Cases reported "Atrophy"

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1/8. Agenesis of the cerebellar vermis: otoneurological report in one patient.

    We have performed otoneurological examination in a case of vermis cerebelli agenesia, CT scan confirmed. Visuo-vestibular interaction (VST) abnormality, with normal saccades and normal smooth pursuit movements, allows to suppose normal functioning of the flocculus and a damage in the nucleus olivaris inferior and, particular, in the paraolive.
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2/8. Amyotrophic cerebellar hypoplasia: a specific form of infantile spinal atrophy.

    Lower motor neuron degeneration, cerebellar hypoplasia, atrophy of pons, olives, and cerebellum, sclerosis of thalamus and pallidum, and deficient myelination were found in a 2-months-old baby with laryngeal paralysis, mental retardation, progressive amyotrophy, and slow nerve conduction velocity. Such changes seem characteristic of an unusual syndrome previously referred to as "cerebellar hypoplasia in Werdnig-Hoffmann disease", or "anterior horn cell disease with pontocerebellar hypoplasia". Although the pathologic changes in lower motor neurons are indistinguishable from those in other cases of infantile spinal muscular atrophy, the consistent reproducibility of a complex pathologic pattern suggests that this is probably a manifestation of a separate disease process. The term "amyotrophic cerebellar hypoplasia" (ACH) is a convenient designation for the syndrome.
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3/8. Hypoplasia of hemispheric white matter, peculiar pallidal changes and dysplastic inferior olives in a child with psychomotor retardation.

    A 25-month-old child with nonprogressive psychomotor retardation is described. Symmetrical hypoplasia of hemispheric white matter with an apparently intact cortex was a major finding. Maternal trauma to abdomen at 25 weeks of gestation was probably implicated in precipitating the condition. Also, changes resembling hypertrophy of the inferior olives were found in the pallida and were associated with dysplasia of the inferior olivary nuclei. There were other findings to suggest that the pallidal and olivary changes were secondary to deafferentation.
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4/8. 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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5/8. Localized proton magnetic resonance spectroscopy of cerebral abnormalities in children with carbohydrate-deficient glycoprotein syndrome.

    Morphologic and metabolic abnormalities in six children aged 2-9 years with carbohydrate-deficient glycoprotein (CDG) syndrome were assessed by magnetic resonance imaging (MRI) and localized proton magnetic resonance spectroscopy (MRS). In all patients, MRI revealed pronounced cerebellar atrophy. Follow-up examinations in two patients suggested early onset and rapid progression in the first years of life. Further pathologies comprised Dandy-Walker malformation, atrophy of the pons, brain stem and olives, supratentorial frontotemporal cortical atrophy, slightly dilated ventricles and a small corpus callosum. Two patients presented with small cysts in the white matter. The prominent metabolic abnormality detected by proton MRS in five patients was a reduction in N-acetylaspartate in white matter by more than 20%, indicating loss of vital neuroaxonal tissue. Further findings in white matter were glutamine and gamma-aminobutyrate increases by a factor of 2. One patient with type III CDG syndrome showed the most severe alterations of metabolite concentrations.
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6/8. Neuropathology of late cortical cerebellar atrophy in japan: distribution of cerebellar change on an autopsy case and review of Japanese cases.

    We report a Japanese autopsy case of late cortical cerebellar atrophy. The patient had showed clinically transient remission during thyrotropin-releasing hormone therapy. Our case suggests that thyrotropin-releasing hormone therapy is worth trying as a treatment of late cortical cerebellar atrophy. Neuropathological examination showed diffuse cerebellar cortical lesions and absence of neuronal loss in the dorsomedial part of the inferior olives. We studied qualitatively the detailed distribution of the cerebellar cortical lesions in 6 sections of the right cerebellum. The cerebellar lesions were more conspicuous in the most lateral hemisphere than in the vermis. We also reviewed 7 Japanese autopsy cases of late cortical cerebellar atrophy.
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7/8. Ponto-cerebellar hypoplasia with dystonia: clinico-pathological findings in a sporadic case.

    microcephaly, absent psychomotor development and dystonic limb movements were the main clinical features of a 3-year-old girl affected by hypoplasia of the pontocerebellar structures. As in the few previously reported cases there are discrepancies between the severity of lesions in the supratentorial and infratentorial compartments. Pathological features such as size reduction of the ventral pons, inferior olive atrophy, dentate nucleus fragmentation, and thinning of the cerebellar cortex suggest an impaired maturation of the involved structures due to a prenatal condition (dated at about 20-28 weeks of gestation). Somatotopic analysis failed to provide conclusive evidence on the primary target of the disease. The affected structures originate from the dorsal rhombencephalic region at about the same gestational age, and their maturation is probably under the control of sets of genes which regulate pattern formation. Early abnormal functioning of such genes might lead to the selected morphogenetical alterations observed in ponto-cerebellar hypoplasia. The normal morphogenetic pattern of the supratentorial structures and the mild lesions observed suggest that their late involvement can be related to a different pathogenetic process.
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8/8. A clinical, genetic, neuropathological study in a Japanese family with SCA 6 and a review of Japanese autopsy cases of autosomal dominant cortical cerebellar atrophy.

    This report concerns a Japanese family with genetically confirmed SCA 6, including an autopsy case, and a review of Japanese autopsy cases of autosomal dominant cortical cerebellar atrophy (ADCCA). The proband (Case 1) was a Japanese woman. She developed gait disturbance at age 62. The father and younger sister (Case 2) had the same disorder. She died at age 67 due to subarachnoid hemorrhage. Neuropathological examination revealed severe loss of purkinje cells in the cerebellum, prominently in the dorsal vermis, and absence of neuronal loss in the inferior olives. Molecular genetic study showed the CAG-repeat expansion of SCA 6 gene. The younger sister (Case 2) developed gait disturbance at age 62. Neurological examination at age 66 revealed cerebellar signs without sensory disturbance. neuroimaging at this time showed cerebellar atrophy, prominently in the vermis. She died of multiple myeloma at age 66. A neuropathological review of Japanese autopsy cases of ADCCA showed that there are two patterns in the distribution of cerebellar cortical lesions of Japanese patients with ADCCA. The distribution of cerebellar cortical lesions in genetically confirmed Japanese patients with SCA 6 is more prominent in the vermis than in the hemisphere.
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