Cases reported "Nerve Degeneration"

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1/33. guillain-barre syndrome occurring in two women after ketoacidosic comatose state disclosing an insulin-dependent diabetes mellitus.

    We report two women who presented with a guillain-barre syndrome just after a ketoacidosic comatose state disclosing an insulin-dependent diabetes mellitus. One had characteristic clinical signs and the other had major motor involvement. At neurophysiologic investigations, one had typical demyelinating neuropathy whereas the second had mainly axonal degeneration. At ultrastructural examination of a peripheral nerve biopsy, features of macrophage-associated demyelination were present in both nerve specimens, thus confirming the diagnosis of acute inflammatory demyelinating polyneuropathy, i.e., guillain-barre syndrome. Prominent axonal involvement was also present in the motor nerves of the second patient. insulin therapy had to be permanently continued and these two cases are quite different from the transient diabetes sometimes observed in certain cases of guillain-barre syndrome. Both the latter and insulin-dependent diabetes mellitus probably have auto-immune mechanisms. It is likely that in our two patients both auto-immune diseases were triggered by a common event. Such cases of guillain-barre syndrome have to be distinguished from other acute diabetic neuropathies.
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2/33. Is cochlear implantation possible after acoustic tumor removal?

    HYPOTHESIS: This study aimed to assess the possibility of hearing restoration after acoustic tumor removal. BACKGROUND: Hearing restoration surgery may be indicated after surgery of bilateral acoustic tumors or surgery of acoustic tumor in the only hearing ear. The choice is between cochlear implantation and brainstem implantation. methods: From the temporal bone collection at the House Ear Institute, los angeles, the author histologically examined eight temporal bones from seven patients who had undergone acoustic tumor removal during their lifetime. Special emphasis was put on examining the patency of the cochlear turns and on survival of the spiral ganglion cells and cochlear nerve. RESULTS: This study showed that after translabyrinthine acoustic tumor removal, there is progressive osteoneogenesis of the cochlea associated with almost complete degeneration of the spiral ganglion cells and cochlear nerve. Similar findings were noticed after middle fossa removal of acoustic tumor with unsuccessful hearing preservation. CONCLUSIONS: The histologic changes described in the cochlea and cochlear nerve represent the effects of ischemia resulting from inadvertent cutting of the blood supply during acoustic tumor removal. It is possible to do cochlear implantation after acoustic tumor removal provided that the result of promontory electrical stimulation test is positive (the cochlear nerve is intact) and that implantation is done at the time of acoustic tumor removal or shortly thereafter, before cochlear ossification is complete.
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3/33. temporal bone histopathologic and genetic studies in Mohr-Tranebjaerg syndrome (DFN-1).

    OBJECTIVE: To describe the temporal bone histopathologic and genetic abnormalities in a case of Mohr-Tranebjaerg syndrome. BACKGROUND: Mohr-Tranebjaezrg syndrome (DFN-1) is an X-linked, recessive, syndromic hearing loss, characterized by postlingual sensorineural hearing loss with onset in childhood, followed in adult life by progressive dystonia, spasticity, dysphagia, and optic atrophy. The syndrome is caused by mutations in the DDP (deafness/dystonia peptide) gene, which are thought to result in mitochondrial dysfunction with subsequent neurodegeneration. The temporal bone pathologic changes in this syndrome have not been reported. methods: hearing loss developed in the patient at age 4, blindness at age 48, and dystonia at age 57. Genetic studies on peripheral blood showed a l51delT mutation in his DDP gene. He died at age 66. The right temporal bone was subjected to light microscopy and polymerase chain reaction-based analysis of the DDP gene sequence. RESULTS: There was near complete loss of spiral ganglion cells with loss of nearly all peripheral and central processes. Only 1,765 spiral ganglion cells remained (8.5% of mean normal for age). The organ of corti (including hair cells), stria vascularis, and spiral ligament were preserved. There was also a severe loss of Scarpa's ganglion cells with preservation of vestibular hair cells. The population of geniculate and trigeminal ganglion cells appeared normal. sequence analysis from temporal bone dna showed the 15ldelT DDP gene mutation. CONCLUSION: Sensorineural hearing loss in Mohr-Tranebjaerg syndrome is the result of a postnatal, progressive, severe auditory neuropathy.
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4/33. Chronic inflammatory polyradiculoneuropathy.

