Cases reported "Neuromyelitis Optica"

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1/47. Systemic lupus erythematosus with optical neuromyelitis (Devic's syndrome). A case with a 35-year follow-up.

    Optical neuromyelitis or Devic's syndrome is a very uncommon neurological manifestation of systemic lupus erythematosus. It is also associated with antiphospholipid antibodies, limited responsiveness to glucocorticoid treatment and a poor prognosis. We report the case of a female systemic lupus erythematosus patient who developed recurrent flares of optical neuritis and transverse myelitis. These flares consistently responded to glucocorticoid therapy. Despite the absence of overt anticardiolipin antibodies in the course of the disease, long-term anticoagulant therapy has been introduced with positive results. Treatments are usually of limited efficacy in Devic's syndrome. In our patient, however, aggressive glucocorticoid treatment resulted in prolonged survival.
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keywords = neuromyelitis, optica
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2/47. Devic's neuromyelitis optica during pregnancy in a patient with systemic lupus erythematosus.

    Neuropsychiatric forms of systemic lupus erythematosus (SLE) vary, most commonly consisting of seizures, psychiatric disturbances, or focal central nervous deficits. This is a new case of neuromyelitis optica or Devic's syndrome during the course of SLE. Few reports of this association exist in the literature. Our objective is to report this unique case of Devic's neuromyelitis optica during pregnancy in a patient with systemic lupus erythematosus. A 28-year-old woman had been diagnosed as having SLE with cutaneous and articular involvement in 1987 when she was 17 years old. She was treated with a synthetic antimalarial agent associated with corticosteroids. In 1994, during the fourth month of pregnancy, she had signs of transverse myelitis with a sensory level at T6 associated with an optic neuropathy suggesting a Devic's syndrome. The patient was managed by plasmapheresis sessions and intravenous corticosteroids. Transverse myelitis recurred postpartum and three years later at the same thoracic level. Management by bolus administration of a steroid and cyclophosphamide resulted in remission again. There have only been around a dozen reports in the literature of patients who had both Devic's neuromyelitis optica and SLE. magnetic resonance imaging is contributive to diagnosis and therapeutic follow-up, showing spinal cord lesions with increased intensity on T2-weighted sequences. Although the clinical course of the present patient has been favourable so far, the prognosis of this neurologic disease is generally considered to be poor with elevated mortality.
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ranking = 3.6504482377802
keywords = neuromyelitis optica, neuromyelitis, optica
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3/47. neuromyelitis optica following infectious mononucleosis.

    A case of moderatley severe neuromyelitis optica following infectious mononucleosis is described as the first reported instance of this complication. Total recovery occurred. Rapid improvement followed the commencement of corticosteroids. It is postulated that the pathological process was one of postinfectious demyelination.
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ranking = 0.6869151537312
keywords = neuromyelitis optica, neuromyelitis, optica
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4/47. neuromyelitis optica after a spinal anaesthesia with bupivacaine.

    We report the case of a 53-year old woman who developed a neuromyelitis optica (NMO or Devic's syndrome) after a spinal anaesthesia with bupivacaine. To our knowledge, the use of bupivacaine in human has not been associated to neurological complications like myelitis or NMO. The pathogenesis of this complication will be discussed.
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ranking = 0.6869151537312
keywords = neuromyelitis optica, neuromyelitis, optica
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5/47. neuromyelitis optica: importance of cerebrospinal fluid examination during relapse.

    Devic's neuromyelitis optica (NMO) is a clinical entity characterised by severe transverse myelitis, optic neuropathy and monophasic or recurrent course. We report the case of a woman affected by myelitis and optic neuritis suggesting Devic's disease. diagnosis was supported by clinical, neuroradiological and biochemical findings. In 14 months, the patient developed 5 clinical exacerbations. Six cerebrospinal fluid (CSF) examinations were performed, 3 during relapses and 3 during remitting phases: all the CSF specimens obtained during relapses showed granulocyte pleocytosis and increased protein level, whereas CSF was normal during stationary phases. Oligoclonal banding was always absent. spinal cord MRI showed altered signal at cervical and thoracic levels. We did not find any concomitant systemic disease. The case we report underlines the importance of CSF examination during clinical relapse in NMO diagnosis.
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ranking = 0.6869151537312
keywords = neuromyelitis optica, neuromyelitis, optica
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6/47. Devic disease and thymoma with anti-central nervous system and antithymus antibodies.

    A patient with myasthenia gravis and thymoma developed neuromyelitis optica (NMO) and necrotizing myositis 4 months after treatment of the tumor. antibodies reacting with the CNS and thymic epithelial cells were detected in the serum during the acute phase of NMO, suggesting that the NMO was linked to the thymoma.
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ranking = 0.52149260539717
keywords = neuromyelitis optica, neuromyelitis, optica
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7/47. Devic's neuromyelitis optica treated with intravenous gamma globulin (IVIG).

