1/19. magnetic resonance imaging in five patients with a tumefactive demyelinating lesion in the central nervous system.Five patients with a tumefactive lesion were clinically followed from 1992 to 1993. Four patients were female; age ranged from 32 to 57 years, the duration of symptoms varied from 3 days to 3 years. Neurological examination disclosed dementia in two patients, aphasia in three, hemiparesis in four, hemihypoaesthesia in three, optical neuritis in two, tetraparesis with sensitive level and neurogenic bladder in one. MRI disclosed lesions with a hypersignal on images assessed at T2 and hyposignal at T1, and gadolinium heterogeneous enhancement; these lesions were located in the: a) temporooccipital region bilaterally and brain stem, b) frontoparietal white matter, c) basal ganglia, bilateral white matter and brain stem, d) left parietal region, e) cervical spinal cord, with enlargement of this region. Cerebral biopsy was performed in three patients; acute and subacute demyelinating disease was diagnosed by histological examination. Two patients had an evolutive diagnosis; exclusion of other pathologies and clinical and radiological improvement after corticotherapy, pointed to an inflammatory disease.- - - - - - - - - - ranking = 1keywords = optica (Clic here for more details about this article) |
2/19. Demyelinating disorder of the central nervous system occurring in black South Africans.OBJECTIVES: To investigate the nature and cause in eight black South African patients of a recurrent (multiphasic), remitting, and relapsing demyelinating disease of the CNS. methods: The clinical and laboratory investigations and radiological manifestations of these patients were documented. RESULTS: Each patient had two or more acute attacks of demyelinating disease affecting the CNS. The clinical presentations of the patients were predominantly those of multiphasic neuromyelitis optica (NMO). Brain MRI in these patients showed features consistent with those described for acute disseminated encephalomyelitis (ADEM), as well as lesions that are described in multiple sclerosis. There was involvement of the corpus callosum in addition to typical ADEM lesions. Laboratory investigations excluded all other known causes of multiphasic CNS demyelination. Oligoclonal antibodies were not detected in these patients at any time. The patients were all from a population with a low risk for MS (black South Africans). CONCLUSION: The patients described here represent a new phenotypic expression of a recurrent (multiphasic), steroid sensitive, inflammatory demyelinating disorder of the CNS occurring in black South Africans. The disorder is either a distinct inflammatory demyelinating disorder of the CNS of as yet unknown aetiology, or a varied form of MS (ADEM/NMO) occurring in a population with a low risk (where the genetic trait and environmental risk factors for MS do not exist) for MS.- - - - - - - - - - ranking = 16.805608534202keywords = neuromyelitis optica, neuromyelitis, optica (Clic here for more details about this article) |
3/19. 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 = 16.805608534202keywords = neuromyelitis optica, neuromyelitis, optica (Clic here for more details about this article) |
4/19. Demyelinating peripheral neuropathy in Devic disease.This is a report of a 20-year-old man with typical features of Devic disease (neuromyelitis optica) associated with demyelinating peripheral neuropathy. A sural nerve biopsy showed markedly decreased myelinated fibers of a large diameter. Teased fiber preparations showed segmental remyelination in 50% of examined fibers, as well as a few demyelination. Demyelination of bilateral optic nerves, spinal cord, and peripheral nerves at the same time suggests a possibility of common pathogenetic mechanisms in both the central and peripheral nervous systems.- - - - - - - - - - ranking = 16.805608534202keywords = neuromyelitis optica, neuromyelitis, optica (Clic here for more details about this article) |
5/19. A rare cystic non-functioning neuroendocrine pancreatic tumor with an unusual presentation.This report describes a patient with a cystic non-functioning neuroendocrine glucagon cell pancreatic tumor presenting with demyelination of the optical nerve that had initially provoked marked monolateral reduced vision and had led to a suspected diagnosis of multiple sclerosis. Cystic degeneration is uncommon in endocrine pancreatic tumors due to their abundant vascular supply. Very few cases of cystic neuroendocrine non-functioning pancreatic tumors have been reported in the international literature. The presence of atypical neurological symptoms, such as sudden visual impairment, should be taken into account in the differential diagnosis for such tumors. The prognosis is poor, because most of these tumors are malignant and diagnosed at an advanced stage. The three-year disease-free survival of our patient, however, encourages the use of aggressive surgical treatment.- - - - - - - - - - ranking = 1keywords = optica (Clic here for more details about this article) |
