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Biomedical subjects

R D Jolly

Publications and source records attributed to R D Jolly.

At least 145 records · Page 8Linked to original sources
PubMed1967

A viral gastroenteritis of Ontario swine. 1. Clinical illness and recovery of the virus.

A severe gastroenteritis affected Ontario pigs in the Spring of 1964 and again in 1966. The mortality of pigs less than a week of age was 100%, in older pigs there were few deaths although morbidity in these pigs approximated 100%. Viruses were isolated from the brains, intestines, and intestinal lymph nodes of baby pigs. One of these isolates, given by mouth to two colostrum deprived piglets, induced a severe enteritis 36 hours after administration and both of the piglets died 24 hours later. The characteristics of the disease and the virus bear a striking resemblance to transmissible gastroenteritis as seen in the United States of America and the United Kingdom.

Animals↗
PubMedDate not supplied

Lysosomal storage diseases.

The majority of lysosomal storage diseases affect the central nervous system. Those that reflect a primary lysosomal disorder are associated with genetically determined deficiencies of specific lysosomal enzymes and storage of the relevant substrate. Autofluorescent lipopigments accumulate in the ceroid-lipofuscinoses, a heterogeneous group of diseases in which lysosomal storage is thought to be a secondary event. In animals, there occurs a group of toxic storage diseases whose pathology mimics that of some of the genetic diseases. In humans some element of control may be achieved by heterozygote detection programmes and/or prenatal diagnosis of pregnancies at risk with elective abortion of an affected foetus. The outlook for specific therapy is not encouraging at this stage.

Enzymes↗
PubMedDate not supplied

Ovine ceroid-lipofuscinosis: a model of Batten's disease.

An inherited neurological disease of sheep was characterized by the intracellular accumulation of autofluorescent lipopigments in neurones and a wide variety of other cells within the body. The staining, fluorescent, ultrastructural and physical characteristics of the storage material were similar to those found in a heterogeneous group of storage diseases of children known as Batten's disease or the ceroid-lipofuscinoses. The ovine disease did not exactly fit any of the main human entities, but had features in common with both the late infantile and juvenile forms. It was concluded that this was a useful model for studying the pathogenesis of this type of storage disease and for therapeutic trials. A flock of sheep is maintained for this purpose.

Animals↗
PubMedDate not supplied

Ceroid-lipofuscinosis (Batten's disease): pathogenesis of blindness in the ovine model.

Blindness is a feature of the group of storage diseases of children known as the ceroid-lipofuscinoses. Sequential studies in the ovine model, which most resembles the juvenile form of human disease, showed clearly that blindness had two components, a central and a peripheral. Whereas the central component, attributable to neuronal death and atrophy of the cerebral cortex, was responsible for early loss of vision, retinal atrophy was also extremely advanced in terminal stages of the disease. The primary retinal change was one of dystrophy of photoreceptor outer segments which preceded degeneration and necrosis of the photoreceptors cells themselves. Electroretinography showed that there was a progressive loss of a- and b-waves during the course of the disease, but this was preceded by a diminished c-wave which was eventually replaced by a negative potential. However, the pigment epithelium remained functionally (azide responsive) and ultrastructurally intact throughout the study. Loss of brain weight with selective cerebral atrophy also correlated with abnormal behaviour and facial manoeuvres that were interpreted as partial seizures that did not become generalized.

Animals↗
PubMedDate not supplied

Stringhalt in horses: a distal axonopathy.

A detailed investigation of the neuropathology of a horse affected with stringhalt was performed. Qualitative and quantitative light and electron microscopy, and single teased fibre preparations of peripheral nerve demonstrated predominantly axonal degeneration, the stage of which was appropriate for the duration of clinical signs. There was selective involvement of large myelinated nerve fibres. A proximal to distal increase in the severity of pathological changes was present in the peripheral nerves. The long left recurrent laryngeal nerve was the most severely affected, followed in order by its right counterpart, the hindlimb and forelimb nerves. Neurogenic atrophy of muscles innervated by affected peripheral nerves also showed a distally graded increase in severity. No lesions were observed in the central nervous system. It was concluded that this disease should be classified as a distal axonopathy.

Animals↗
PubMedDate not supplied

Ceroid-lipofuscinosis (Batten's disease): pathogenesis and sequential neuropathological changes in the ovine model.

A sequential morphological study of ovine ceroid-lipofuscinosis showed that brains of affected lambs were normal at birth, grew until four months of age but then atrophied. Laminar necrosis of cerebral cortex was noted at 10 weeks, occurring first in the parietal area, then spreading to involve frontal and occipital areas while the temporal lobe was least and last affected. With progression of the disease, the laminar pattern was lost. Neuronal necrosis was accompanied by a severe astrocytosis. The granular and multilamellar storage cytosomes increased in size with age. Their structure was interpreted as paracrystalline in which repeating molecules of the dominantly stored lipid binding subunit of mitochondrial ATP synthase interact with neutral lipids and phospholipids. Abnormal cytosomes in neurons of lamb fetuses and a neonate were interpreted as early lesions which contained whorls or stacks of bilayered membrane as well as the more complex multilamellar material. The underlying anomaly leading to the storage of the lipid binding subunit of mitochondrial ATPsynthase remains to be defined. However, it is noted that this disease should be regarded as a lipid binding protein proteinosis or alternatively as a proteolipid proteinosis.

Age Factors↗