Cases reported "Nerve Degeneration"

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1/11. Degeneration of the inferior olive in spinocerebellar ataxia 6 may depend on disease duration: report of two autopsy cases and statistical analysis of autopsy cases reported to date.

    This report concerns a clinicopathological study of two autopsied patients with spinocerebellar ataxia 6 (SCA6), and a statistical analysis between neuronal loss of the inferior olive and disease duration of 15 SCA6 autopsy cases reported to date, including the two cases reported in this study. Cases 1 and 2 came from independent Japanese families. Case 1 developed gait disturbance at age 35 years and died at age 78 years; she had a CAG-repeat expansion of the SCA6 gene (25/13). Case 2 presented with gait disturbance at age 68 years and died at age 78 years; he had an expanded CAG-repeat of the SCA6 gene (22/13). Neuropathological examination of both cases disclosed not only neuronal loss of the purkinje cells and inferior olive, but also some unnoticed features, including cactus-like expansion of the dendrite of purkinje cells and relative preservation of Golgi cells in the granular layer of the cerebellum. Exploratory statistical analysis between 11 SCA6 autopsy cases with neuronal loss in the inferior olive (average disease duration: 27 years) and four SCA6 autopsy cases without neuronal loss in the olive (average disease duration: 14.5 years) was investigated by Kaplan-Meier estimates of survival and log-rank test, retrospectively. Kaplan-Meier estimates of survival revealed an obvious difference between the two groups. survival of 10 years after the disease onset was 90.9% in the former 11 SCA6 autopsy cases, but was 50% in the latter four SCA6 autopsy cases. Furthermore, a log-rank test on the two groups disclosed a significant difference (P=0.0450). We postulate that the neuronal loss of the inferior olive in SCA6 may depend on disease duration.
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2/11. Progressive bulbar dysfunction caused by a predominantly venous vascular malformation of the medulla oblongata.

    We describe a 58-year-old patient with a rare predominantly venous vascular malformation of the medulla oblongata, which caused a progressive bulbar dysfunction consisting of hiccoughs, dysphagia, hoarseness, dysarthria, gait ataxia and dysuria over a period of 11 months. On autopsy, a large dilated vein with focal marked intimal fibroelastic thickening was present on the ventral surface of the medulla. Microscopically, moderate proliferation of capillaries and veins was observed which was confined primarily to the medulla. The veins displayed abnormal dilatation and tortuosity; prominent thickening of vessel walls was also present in the veins and capillaries. The venous abnormalities were prominent in the parenchyma of the medulla, but much less apparent in its subarachnoid space. Multifocal neuronal loss and gliosis were observed, most prominently in the inferior olives, hypoglossal, dorsal vagal and ambiguus nuclei. The histopathologic findings suggested that abnormal venous drainage within the parenchyma of the medulla was the most critical factor for the pathogenesis of this patient's neurologic symptomatology.
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3/11. 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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4/11. Neuropathologic findings in progressive supranuclear palsy. A brief review with two additional case reports.

    Recent studies utilizing electron microscopy and immunohistochemistry have added to the understanding of the pathology of progressive supranuclear palsy. This article reviews the neuropathologic findings of progressive supranuclear palsy and includes two new case reports. Findings in the patients reported here include extensive neuronal loss with reactive gliosis in deep subcortical gray matter such as globus pallidus, subthalamic nuclei, substantia nigra, red nucleus, dentate nucleus, and inferior olives. neurofibrillary tangles were also noted in thalamus, hypothalamus, striatum, nucleus basalis, locus ceruleus, basis pontis nuclei, midbrain, pontine, and medullary reticular formation as well as in various cranial nerve nuclei.
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5/11. Clinical and neuropathological aspects of long-term damage to the central nervous system after lithium medication.

    A female patient, who died at the age of 61 and had suffered from several manic-depressive psychoses for more than 30 years, developed three phases of intoxication under lithium therapy. There was a 15-year history of electro- and Pentetrazol-induced convulsive therapy prior to lithium medication; neuroleptics were still administered during lithium therapy. The last lithium intoxication, 3 years prior to death was during a low-dosage therapy with normal lithium levels followed by severe lasting impairment: akinesia, rigidity, dysarthria, ataxia, and an organic alteration in character. For the first time, neuropathological findings could be established in such a case: extensive damage to granule and purkinje cells in the cerebellum; gliosis in the dentate nucleus, the inferior olives, and the nucleus ruber; cytoplasmic inclusions in various nerve cells of the cranial nerve nuclei; cytoplasmic vacuoles, especially in the cells of the supra-optic nucleus. Surprisingly little damage could be found in the substantia nigra and in the neostriatum. The clinical course as well as the pattern and intensity of the brain damage oppose an interpretation as a consequence of preceding convulsive shock therapy.
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6/11. Autosomal recessive spino-olivo-cerebellar degeneration without ataxia.