    BACKGROUND: Devic's syndrome is a demyelinating disease of the spinal cord and optic nerves. It tends to have a poor prognosis, probably due to the occurrence of necrosis within lesions. There is no proven effective treatment although relapses are commonly treated with corticosteroids and people with recurrent attacks may be managed with chronic immune suppressing treatments. Intravenous gamma globulin (IVIG) and plasma exchange are reasonable treatment options because Devic's syndrome is believed to be antibody mediated. We report two patients of Devic's syndrome that stabilized following initiation of monthly IVIG. PATIENT 1: A 42-year-old woman with a 23 year history of Devic's syndrome continued to have frequent attacks of optic neuritis unresponsive to daily corticosteroids and azathioprine. Since initiation of monthly IVIG 5 1/2 years ago she has had no further definite attacks. She has also noted minimal improvement in color perception. PATIENT 2: A 58-year-old woman with a three year history of Devic's syndrome experienced five attacks during the first 16 months of disease. Monthly IVIG was associated with complete cessation of relapses and significantly improved neurological status over one year of treatment. CONCLUSIONS: Because active Devic's disease often results in severe, permanent neurological impairment, preventive intervention should be considered. These cases suggest that IVIG may be effective in preventing attacks and possibly in enhancing neurological recovery. Randomized controlled trials will be needed to confirm this and to determine optimal dosing and treatment duration.
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ranking = 2.0859704215887
keywords = neuromyelitis optica, neuromyelitis, optica
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8/47. A central demyelinating disease with atypical features.

    There are clinical, laboratory and imaging criteria to distinguish multiple sclerosis (MS) from neuromyelitis optica (NMO) and acute disseminated encephalomyelitis (ADEM). While MS has unknown aetiology, NMO is commonly associated with vasculitis and ADEM is supposed to be parainfectious in origin. In the present study, six patients are described from a group of 67 with a central demyelinating disorder whose clinical presentation did not conform to existing diagnostic criteria for ADEM, NMO or MS. Their clinical, laboratory and imaging characteristics were studied and analysed. Some features suggested a particular diagnosis but some other features favoured another diagnosis. The features included spinal cord involvement in a large vertical segment with cord swelling, optic neuritis, no lesions in the cerebral cortex, paraplegia with urinary retention during the acute phase, no oligoclonal band in cerebrospinal fluid, absence of any evidence of vasculitis, wide time-gap between spinal cord and optic nerve involvement, good recovery from acute phase of disease and a relatively benign course. We conclude that there exists a subpopulation of patients with central demyelinating disease in this region with mixed clinical features. overall features suggested either a widened clinical spectrum of MS, NMO or ADEM or a possible overlap between them.
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ranking = 0.52149260539717
keywords = neuromyelitis optica, neuromyelitis, optica
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9/47. neuromyelitis optica in a child with atypical onset and severe outcome.

    We report on a seven-year-old boy with inflammatory relapsing-remitting CNS disease, involving the optic nerves and spinal cord, with increasingly severe sequelae after each relapse. Clinical course, neuroimaging and laboratory findings were consistent with neuromyelitis optica. biopsy of leptomeninges and underlying nervous tissue showed increased vascularization and thickened hyalinized vessel walls, reported as suggestive for neuromyelitis optica. Clinical features at onset were atypical, rendering the case highly unusual and the diagnosis tentative.
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ranking = 1.2084077591284
keywords = neuromyelitis optica, neuromyelitis, optica
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10/47. Recurrent neuromyelitis optica with diffuse central nervous system involvement: case report.

    Several demyelinating disorders can affect children. The differential diagnosis between these diseases is usually an arduous task. Diagnostic criteria have been proposed for some of these disorders, however most of them have not yet been clinically and prospectively validated. Here we present a case of a ten year-old boy with recurrent bilateral optic neuritis and spinal cord involvement. Clinical and cerebrospinal fluid data have fulfilled diagnostic criteria for Devic's neuromyelitis optica (NMO). The differential diagnosis with multiple sclerosis (MS) has become troublesome since not only optic nerves and spinal cord were involved. In one of the relapses a left hemiparesis with facial involvement was registered. magnetic resonance imaging was also compatible with MS. This case illustrates that CNS demyelinating disorders can fulfill diagnostic criteria for more than one demyelinating disease, making the clinical judgment an important tool in the management of these patients.
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ranking = 2.6074630269858
keywords = neuromyelitis optica, neuromyelitis, optica
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