Biomedical subjects
Publications and source records attributed to S Pogacar.
We report a patient with an acute monophasic demyelinating disease leading to death 29 days after onset. Neuroimaging showed progressive white matter attenuation, and neuropathology was characteristic of multiple sclerosis. Death in acute MS of the Marburg type may result from severe disseminated demyelination or involvement of critical brainstem structures.
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This is a pathological analysis of a 50-year-old severely mentally retarded female with trisomy 21 who five years prior to her demise developed progressive dementia, epileptic seizures and choreiform movements. The necropsy revealed gross and microscopic features of Alzheimer's and Pick's disease. Ultrastructurally the majority of neurofibrillary changes and all studied Pick bodies consisted of 15 nm straight tubules. A few neurofibrillary changes were composed of 22 nm paired helical filaments, which were also found in the enlarged neurites of neuritic plaques. A few paired helical filaments were interspersed between straight tubules of Pick bodies. These findings in a patient with Down's syndrome and previous reports of atypical features of Alzheimer's disease indicate that very probably our case is a variant of Alzheimer's disease, thus broadening the spectrum of pathological changes observed in Alzheimer's disease.
In five generations of Anglo-Saxon family W, 28 members were affected with dominant hereditary ataxia. In 1969, two members of this family were reported as prototypes of dominant spinopontine atrophy. We discuss two additional members of the original family, with one postmortem examination, including ultrastructual study. In contrast to previously accepted clinical generalizations, we found abolished tendon reflexes and flexion contractures of the lower extremities in patient 1 and onset of illness at the age of 18 years, palatal myoclonus, and optic atrophy in patient 2. Dementia was observed in both patients. Unlike in previous pathological reports, our patient 1 showed definite involvement of the cerebellum and mild degeneration of the inferior olivary nuclei. We conclude, therefore, that clinical and pathological distinction between dominant spinopontine atrophy and olivopontocerebellar atrophy is not clear and raises the question of the justification for regarding dominant spinopontine atrophy as a nosological entity.
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