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Biomedical subjects

W R Markesbery

Publications and source records attributed to W R Markesbery.

At least 217 records · Page 12Linked to original sources

Elevated manganese levels associated with dementia and extrapyramidal signs.

In a patient with elevated manganese levels, dementia, and an extrapyramidal syndrome, brain biopsy showed numerous neuritic plaques and neurofibrillary tangles typical of Alzheimer's disease. Manganese was elevated beyond toxic levels in serum, hair, urine, feces, and brain. The possible relationship of elevated manganese levels, dementia, and the extrapyramidal syndrome warrants further studies of similar cases.

Basal Ganglia Diseases↗

Distal myopathy: electron microscopic and histochemical studies.

This report describes the clinical, laboratory, and muscle biopsy histochemical and electron microscopic studies of one inherited and two sporadic cases of distal myopathy. Histopathologic and histochemical studies showed numerous myopathic alterations and no significant evidence of denervation. Electron microscopic studies showed a broad spectrum of nonspecific alterations similar to those in other forms of muscular dystrophy. Autophagic vacuoles were prominent in all cases. The inherited case was characterized by an unusual focal granular degeneration that, ultrastructurally, was composed of homogeneous fine granules devoid of other organelles or myofilamens.

Adult↗

Ultrastructural observations of the myenteric plexus of the pylorus in infantile hypertrophic pyloric stenosis.

Myenteric plexuses and smooth muscle of the pylorus obtained by biopsy at pyloro myotomy in 5 infants with hypertrophic pyloric stenosis were studied by electron microscopy. Animal controls consisted of pyloruses from 2 infant and 2 adult rabbits and 2 adult rats. The principal findings in the plexuses of the patients were moderate numbers of variably sized, swollen degenerating axons that contained dense bodies, lamellated figures, vacuoles, granular or fibrillar material, and swollen mitochondria. The significance of this alteration in the etiology and pathogenesis of this disorder is not clear. Neurons in the plexuses showed no definite abnormalities. Although these findings do not confirm previous light microscopic observation of neuronal changes in the pyloric myenteric plexuses, they do not exclude a neurogenic mechanism for this disorder. The presence of small immature neurons and large mature neurons suggests that neuronal maturation and development in infantile hypertrophic pyloric stenosis are not impaired as previously reported. No ultrastructural changes were found in interstitial cells of the myenteric plexuses. Except for hypertrophy of the circular smooth muscle layers, no specific alterations were found in muscle fibers of the pylorus.

Animals↗

Late-infantile neuronal ceroid-lipofuscinosis. An ultrastructural study of lymphocyte inclusions.

Ultrastructural study of peripheral blood from two patients with late-infantile neuronal ceroid-lipofuscinosis with curvilinear bodies, demonstrated in brain biopsies revealed curvilinear bodies in lymphocytes. These findings indicate that ultrastructural investigation of circulating lymphocytes may be useful in the diagnosis of late-infantile neuronal ceroid-lipofuscinosis and may circumvent the need for brain biopsy. Curvilinear bodies were also present in the lymphocytes of a neurologically normal younger female sibling of patient 1, who has had a single seizure, leading to speculation that she may be in the early stages of the same disease. This case suggests the possibility of early detection of this disorder and indicates the importance of screening lymphocytes of siblings of affected patients. Tubular cytoplasm inclusions were present in a high percentage of lymphocytes of the asymptomatic parents and a younger sibling of these patients.

Cerebral Cortex↗

Ultrastructural study of the pineal germinoma in vivo and in vitro.

The fine structure of a pineal germinoma was studied in tissue blocks and in tissue culture. The original tumor contained large primary tumor cells, lymphocytes, macrophages, plasma cells, and astrocytes. The large primary tumor cells were characterized by large nuclei, relatively scanty organelles, abundant glycogen, and junctional complexes. The neoplasm grew well in vitro and, with the exception of plasma cells, contained the same cell types found in vivo. The ultrastructural characteristics of the cells in vivo and in vitro were similar. In culture there were few large primary tumor cells. Macrophages, often involved in active phagocytosis, were prominent in the original tumor and were the most common cell type found in vitro. A tumor-associated immune response is postulated in this neoplasm.

Adolescent↗

Perivascular hemosiderin deposition in muscle in Waldenström's macroglobulinemia.

A 63-year old woman with Waldenström's macroglobulinemina of 5 years' duration developed disabling pain in her extremities. The patient had required frequent plasmapheresis and transfusion for treatment of hypervisosity. Muscle biopsy from 2 separate sites showed striking deposition of hemosiderin in macrophages surrounding small vessels and features of slight denervation. Immunofluorescence demonstrated IgM deposition in vessel walls. Perivascular hemosiderin deposition in muscle has not previously been desicribed and may reflect traansfusion-hemosiderosis in a patient with a dysglobulinemia.

Basement Membrane↗

Human fetal cerebellar cortex: organization and maturation of cells in vitro.

Human fetal cerebellar cortex was maintained up to 5 months in vitro. Important features included early migration of granule neurons followed by maturation of Purkinje and granule neurons. Unique areas of organization developed in which a rim of leptomeningeal cells surrounded an explant and its outgrowth zone; these areas subsequently grew as well-defined units.

Cell Nucleus↗