Cases reported "Nerve Degeneration"

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11/45. Are vestibular sensory cells preserved after destruction of Scarpa's ganglion? A study based on metastatic tumors of temporal bone.

    OBJECTIVE: The contribution of nerve fibers to the maintenance of vestibular sensory cells is a controversial issue in previous studies using animals and has not yet been studied in humans. The authors investigated this issue by observing vestibular end organs in the temporal bone of three patients in whom the internal auditory canal was infiltrated with tumor cells, and Scarpa's ganglion cells showed complete degeneration. STUDY DESIGN: Retrospective case review. SETTING: University Hospital, Department of otolaryngology. patients: Three patients with malignant metastatic temporal bone tumors. INTERVENTION: We investigated the preservative state of vestibular sensory hair cells with the Scalpa's ganglion was destructed. MAIN OUTCOME MEASURES: Maintenances of vestibular sensory hair cells. RESULTS: We found that sensory cells were intact despite the severe destruction of Scarpa's ganglion cells in two of the patients. CONCLUSION: The findings suggest that human vestibular sensory cells can be maintained for an indefinite period after denervation.
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12/45. Neuronal intranuclear hyaline inclusion disease: report of a case and review of the literature.

    A case of neuronal intranuclear hyaline inclusion disease (NIHID) is described. The patient was a 26-year-old man who died of a progressive neurologic disorder, the onset of which occurred at the age of 11 years. Clinically, the disease presented as juvenile parkinsonism, and pathologically it was characterized by multiple-system degeneration in conjunction with the ubiquitous presence of intranuclear hyaline inclusions in neurons of the central and peripheral nervous system including the autonomic ganglia. Smaller and less eosinophilic intranuclear inclusions were also present in a small number of glial cells. The neuronal inclusions emitted a strong yellow-green autofluorescence under ultraviolet light and were composed of filaments 10-15 nm in diameter. The glial inclusions also consisted of similar filaments but their autofluorescence could not be determined with certainty because of their small size and background autofluorescence. A review of the literature revealed 19 similar autopsy cases up to 1987. Since the clinical presentation and distribution of neuronal loss as well as the characteristics of the inclusions showed some differences among the cases, some authors speculated that NIHID represented more than one variant of a multiple-system degenerative disease. However, about half of the reported cases had favorable sites of neurodegeneration, such as the pallidum, substantia nigra, motor nuclei of the brain stem, anterior horn cells, Clarke's column and spinal ganglion as well as similarities among the inclusions. Thus, there seems to be a discrete group among cases of NIHID.
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13/45. The metabolic landscape of cortico-basal ganglionic degeneration: regional asymmetries studied with positron emission tomography.

    Regional metabolic rate for glucose (rCMRGlc) was estimated using [18F]fluorodeoxyglucose (FDG) and positron emission tomography (PET) in five patients (four men, one woman; mean age 68; mean disease duration 2.4 years) with clinical findings consistent with the syndrome of cortico-basal ganglionic degeneration (CBGD). Left-right rCMRGlc asymmetry, (L-R)/(L R) x 100, was calculated for 13 grey matter regions and compared with regional metabolic data from 18 normal volunteers and nine patients with asymmetrical Parkinson's disease (PD). In the CBGD group mean metabolic asymmetry values in the thalamus, inferior parietal lobule and hippocampus were greater than those measured in normal control subjects and patients with asymmetrical PD (p less than 0.02). parietal lobe asymmetry of 5% or more was evident in all CBGD patients, whereas in PD patients and normal controls, all regional asymmetry measures were less than 5% in absolute value. Measures of frontal, parietal and hemispheric metabolic asymmetry were found to be positively correlated with asymmetries in thalamic rCMRGlc (p less than 0.05). The presence of cortico-thalamic metabolic asymmetry is consistent with the focal neuropathological changes reported in CBGD brains. Our findings suggest that metabolic asymmetries detected with FDG/PET may support a diagnosis of CBGD in life.
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14/45. Histological changes of vestibular nerves and ganglion cells in the acoustic tumor in relation to clinical functions.