    The diagnostic criteria, natural history, nerve conduction characteristics, pathology, laboratory features, and efficacy of corticosteroid treatment have been evaluated personally in 53 patients with chronic inflammatory polyradiculoneuropathy (CIP) who were followed up for an average of about 7.5 years. These were patients whose monophasic neurologic deficit had not crested by 6 months, patients with recurrences, and patients with a steady or stepwise progression. The typical features of CIP include absence of an associated disease, frequent history of preceding infection or receipt of foreign protein, and tendency to involve cranial, truncal, and proximal as well as distal limb structures and to have diffusely slow conduction velocity of peripheral nerves. The most marked slowing is often very proximal. The pathologic features include serous edema, mononuclear cell infiltrates (especially in perivascular areas, but without evidence of vasculitis), macrophage-induced segmental demyelination, and hypertrophic neuritis. If our patients are representative, complete recovery occurs only infrequently; about 60% of patients are able to be ambulatory and work, 25% become confined to a wheelchair or become bedridden, and approximately 10% die from their disease. Although the bulk of the pathologic changes affect spinal roots and proximal nerves, the brain and spinal cord may be involved also. Degeneration into linear rows of myelin ovoids is the predominant type of myelinated fiber degeneration of the sural nerve at the ankle.
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5/33. Histopathological observations of presbycusis.

    temporal bone histopathology of 17 aged patients who had spontaneous and gradually progressive bilateral sensorineural hearing losses associated with aging was studied. Six cases in the present material showed the gradually sloping audiometric curve; nine cases, abrupt high tone hearing loss; and two cases, the flat audiometric curve. The most prominent histopathological change in the inner ear was a decrease in the population of the spiral ganglion cells. However, diffuse senile atrophy was also often seen in the organ of corti and the stria vascularis. A positive correlation between the degree of artheriosclerosis and the degree of sensorineural degeneration in the cochlea was not obtained in the present cases. Also, the correlation was not found to be consistent between the type of the audiometric curve and the localization of lesions in the sensory, the neural or the vascular elements in the cochlea. Our observations show that a certain type of audiometric curve does not necessarily indicate a lesion in a specific cochlear element.
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6/33. Peripheral motor-sensory neuropathy in membranous lipodystrophy (Nasu's disease): a case report.

    A case of membranous lipodystrophy (ML) with peripheral polyneuropathy (PN) is described. A 40-year-old woman presented with dementia, multiple lytic bone lesions with typical membranous material in adipose tissues and neurophysiological and pathological findings of peripheral neuropathy. We describe the first case of ML with PN. It is interesting since nerve involvement might be an additional cause of symptoms usually attributed to bone lesions.
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7/33. Cochlear degeneration in leigh disease: histopathologic features.

    OBJECTIVE: To describe pathologic findings from temporal bones acquired from an infant with leigh disease. STUDY DESIGN: Retrospective case review. MATERIALS AND methods: Temporal bones were taken at autopsy from an 8-month-old infant with leigh disease. The right temporal bone was studied by microdissection. The middle ear was examined and the inner ear sensory organs dissected for study by light microscopy. The left temporal bone was embedded in celloidin, and sections were cut for microscopic examination. RESULTS: Middle ear structures were normal bilaterally. There was, however, evidence of otitis media in both middle ears, which was more severe on the left side. Inner and outer hair cell loss, patchy degeneration of organ of corti, and loss of nerve fibers in the osseous spiral lamina were found in the basal and middle turns of both cochleas. Basophilic deposits in the stria vascularis were observed in the apical portion of the left cochlea. CONCLUSIONS: Inner ear sensorineural degeneration may occur in leigh disease. Possible cochlear dysfunction caused by the degenerative changes needs to be considered in the hearing assessment of patients with leigh disease.
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8/33. pathology and pathophysiology of idiopathic sudden sensorineural hearing loss.

