1/33. 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.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/33. Progressive necrotic myelopathy: clinical course in 9 patients.OBJECTIVE: To review the clinical, laboratory, and radiological findings of 9 patients who had progressive idiopathic myelopathy with evidence of spinal cord necrosis. DESIGN AND methods: We reviewed personally examined cases of myelopathy that fulfilled the following criteria: (1) regional loss of reflexes, flaccidity, and muscle atrophy; (2) magnetic resonance imaging showing a shrunken or cavitated cord without evidence of arteriovenous malformation; (3) electromyogram showing denervation over several contiguous spinal cord sgements with preservation of sensory potentials in some cases; and (4) the absence of evidence of systemic disease or neoplasm. RESULTS: The illness began in these patients after the age of 40 years, with prominent burning or tingling limb pain, occasionally with radicular features or with less well-defined back, neck, or abdominal pain. Leg or infrequently arm weakness appeared concurrently or soon after the onset of pain. The most distinctive feature was a saltatory progression of symptoms, punctuated by both acute and subacute worsenings approximately every 3 to 9 months, culminating in paraplegia or tetraplegia. The distinguishing clinical findings, together indicative of destruction of gray matter elements of the cord, were limb atrophy, persistent areflexia, and flaccidity. The concentration of cerebrospinal fluid protein was typically elevated between 500 g/L and 1000 g/L, without oligoclonal bands, accompanied infrequently by pleocytosis. magnetic resonance imaging showed features suggesting cord necrosis, specifically swelling, T2-weighted hyperintensity, and gadolinium enhancement over several spinal cord segments, succeeded months later by atrophy in the same regions. necrosis of the cord was found in biopsy material from one patient and postmortem pathology in another case, but inflammation and blood vessel abnormalities were absent. Only 2 patients had prolonged visual evoked responses. The disease progressed despite immune-modulating treatments although several patients had brief epochs of limited improvement. CONCLUSIONS: The saltatory course, prolonged visual evoked responses in 2 patients, and a cranial abnormality on magnetic resonance imaging in another, raised the possibility of a link to multiple sclerosis. However, the normal cranial magnetic resonance imaging scans in 6 other patients, uniformly absent oligoclonal bands, and poor response to treatment were atypical for multiple sclerosis. On the basis of shared clinical and laboratory features, idiopathic progressive necrotic myelopathy is indistinguishable from a limited form of Devic disease.- - - - - - - - - - ranking = 4keywords = spinal (Clic here for more details about this article) |
3/33. 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.- - - - - - - - - - ranking = 5keywords = spinal (Clic here for more details about this article) |
4/33. 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.- - - - - - - - - - ranking = 5keywords = spinal (Clic here for more details about this article) |
5/33. 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.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
6/33. 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.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
7/33. 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.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
8/33. 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.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
9/33. Successful high dose glucocorticoid treatment for subacute neuromyelitis optica with systemic lupus erythematosus.A 54-year-old Japanese woman with a 6-year history of systemic lupus erythematosus (SLE) was admitted to our hospital suffering from acute blindness in her right eye. Her condition recovered after steroid pulse therapy, however, 18 months later she suffered from nuchal pain for 2 weeks after which right hemiparesis with urinary incontinence developed. A spinal magnetic resonance imaging (MRI) revealed cord swelling from C2 to C7. She was diagnosed with neuromyelitis optica (NMO) and intravenous steroid administrations were immediately commenced. Her condition promptly improved. This case was unique because the steroid treatment was quite effective for this case of myelitis, which had passed the acute phase. We supposed that, because most of the lesion was not necrotic or demyelinated, but rather showed edematous change caused by vasculitis based on autoimmune pathogenesis, the symptoms progressed rather gradually and improved promptly in response to glucocorticoid treatment.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
10/33. An autopsied case of neuromyelitis optica with a large cavitary cerebral lesion.We report a case of neuromyelitis optica (NMO) with a large cerebral lesion. The patient had an episode of fever and consciousness disturbance with a tumefactive frontal white matter lesion at age 43, and then repeated bilateral optic neuritis and transverse myelitis until she died at age 63. Histopathological examinations revealed that marked tissue destruction, cavities and inflammatory changes typical of NMO were seen in the cerebrum as well as the optic nerves and spinal cord. This is the first autopsied case of NMO with a tumefactive cerebral lesion that later became cavitary.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
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