Springer Science and Business Media LLC

We have developed monoclonal antibodies that detect normal microtubule-associated protein-2 (MAP2) epitopes in routinely fixed, paraffin-embedded tissue. The somatodendritic distribution of MAP2 in bovine and human nervous tissue was confirmed with several of these antibodies. Furthermore, some of these antibodies immunohistochemically labeled certain pathological structures in Alzheimer brain, especially neurites in senile plaques. Electron microscopic observations, however, indicate that these MAP2 epitopes are not located in the Alzheimer paired helical filaments themselves, but in amorphous granular structures coexistent with them. While the pathological nature of these structures is undetermined, they may represent artefactual modifications of normal cytoskeletal components.

A large suprasellar, partly cystic, contrast-enhancing tumor was resected from a 19-year-old woman who presented with bitemporal visual field defects and reduced visual acuity. Grossly, the tumor was brown and located in the subarachnoid space. Histologically, it was composed of spindle and pleomorphic cells, including giant tumor cells, with markedly pleomorphic nuclei. Reticulin fibers surrounded single cells and small groups of cells. Very few mitotic figures were found in the tumor, and no necrosis or microvascular proliferation was seen. The tumor thereby resembled a pleomorphic xanthoastrocytoma. Many of the tumor cells contained a dark-brown intracytoplasmic pigment, shown to be melanosomal melanin by ultrastructural examination. Immunohistochemical examination demonstrated that the pigment was present in glial tumor cells. Only four cases of pigmented astrocytic tumors have been published, none of these were suprasellar. Our patient received fractionated radiotherapy with a total dose of 48.6 Gy 14 months after gross total removal of the tumor. She is alive without relapse after 12-year follow-up.

Central nervous system (CNS) myelinspecific antiserum was capable of initiating primary demyelination within 24 h following injection into the dorsal column of guinea pig spinal cord. Control serum injected in the same manner did not produce demyelination. The demyelinating lesions occurred as local linear plaques of completely denuded intact axons surrounded by partially demyelinated and myelinated normal axons. Antiserum-mediated demyelination was followed by mononuclear cell infiltration 7–10 days later. Ultrastructural examination revealed vesiculation of myelin followed by cleavage of myelin lamellae at the intraperiod line. Remyelination began between 7 and 10 days following injection and correlated well with clinical evidence of recovery. The results of this study point to the importance of circulating autimyelin antibodies in the pathogenesis of demyelinating encephalitis. The model represents an in vivo approach to the study of the pathogenesis of immune-mediated myelinolysis in demyelinating disorders like multiple sclerosis (MS), subacute sclerosing panencephalitis (SSPE), and canine distemper encephalitis (CDE).

Menkes gene (Mc1 or MNK, encoding putative copper-transporting ATPase) expression was investigated and compared in normal and macular mutant mouse brain. Northern blot analysis showed a distinct 8.3-kb transcript and no obvious difference in size or extent in normal mice and macular mutants on postnatal days 0, 4, 7, 10 or 13. In situ hybridization revealed that certain specific populations of cells in the brain express Menkes mRNA, and that their localization in normal and mutant mice did not differ and was conserved on days 4, 10 and 13. The most intense hybridization signals were observed in the hippocampal CA1 region and dentate gyrus, the olfactory bulb nuclei, the cerebellar granular cell layer, the choroid plexus and the ependyma, with less intense signals in the hippocampal CA3 region and cerebellar Purkinje cells. In addition, necrotic neuronal cell death was predominantly observed in the CA3 region and the Purkinje cells of macular mice after postnatal day 10. The finding that the regions that had lower expression level of Menkes mRNA corresponded to those showing neuronal necrosis suggests that the Menkes gene may be responsible for the neuronal degeneration in some specific portions of the brain and clinical manifestations in this mutant.

Một dòng họ lớn với hai anh em được khám nghiệm tử thi có một hội chứng bao gồm chứng mất khả năng điều phối, chứng sa sút trí tuệ và một số đặc điểm giống như bệnh Parkinson được báo cáo; di truyền có vẻ là trội gen thường. Về mặt bệnh lý thần kinh, có sự hiện diện của các mảng và tân sinh neurofibril trong vỏ não cũng như một số trong nhân trung ương, đặc biệt giống như các mảng thấy trong Kuru; chỉ có một bệnh lý tủy sống tối thiểu (khu vực đường pyramidal). Những vấn đề liên quan đến việc phân loại tình trạng này - bệnh Alzheimer với sự liên quan của tiểu não hay các thực thể khác, chẳng hạn như tình trạng Gerstmann-Sträussler-Scheinker (1936), đặc biệt là bây giờ khi đã có báo cáo về việc truyền bệnh sang động vật trong trường hợp sau - được thảo luận. Một số khía cạnh lý thuyết liên quan thu được từ công trình trên động vật, đặc biệt là trong bệnh scrapie, cũng được xem xét.