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It was difficult to make a definite pathological diagnosis in a 73-year-old man with Creutzfeldt-Jakob disease (CJD) due to extensive amyloid angiopathy which lacked any severe spongiform changes. Immunostaining using anti-prion protein (PrP) antibody revealed fine granular deposits in the gray matter, after hydrolytic autoclaving pretreatment on tissue sections. Western blotting also revealed an abnormal isoform of PrP, but PrP gene analysis did not show any abnormalities. The primary transmission experiments were repeated three times and induced spongiform encephalopathy in a few mice after a long incubation period.

A primary Burkitt-type lymphoma of the CNS was reported which developed in an 11-year-old boy 6 months after extirpation of an astrocytoma. Primary Burkitt-type lymphomas have been described only twice in the literature. The morphological aspect is interesting with regard to the problem of collision tumors.

A new family with the Gerstmann-Sträussler type of subacute spongiform encephalopathy is described. Atactic symptoms, dysarthrias, and personality changes characterized the clinical course. The clinical pattern of a father and his two sons was very similar. They had been in contact with sheep by occupation. A rapidly progressing demyelinating disease (transitional diffuse-multiple sclerosis) occurred in the same family, as well as numerous cases of carcinoma. Morphologically, the Sträussler type can be differentiated from other subacute spongy encephalopathies by the occurrence of Kuru-Plaques and numerous multicentric floccular plaques both in the cerebral and cerebellar cortex, basal ganglia, and white matter.

A case of a fibrous xanthomatous tumor of the meninges is reported. This is a rare tumor of childhood in which the characteristic pleomorphic histology contrasts with the good clinical prognosis. These tumors were reclassified as pleomorphic xanthoastrocytomas (PXA) due to their glial fibrillary acidic protein (GFAP) positivity. In the present tumor, GFAP was absent from nearly all cell bodies in most of the leptomeningeal regions of the tumor but could be detected with greater frequency at the cortical-leptomeningeal border zones and in the areas in which the tumor had infiltrated the cortex. All the tumor cells expressed vimentin and in, addition, most expressed alpha-1-antitrypsin, alpha-1-antichymotrypsin, tartrate-resistant acid phosphatase, common leukocyte antigen, and OKM1. This spectrum of marker staining corresponded not only to the pattern observed in two cutaneous fibrous histiocytomas and one malignant fibrous histiocytoma, but also to the results previously published in the literature with regard to fibrohistiocytic tumors. By contrast, this spectrum of monocytic-histiocytic marker staining was not seen in gliomas. We, therefore, regard the PXA as a mesenchymal tumor of the meninges, identical to benign fibrous histiocytomas elsewhere in the body. The possible reasons why this mesenchymal tumor can show GFAP positivity in the leptomeningeal border zone are discussed.

The process of Giant Axonal Neuropathy (GAN) is not restricted to the peripheral nerves, but also involves the central nervous system. In a 25 year old man with normal hair, abundant axon swellings and spheroids were observed in the spinal cord, brain system, and cerebral cortex. The findings in the sural nerve have already been published by Boltshauser et al. (1977). Accumulations of filaments in the axons and in the perineural cells were accompanied by Rosenthal fibres. The ultrastructural pattern of GAN differs clearly from that of Neuroaxonal Dystrophies.

In 30 meningiomas we investigated the proliferation rate of various subtypes with the monoclonal antibody Ki-67. Frozen sections were incubated with Ki-67 antibody using a modified Alkaline Phosphatase anti-Alkaline Phosphatase (APAAP)-technique and evaluation of proliferation rate was done by cell counting. Meningiomas of the meningiotheliomatous, fibrous and angioblastic subtype without atypical histological findings contained 1% or less proliferating cells. In recurrent tumors, in transitional and in anaplastic meningiomas there is a marked increase of proliferating cells up to 20%. The distribution of marked cells varies in recurrent tumors and anaplastic meningiomas, and a focal proliferation of tumor cells was seen in meningiomas from transitional type. Immunohistological labelling of proliferating cells in meningiomas may allow a more precise prediction of the proliferation potential of each meningioma.