A 25-year-old man was admitted to the Department of Neurology, Gunma University Hospital, in June 1997. He had an intellectual disability and had suffered from repeated infection since childhood. Cerebellar ataxia had developed at 19 years of age and he had been clinically diagnosed with ataxia telangiectasia (AT) comprising cerebellar ataxia and oculocutaneous telangiectasia at 24 years of age. He died from pneumonia and renal failure at 26 years of age. Neuropathological examination revealed Purkinje cell loss and atrophy of the dentate nuclei in the cerebellum, anterior horn-cell atrophy and demyelination of the gracile fasciculi in the spinal cord, and the existence of nucleocytomegalic cells in the anterior pituitary gland. These neuropathological findings correlated with previously reported cases of AT. In addition, spongy degeneration was found, predominantly around the blood vessels in the cerebral cortex. Diffuse spongy degeneration and multiple foci of coagulative necrosis with calcification were noted in the white matter. Abnormal vasculature was noted in both degenerative and necrotic areas in the cerebral cortex and in the white matter. The vessels at the center of the areas of spongy degeneration in the cerebral cortex had irregularly arranged and enlarged smooth-muscle-cell nuclei and a distorted, narrow lumen. The vessels present in the white matter were hyalinized. To our knowledge, these vascular abnormalities in the brain parenchyma have not been reported previously.

No abstract

Cellular markers are useful in the immunohistochemical studies of normal and pathological tissues. Herein, the development of a monoclonal antibody, Schwann/2E, which reacts with Schwann cells and myelin of the peripheral nervous system (PNS) is described. The Schwann/2E antibody was secreted by a hybridoma of mouse myeloma cells and mouse spleen cells that were immunized in vivo with a cytoskeletal fraction of the human spinal nerve. This antibody immunostained formalin-fixed, paraffin-embedded human, rat, and mouse tissue by the indirect immunoperoxidase method. Schwann cells and myelin of the PNS were intensely labeled by the Schwann/2E antibody. Both the nuclei and cytoplasm of the Schwann cells were labeled. As shown by a comparative light and an electron microscopic study, the Schwann/2E antibody immunoreacted with the Schwann cells that had myelinated axons, but not with those that had unmyelinated axons. The endoneurial fibroblast was not immunolabeled. This antibody slightly stained the endothelial cells of the lung and kidney. Myelin and oligodendroglia of the central nervous system did not react with the Schwann/2E antibody. The Schwann/2E antigen was stable in several histological fixatives. These results indicate that, under normal and pathological conditions, the Schwann/2E antibody could be a useful immunohistochemical marker of Schwann cells and myelin of the PNS.

A report of an unusual esophageal tumor in an 81-year-old man is presented. The primary tumor was diagnosed as undifferentiated carcinoma at biopsy and had disappeared after irradiation treatment. However, multiple metastases were noted in the brain, lungs, kidneys, adrenals and spleen at autopsy. Histologically, metastases showed marked cartilaginous metaplasia as demonstrated by light microscopy, histochemical and immunohistochemical studies, although the initial biopsy sample did not possess chondroid matrix. Furthermore, an apparent transition could be traced from carcinomatous to chondroid cells, suggesting that the chondroid cells were derived from carcinoma cells. The carcinomatous area partially showed both squamous and glandular differentiation, although they were poorly differentiated. A retrospective immunohistochemical study that used a panel of antibodies suggested a phenotypic relevance between primary and metastatic tumors.

Ten hybridomas producing monoclonal antibodies against pineal antigens were established by hybridizing mouse myeloma cells with spleen cells from BALB/c mice immunized with human pineal body homogenate. Seven antibodies immunohistochemically reacted with the pinealocytes and three reacted with the pineal interstitial cells. According to the antibodies applied, the pinealocytes were immunostained in a variable manner; granularly with PP1, PP4 and PP6 antibodies, diffusely with PP2 and PP5, membraneously with PP3, and apically with PP7. The PI1 and P12 antibodies immunolabeled most of the interstitial cells, but PX1 immunolabeled only a small population of these cells. An immunoblotting study indicated that single or multiple polypeptides of the pineal homogenate constituted the antigen epitopes for these antibodies. Most antibodies also immunoreacted with several cell types of the extra-pineal tissues, thus these antibodies are not specific to the human pineal body. This series of monoclonal antibodies should be available for immunohistochemical studies involving the normal and pathological human pineal body.