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1/6. Degeneration of posterior column nucleus, inferior olivary nucleus and cerebellar cortex: system degeneration of paraneoplastic disease?

    Degeneration of the posterior column nucleus, inferior olive, and cerebellar cortex is reported in a cancer patient. A 70-year-old man developed an ataxic gait and rapidly progressive disturbance of deep sensation over six months, followed by an abasic state. Early well-differentiated tubular adenocarcinoma was detected and total gastrectomy was performed. He died due to pulmonary tuberculosis about two years and nine months after the operation. Degeneration of the posterior column nuclei was found, explaining the disturbance of deep sensation noted in the clinical course, although there were few changes in the peripheral nerves, dorsal root ganglia, and spinal cord. Degeneration of the inferior olive and cerebellar corte was also found. Metastatic small cell carcinoma was present in the right pulmonary hilar and paratracheal lymph nodes at autopsy without any detectable primary focus. There was neither recurrence nor metastasis of the gastric carcinoma. This is the first case report of this type of central nervous system degeneration in a cancer patient, and its pathogenesis and etiology remain obscure. We discuss whether system degeneration or paraneoplastic degeneration was the etiology.
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2/6. X-linked olivopontocerebellar atrophy.

    We present a kindred with a relatively pure cerebellar degeneration that demonstrates X-linked recessive inheritance. The unique clinical picture of affected patients in our kindred is characterized by an infantile onset of ataxia; very slow rate of progression; normal strength, reflexes, and sensation; and cerebellar degeneration with involvement of the olive and pons demonstrated by neuroimaging techniques. The distinction between this and other reported olivopontocerebellar degenerations is made on the basis of the clinical features and mode of inheritance. It is not clear if the distinct clinical pattern in this kindred represents variable expression of a previously reported condition, allelic variance of previously reported kindreds, or a separate clinical entity. Molecular analysis, currently underway, may help settle the issue.
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3/6. Neocerebellar hypoplasia with systemic combined olivo-ponto-dentatal degeneration in a 9-day-old baby: contribution to the problem of relations between malformation and systemic degeneration in early life.

    Three different cerebral alterations, apparently formed consecutively, were observed in a 9-day-old baby. Marked cortico-neocerebellar hypoplasia was seen in a relatively well-developed paleocortex. Its teratological stage was apparently the 3rd fetal month. Almost total nerve cell loss and marked proliferation of protoplasmic and fibrous astrocytes were found in the nuclei pontis and inferior olive. Perihypoglossal and pararaphal nuclei, which are related to the cerebellum, were also affected. This degenerative process must have resulted from neuronal deprivation or inactivity, as the pertinent cortico-cerebellar area was hypoplastic, and therefore any neuronal imput was impossible (olivo-ponto-dentatal degeneration due to cortico-cerebellar hypoplasia). Massive symmetrical necrosis in the cerebral white and grey matter, basal ganglia, midbrain and bulbus, is interpreted as hypoxic damage due to perinatal convulsive attacks and cessation of respiration.
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4/6. Infantile multiple system atrophy with cytoplasmic and intranuclear glioneuronal inclusions.

    This report presents a case of infantile multiple system atrophy with probably autosomal recessive inheritance. The female patient developed generalized muscular hypotonia, myoclonias and tonic-clonic seizures at the age of 8 months, followed by gradual development of choreoathetotic hyperkinesia and increasing psychomotor retardation. Metabolic disease was ruled out and the child died of aspiration pneumonia at the age of 5 years. General autopsy was unremarkable, but neuropathological examination showed degeneration of cerebellum, inferior olives, medial thalamus, Clarke's nucleus, anterior horn cells, corticospinal, spinocerebellar tracts, and posterior columns. Immunohistochemically many neurons contained intranuclear and intracytoplasmic ubiquitin-positive inclusions, which did not contain neurofilament or tau epitopes and ultra-structurally consisted of granulofilamentous material. We tentatively classify this case as a form of infantile multiple system atrophy linked to neuronal intranuclear hyaline inclusion disease.
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5/6. Olivopontocerebellar atrophy with retinal degeneration. Fundus characteristics and diagnostic MRI findings.

    Olivopontocerebellar atrophy with retinal degeneration (OPCA type III) and autosomal dominant cerebellar atrophy of late onset (type II) appear to represent the same disease which is characterized by dominantly inherited cerebellar ataxia and pigmentary retinal degeneration. In June, 1988, a 15-year-old girl presented with objective visual acuity loss but no other findings. When seen again in January, 1991, macular changes were noted. At this time, her mother, diagnosed with multiple sclerosis, was examined and found to have atrophic macular lesions and poor vision. A brain MRI was performed which showed findings diagnostic of OPCA type III, including cerebellar and pontine atrophy and specific loss of the inferior olives. Fundus features, electroretinographic results and other clinical findings in OPCA type III are discussed and diagnostic MRI lesions are demonstrated.
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6/6. Fatal infantile encephalopathy with olivopontocerebellar hypoplasia and micrencephaly. Report of three siblings.

    We report three siblings born with severe neonatal encephalopathy, manifested clinically by microcephaly, myoclonus, and muscular hypertonus. Karyotypic analyses and all biochemical investigations were unrevealing. All three patients died during infancy. Postmortem examination of the brain in one child disclosed severe neuronal loss in the inferior olives and the pontine nuclei. There was also severe hypoplasia of the cerebellum and micrencephaly. There was diffuse gliosis of the white matter in all areas of the brain. We believe this may represent a previously undescribed form of familial infantile encephalopathy with olivopontocerebellar hypoplasia.
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