    The superior vestibular nerves and vestibular ganglion cells were observed electron microscopically in acoustic tumors removed surgically from 15 patients. Furthermore, the sensory cells of the vestibular organs were examined. Histological changes of vestibular nerves and ganglion cells were compared with the caloric responses of the patients. In most cases, the degree of degeneration of myelinated nerve fibers was proportional to the reduced responses of vestibular nerves. The myelinated nerve fibers decreased in number and showed some features of demyelination. The alteration of vestibular ganglion cells was unremarkable in comparison with vestibular nerves. In some cases, the number of lipofuscin granules, vacuolar organelles and amorphous materials markedly increased in the ganglion cells. However, the sensory cells of the vestibular organs appeared normal.
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15/45. Axonal degeneration of the peripheral nerves and postganglionic anhidrosis in a patient with multiple sclerosis.

    A 36-year-old woman had, since the age of 24, numerous episodes of visual loss and spinal symptoms and signs at various levels, and was diagnosed as multiple sclerosis(MS). CSF myelin-basic-protein was increased. Neurological and electrophysiological investigations suggested the peripheral nerve involvement. sural nerve biopsy performed about six years after the onset, revealed severe loss of both myelinated and unmyelinated fibers. Subsequently, histamine skin reaction was defective in the lower limbs. Tests on sudomotor and pupillary functions indicated deficits of both central and postganglionic sympathetic systems. Though we could not detect causative factors for the peripheral nerve lesions, our patient appears to be the first documented case of MS associated with axonal degeneration of the peripheral somatic and autonomic nervous systems.
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16/45. Axonal polyneuropathy without vasculitis, follicular lymphoma and primitive sicca syndrome. A rare association.

    A woman suffering from primary sjogren's syndrome developed sensitive neuropathy. The disease was further complicated by follicular lymphoma, an unusual albeit already described complication of Sjogren's disease. The clinical data suggest that mononuclear infiltration of the dorsal root ganglions could be at the origin of a sensitive neuropathy, inducing an axonal degeneration of the nerves.
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keywords = ganglion
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17/45. Posterior interosseous nerve axonotmesis from compression by a ganglion.

    A complete posterior interosseous nerve palsy resulted from axonotmesis caused by pressure from a ganglion cyst. Excision of the neuroma and interpositional nerve grafting gave a most satisfactory outcome.
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keywords = ganglion
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18/45. Degeneration of cochlear neurons as seen in the spiral ganglion of man.

    A light and electron microscopic study of the spiral ganglion cells of a normal individual, a patient with Meniere's syndrome, and a patient with long-standing unilateral profound deafness was done to evaluate patterns of neural degeneration in the human inner ear. Parametric data for the normal spiral ganglion are presented and compared with the pathologic ganglia. In the ear with Meniere's syndrome, the nuclear area and axonic diameter of spiral ganglion cells were significantly smaller than in the contralateral and the normal ear. This was interpreted as evidence of neuronal degeneration in Meniere's syndrome. In the spiral ganglion of the long-standing deaf ear, there appeared to be selective preservation of large cells with no dendritic processes, contrary to the pattern of degeneration seen in the spiral ganglion of the animal.
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ranking = 1.2857142857143
keywords = ganglion
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19/45. Sensory and autonomic polyneuropathy associated with hypergammaglobulinemia.

    Sensory and autonomic polyneuropathy is a rare disease characterized by a sensory nerve disorder and postganglionic autonomic dysfunction. The etiology of this disease is unknown. We described a 51-year-old woman who had a chronic sensory dominant polyneuropathy and dysautonomia associated with hypergammaglobulinemia. In the previous reports of sensory and autonomic polyneuropathy, not much attention was given to coexisting hypergammaglobulinemia. By reviewing the literatures, hypergammaglobulinemia was frequently present in these case reports. This fact leads us to consider that an immunological mechanism may be playing a role in the pathogenesis of this disorder.
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keywords = ganglion
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20/45. Hypothalamic gangliocytoma. Selective appearance of neurofibrillary changes, granulovacuolar degeneration, and argentophilic bodies.

    A hamartomatous gangliocytoma was observed in the hypothalamus of a 54-year-old woman. The ganglion cells were atypical, highly pleomorphic and often multinucleated, and they possessed neurofibrillary changes, granulovacuolar degeneration, and argentophilic bodies. The neuronal changes were highly selective and were not found in other parts of the brain. The tumor was also characterized by the presence of angiomatous blood vessels having such degenerative changes as fibrosis and thrombosis. The angiomatous blood vessels were found only in the lesion. The ultrastructural features of the neurofibrillary changes were similar to those observed in Alzheimer's disease. Vascular alterations have been suggested to be a possible contributor to the morphogenesis of neurofibrillary changes. In this case the exact etiology of these neuronal changes remains unclear, however, the possibility of a regional vascular role is considered with respect to their morphogenesis.
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