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Biomedical subjects

I Janota

Publications and source records attributed to I Janota.

At least 37 records · Page 2Linked to original sources
PubMed1988

Adult polyglucosan body disease (APBD).

Three patients aged 63, 63 and 74 years had various combinations of progressive lower and upper motor neuron dysfunction, sensory loss, urinary incontinence and dementia. Postmortem examinations in two cases showed moderate cerebral and spinal atrophy, ill-defined areas of incomplete myelin loss in white matter and small necrotic foci in the white matter of gyri, around the basal ganglia and near the dentate nuclei. The main microscopic abnormality was a massive accumulation of PAS-positive polyglucosan bodies (PB) of various sizes and shapes in the cerebral hemispheres, brainstem, cerebellum, spinal cord, nerve roots and nerves. These PB were found in the processes of nerve cells and astrocytes, but not in their perikarya. Similar PB were present in peripheral nerves and in the lungs, heart, liver and kidneys. In the third case, a nerve biopsy revealed several, unusually large, PB in the axons of myelinated fibers. These clinicopathologic features are consistent with adult polyglucosan body disease (APBD) and are distinctive from other conditions in which PB may accumulate. Twelve similar cases have been reported previously. The diagnosis can be made by nerve biopsy. The pathogenesis of APBD is not known, but it may be a polysaccharide storage disease.

Aged↗
PubMed1987

Relationships between neuropathology and cognitive functioning in temporal lobectomy patients.

Cognitive functions were examined before operation and 4 weeks after operation with respect to pathology in 40 patients who underwent temporal lobectomy for epilepsy. Hippocampal sclerosis was associated with febrile convulsions, an earlier onset of regular seizures, poorer preoperative intelligence and with a tendency towards greater cognitive improvement across the operation than found in patients with tumour-like malformations or non-specific pathology. Damage to the amygdala was associated with a poorer outcome for the retention in memory of verbal and non-verbal material. The absence of any specific abnormality in the resected tissue was not associated with a poorer cognitive outcome 4 weeks after the operation.

Adult↗
PubMed1987

Early-onset Huntington's chorea. Diagnostic clues.

A patient with early-onset Huntington's chorea is described. The diagnosis had been overlooked during previous contacts with psychiatric services because there was no family history, and features typical of the adult disease were absent. Distinctive findings on CT scan and electroencephalography were observed.

Adult↗
PubMed1985

Luria's frontal lobe syndrome: psychological and anatomical considerations.

Luria has described a syndrome of disinhibited and impulsive behaviour, in which the patient is unable to follow sequential instructions. This he attributes to localised frontal lobe damage. However, group studies of patients with focal lesions of the frontal lobes fail to reveal such a syndrome. A patient is described who displayed a form of Luria's syndrome temporarily. Psychometric and post mortem evidence indicate that the syndrome arises only under conditions of more global cerebral dysfunction.

Brain↗
PubMed1984

Trisomy 5p: a second case occurring in a previously described kindred.

A second child with trisomy 5p has been born in the kindred reported by Brimble-combe et al. The cytogenetic findings were similar to those of the index case except that the derivation was paternal instead of maternal. Improved banding techniques enabled more accurate designation of the breakpoints. The clinical and necropsy findings are described. Three non-specific phenotypic malformations and one brain abnormality, possibly specific, were common to both.

Abnormalities, Multiple↗
PubMed1981

Zinc and dementia.

Explore the source record for details and available documents.

Alzheimer Disease↗
PubMed1981

Dementia, deep white matter damage and hypertension: 'Binswanger's disease'.

The clinical and neuropathological features are reported of 7 patients with organic intellectual impairment or dementia, hypertension and ischaemic destruction predominantly of the deep white matter of the cerebral hemispheres resembling that seen in infarction. The white matter changes have been dismissed as rare n the past, usually under the name of Binswanger's disease or subcortical arteriosclerotic encephalopathy, and without much concern for hypertension. There are now indications that this sort of case may not be uncommon. It can be suspected in life on CT scans. The accuracy of assessment of the pathological substrate of organic dementia, and therefore the strategies of research and treatment, might well benefit from further clinicopathological studies.

Aged↗
PubMed1979

Neuroaxonal dystrophy in the neonate. A case report.

The nervous system of a female infant who was hypotonic from birth and survived for 8 days was examined. Neuropathological and electron microscopic study revealed numerous neuroaxonal swellings, so-called spheroids, in the brainstem and extensive necrosis in the cerebral hemispheres and cerebellum.

Autopsy↗
PubMed1979

Widespread intranuclear neuronal corpuscles (Marinesco bodies) associated with a familial spinal degeneration with cranial and peripheral nerve involvement.

Widespread intranuclear, paranucleolar, acidophilic corpuscles, known as Marinesco bodies, were found in association with degeneration of the posterior columns of ting at the age of 45 years. Neurological examination of his sister (aged 51 years) inciates that she is suffering from the same diseases. The cases appear to belong to the predominantly spinal form of spino-cerebellar degenerations or Friedreich's ataxia. This report, which includes an electron microscope examination, considers the pathological significance of Marinesco bodies.

Adult↗