Cases reported "Nerve Degeneration"

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1/4. Intramedullary schwannoma of the spinal cord. A case report and review of the literature.

    A 9-year-old boy presented with the numbness in both arms and hands, and neck stiffness. On examination, he had a slight quadriparesis and restricted neck movements. There were no signs of von Recklinghausen's disease. magnetic resonance imaging (MRI) scan disclosed a gadolinium enhanced intramedullary tumor located at C6-T1 associated with syringomyelia. C6-T1 laminectomies were performed and the intramedullary tumor was totally removed by a microsurgical technique. Postoperative course was uneventful. The pathological examination revealed an intramedullary schwannoma. The occurrence of intramedullary schwannoma in a patient without signs of von Recklinghausen's disease is extremely rare. We have been able to find 57 cases of intramedullary schwannoma reported in the literature. Intramedullary schwannomas are usually seen in males. The ages of the patients ranged from 9 to 75 years (mean 40.44 years). Only 4 cases in the pediatric age group have been reported. The duration of symptoms ranged from 3 months to 20 years (mean 31.03 months). Symptoms and signs varied with the location of tumor. The vertebral levels of intramedullary schwannomas were usually cervical (61%). MRI has been the choice of diagnostic tool in the cases reported since 1986. The majority of the cases showed either a partial or complete recovery in the postoperative period.
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2/4. Facial neuropathy due to axonal degeneration and microvasculitis following gamma knife surgery for vestibular schwannoma: a histological analysis. Case report.

    Complete facial palsy (House-Brackmann Grade VI) developed in a 63-year-old man with a vestibular schwannoma 25 months after he had undergone two gamma knife surgeries performed 33 months apart and involving a cumulative dose of 24 Gy directed to the tumor margin at the 50% isodose line. magnetic resonance imaging demonstrated tumor enlargement with central nonenhancement, which initially had been recognized 21 months after the second radiosurgery. microsurgery was performed to achieve total removal of the tumor. Histological and immunohistochemical examinations of the facial nerve specimen removed from the edge of the tumor revealed a loss of axons, proliferation of schwann cells, and microvasculitis. In this case, microvasculitis and axonal degeneration were probably the major causes of the radiation-induced facial neuropathy.
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keywords = schwann
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3/4. Ocular neuromyotonia with spastic lid closure.

    Ocular neuromyotonia is characterized by tonic spasms of extraocular muscles evoked by eccentric gaze that induces transient strabismus and diplopia. We report the case of a 70-year-old woman who initially presented with unilateral deficits in fifth and sixth cranial nerve functions attributed to a fifth cranial nerve schwannoma. After radiation treatment, she developed neuromyotonia and synkinesis of the ipsilateral third cranial nerve. During the attacks of neuromyotonia, the left eyelids were often spastically closed, a phenomenon not previously reported. The ocular neuromyotonia regressed spontaneously within 3 years, but components of the synkinesis persisted. Ephaptic transmission in a damaged third cranial nerve may be responsible for the neuromyotonia and synkinesis. synkinesis is a more enduring manifestation.
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keywords = schwann
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4/4. Clinical and ultrastructural study of a sporadic case of hereditary sensory neuropathy. Morphological evidence for a slow rate fiber degeneration.

    A sporadic case of hereditary sensory neuropathy, with a clinical course and severe trophic and sensory alterations typical for type II, is presented. There was a severe loss of myelin in the sural nerve biopsy taken from the ankle. The most impressive microscopic feature was the number of rudimentary onion bulbs with an empty core which contained nude axons in the peripheral schwann cell layers; they were interpreted as vestigial structures left by the former myelinated fibers. Electron microscopy also revealed a definite involvement of unmyelinated fibers with attempted regeneration, which was confirmed by the overrepresentation of small axons on their frequency distribution curve. The whole ultrastructural picture suggested the protracted nature of the fiber involvement. This may be considered as agreeing with the slow course proposed for a system degeneration.
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keywords = schwann cell, schwann
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