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Biomedical subjects

R Fankhauser

Publications and source records attributed to R Fankhauser.

At least 37 records · Page 2Linked to original sources

Hereditary neurovisceral mannosidosis associated with alpha-mannosidase deficiency in a family of Persian cats.

Several kittens in a family of Persian cats had a metabolic storage disease. Clinically the disorder was characterized by hepatomegaly, neurological signs and early death. The microscopic lesions consisted of widespread vacuolation of neurons and glial cells in the central nervous system and in liver cells. Electronmicroscopically the lesions consisted of intracytoplasmic accumulation of membrane-bound "empty" vacuoles. In addition to the storage disease, poor myelination of the cerebral white matter was found. The defect was reproduced in breeding trials. On biochemical analysis of brain tissue, deficient function of the enzyme alpha-mannosidase was detected. The clinical and pathological features of mannosidosis in Persian cats were compared to similar defects in other species.

Animals↗

Immunoglobulins in demyelinating lesions in canine distemper encephalitis. An immunohistological study.

The brains of 14 dogs with canine distemper encephalitis were examined with immunohistologic techniques to search for immunoglobulin in demyelinating lesions. Four types of lesions presumably representing a temporal sequence of lesion development were distinguished. Immunohistologic findings included immunoglobulin bearing lymphoid cells, amorphous Ig containing material, immunoglobulin bound to the tissue and immunoglobulin containing macrophages and astrocytes. The humoral immune response was absent or very minimal in acute lesions and very intense in chronic lesions. It was concluded that early demyelination in canine distemper encephalitis occurs in the absence of a local humoral immune response but that this response may aggravate and accelerate myelin destruction in the later stages of the disease.

Acute Disease↗

Immunohistological studies on primary reticulosis of the canine brain.

Brains of 17 dogs with primary reticulosis, and of two with metastatic and one with primary lymphosarcoma, were examined by immunohistologic techniques with antisera against the major immunoglobulin classes of the dog. On the basis of immunoglobulin in tumour cells, two reticuloses were reclassified as primary lymphosarcomas of the brain. The tumour cells in three other dogs with neoplastic reticulosis and in six dogs with microgliomatosis did not contained immunoglobulin. Many cells in inflammatory reticulosis were immunoglobulin-bearing. Although some of these lesions could have been lymphosarcomas, insufficient data were available to ascertain either an inflammatory or a neoplastic nature. It was concluded that some lesions previously classified as reticulosis are in fact primary histiocytic lymphosarcomas of the brain, while others may be true reticulosarcomas.

Animals↗

Encephalopathy with Rosenthal fibre formation in a sheep.

In the brain of a 4-year-old ewe extensive Rosenthal fiber (RF) formation was found in a subpial and subependymal location and in particular in the cerebellar white matter. Here, the RFs were concentrated around blood vessels, and there was diffuse myelin rarefaction. The animal had given birth to two lambs, which were still healthy at the age of 3 weeks when their mother died after a short illness with astasia and alternating states of somnolence and hyperexcitability.

Animals↗