    Five adult siblings from a sibship of ten suffering from an external ophthalmoplegia with a spastic paraplegia are reported. In addition, optic nerve atrophy was present in three of the patients and dementia in two; extrapyramidal signs and cerebellar ataxia were found only in one patient. Contrary to earlier studies of patients with comparable neurological signs the pattern of inheritance was autosomal recessive. Neuropathological investigation of the index case, who had never shown ataxia, nevertheless showed demyelination of the spinocerebellar and the olivocerebellar pathways, and also a severe loss of purkinje cells, of cells in Clarke's column and in the inferior olives. The dentate nucleus was severely gliotic but showed no cell loss. Earlier neuropathological investigations of this disorder, but with an autosomal dominant heredity, were incomplete. It is concluded that the five siblings of this family have a unique autosomal recessive disorder, which should be considered a distinct entity.
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7/11. Thalamic degeneration.

    A man of 61 died after an illness of 20 years duration. The clinical picture displayed progressive dementia, loss of memory, apathy, inertia, emotional lability, temporal and local disorientation, visual hallucination and apraxia. Neuropathological findings predominated in the thalamus and displayed extensive nerve cell loss and gliosis in the anterior, medial, and posterior formations, whereas the lateral formations were somewhat better preserved. The lateral amygdala were severely affected. The cortex showed widespread moderate damage in the third or fourth layer which are interpreted as transneuronal degeneration. In addition moderate atrophy of the vestibular nuclei, the inferior olives and the cerebellum was noted. This case seems to be one of the very rare examples of isolated thalamic degeneration according to Martin (1975).
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8/11. Joseph disease: a multisystem degenerative disorder of the nervous system.

    We studied Joseph disease clinically and pathologically in two patients of Portuguese ancestry, but from different families. We found involvement of spinocerebellar tracts, Clarke's column, anterior horn cells, motor cranial nerve nuclei, and substantia nigra. One patient also had pallidosubthalamic and pontocerebellar degeneration with normal inferior olives. The second patient, a Joseph family member, had nerve cell loss in the subthalamic nucleus. The neostriatum appeared normal in both cases. The pigmented nuclei contained a few lewy bodies. The almost identical pathology in two families support the hypothesis that Joseph disease is a genetic entity.
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9/11. Thalamo-olivary degeneration in a patient with laryngopharyngeal dystonia.

    A 67 year old woman with a two year history of laryngopharyngeal dystonia, spasmodic dysphonia, and parkinsonism succumbed to Wernicke's encephalopathy and died six months later. Necropsy showed, besides Wernicke's encephalopathy, degenerative changes in selected thalamic nuclei (dorsomedial, pulvinar, and the medial geniculate bodies) and the inferior olives and numerous cerebellar torpedoes. The substantia nigra and basal ganglia were spared. Immunostaining for prion protein was negative. This patient indicated a new type of presentation of so-called pure thalamic degeneration, or more precisely thalamo-olivary degeneration.
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10/11. Neuronal degeneration in subacute necrotizing encephalomyelopathy (Leigh's disease). Case report.

    We report clinical, radiological and pathological findings in a 5-year-old girl who died of subacute necrotizing encephalomyelopathy (SNE) after 4 weeks of illness. autopsy revealed endothelial swelling and vacuolar degeneration of the neuropil in the brain, brain stem and cerebellum. In addition, the affected areas showed degeneration of the neurons which was different from anoxic nerve cell damage both with regard to morphological picture and topographical distribution. This neuronal degeneration was probably due to the underlying metabolic defect in SNE per se and resembled in several aspects the nerve cell changes seen in the thalami and inferior olives in active Wernicke's encephalopathy. It is our opinion that more attention should be paid to the nerve cell degeneration in SNE rather than focusing on the relative preservation of these cells.
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