (XL MTM). We found complete linkage of XLMTM to all four informative Xq28 markers analysed, with GCPIRCP (Z=3-876, 0=0.00), with DXS15 (Z= 3.737, 0=0-00), with DXS52 (Z=2*709, 0=0-00), and with F8C (Z= 1-020, 0=0.00). In mitochondria, glycogen, and large nuclei, singly or in chains. This central area is similar in appearance to the myotubes seen in fetal muscle. Central nuclei are seen in other neonatal myopathies, including congenital myotonic dystrophy, but do not usually persist in these conditions, either in tissue culture or in muscle biopsies taken at a later stage. In contrast, the myotubular appearance does persist in the severe neonatal MTM, being found after tissue culture for several weeks,5 and on repeat muscle biopsy after many months.6 This supports the concept of this form of MTM displaying a maturational arrest of muscle ultrastructural development, and so the pathogenetic term 'myotubular' is preferred by some to the purely descriptive 'centronuclear', although neither word encompasses all the pathological characteristics of the disorder. 4 There are some features of MTM that suggest a neurogenic element in its aetiology. Thus, degeneration and regeneration have been described in axons of the sciatic nerve of a male infant who died at 9 months.6 The fact that this boy survived for so long might indicate, however, that the diagnosis in his case could be distinct from the X linked severe neonatal form considered in this paper. In another study, electromyography indicated
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