    BACKGROUND: The cause and pathogenesis of idiopathic sudden sensorineural hearing loss remain unknown. Proposed theories include vascular occlusion, membrane breaks, and viral cochleitis. AIMS: To describe the temporal bone histopathology in 17 ears (aged 45-94 yr) with idiopathic sudden sensorineural hearing loss in our temporal bone collection and to discuss the implications of the histopathologic findings with respect to the pathophysiology of idiopathic sudden sensorineural hearing loss. methods: Standard light microscopy using hematoxylin and eosin-stained sections was used to assess the otologic abnormalities. RESULTS: Hearing had recovered in two ears and no histologic correlates were found for the hearing loss in both ears. In the remaining 15 ears, the predominant abnormalities were as follows: 1) loss of hair cells and supporting cells of the organ of corti (with or without atrophy of the tectorial membrane, stria vascularis, spiral limbus, and cochlear neurons) (13 ears); 2) loss of the tectorial membrane, supporting cells, and stria vascularis (1 ear); and 3) loss of cochlear neurons only (1 ear). Evidence of a possible vascular cause for the idiopathic sudden sensorineural hearing loss was observed in only one ear. No membrane breaks were observed in any ear. Only 1 of the 17 temporal bones was acquired acutely during idiopathic sudden sensorineural hearing loss, and this ear did not demonstrate any leukocytic invasion, hypervascularity, or hemorrhage within the labyrinth, as might be expected with a viral cochleitis. DISCUSSION: The temporal bone findings do not support the concept of membrane breaks, perilymphatic fistulae, or vascular occlusion as common causes for idiopathic sudden sensorineural hearing loss. The finding in our one case acquired acutely during idiopathic sudden sensorineural hearing loss as well as other clinical and experimental observations do not strongly support the theory of viral cochleitis. CONCLUSION: We put forth the hypothesis that idiopathic sudden sensorineural hearing loss may be the result of pathologic activation of cellular stress pathways involving nuclear factor-kappaB within the cochlea.
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9/33. 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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10/33. Paraneoplastic degeneration of the substantia nigra with dystonia and parkinsonism.

    A 42-year-old woman suffered unexplained weight loss followed by action tremor and difficulty initiating gait. Three months after onset of symptoms, infiltrating ductal carcinoma of the breast, metastatic to liver and lymph nodes, was diagnosed and treated briefly with cyclophosphamide, methotrexate, and 5-flourouracil (5FU). Severe symmetric action and postural tremor with a myoclonic component developed, with minimal rest tremor, severe dysarthria and dysphagia, small-stepped and slightly ataxic gait progressing to a bedbound state, and severe widespread dystonic posturing. The latter began as a typical parkinsonian posture of trunk and upper extremities and progressed to a fixed and painful flexion of the elbows and wrists and extension of fingers and neck. Sinemet, anticholinergics, baclofen, diazepam, and plasmapheresis gave no benefit. The patient died of complications of immobility 5 months after neurologic symptom onset. autopsy revealed many pigment-laden macrophages in substantia nigra and moderate loss of pigmented neurons. inflammation, lewy bodies, and tumor were absent. Cerebellar purkinje cells were moderately depleted. Mild neuronal loss and gliosis were present in globus pallidus and cerebellar cortex. Stains for anti-human IgG, IgM, kappa, and lambda were negative. This, to our knowledge, is the first report of paraneoplastic degeneration of substantia nigra or paraneoplastic parkinsonism.
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