6/19. neuromyelitis optica (Devic's syndrome) and pulmonary tuberculosis.neuromyelitis optica and acute necrotic myelopathy occur in association with pulmonary tuberculosis. We studied 8 patients with either neuromyelitis optica (6), acute myelopathy (1), or acute optic neuropathy (1) in close temporal association with pulmonary tuberculosis, but with no evidence for CNS tuberculosis. Neurologic symptoms preceded the use of antituberculosis medication in 5 patients. Different patients showed similar clinical features, suggesting a consistent disease pattern. autopsy examination (1 patient) revealed extensive spinal cord and optic nerve demyelination. We identified only 5 additional patients seen over the same period with idiopathic neuromyelitis optica, thus suggesting that the close temporal relationship to pulmonary tuberculosis is not coincidental. The syndrome is most likely due to an immune reaction to tuberculosis rather than the use of antituberculosis medication.- - - - - - - - - - ranking = 38.611217068403keywords = neuromyelitis optica, neuromyelitis, optica (Clic here for more details about this article) |
7/19. Devic's neuromyelitis optica: long-term follow-up and serial CSF findings in two cases.Sixteen cerebrospinal fluid (CSF) specimens serially obtained during long-term follow-up of two patients with Devic's neuromyelitis optica (DNO) were compared with 65 CSF samples from patients with multiple sclerosis (MS). By statistical analysis, the CSF profile in DNO was found to differ from that observed in MS, mainly showing pleocytosis, blood-brain barrier damage, and absence of persistent immunoglobulin g synthesis within the central nervous system. oligoclonal bands, detected with isoelectric focusing, were present in CSF of 92% of the patients with MS, and in three CSF specimens from one patient with DNO during the first 6 months after disease onset. The bands disappeared in two subsequent samples. This finding has never been described in MS. One patient with DNO had an apparent chronic-relapsing course probably due to steroid dependence. The clinical and CSF features of our cases favour the nosographic independence of DNO and MS.- - - - - - - - - - ranking = 84.028042671008keywords = neuromyelitis optica, neuromyelitis, optica (Clic here for more details about this article) |
8/19. neuromyelitis optica (Devic's disease) report of one case.neuromyelitis optica also named Devic's disease is an acute combined optic neuritis and transverse myelitis. It is thought to be a variant of multiple sclerosis, but its clinical presentation probably has only one attack without further recurrence and exacerbation. We present a 12-year-old girl who suffered from sudden onset of lower extremeties weakness, sensory loss and blurred vision after a prodromal URI symptom. CSF examination showed mild pleocytosis, elevated immunoglobulins, mild elevation of protein concentration. No oligoclonal band was detected. serum virology showed high titer of anti-EB virus antibody. Visual evoke potential showed prolong of latency and decreased amplitude of both eyes. After prednisolone treatment, her visual accuity began to improve on the 7th hospital day and motor function improved on the 11th hospital day. Two years later, she has normal visual accuity, normal motor function and shows no evidence of disease recurrence.- - - - - - - - - - ranking = 5keywords = optica (Clic here for more details about this article) |
9/19. neuromyelitis optica in the elderly.Acute transverse myelitis and monolateral optic neuritis developed within a few weeks of each other in a 68-year-old woman. CSF findings consisted of elevated protein, pleocytosis, increased IgG and presence of one oligoclonal band in the electrophoresis. MRI investigation revealed diffusely increased signal on the T2-weighted images in the cervical and thoracic tracts of the spinal cord. Notwithstanding an intense steroid therapy, the patient died after 6 weeks. Despite the absence of post-mortem examination, clinical, laboratory and neuroimaging findings are consistent with the diagnosis of neuromyelitis optica (NO) related to multiple sclerosis. This case serves to document that NO can occur initially at an advanced age.- - - - - - - - - - ranking = 20.805608534202keywords = neuromyelitis optica, neuromyelitis, optica (Clic here for more details about this article) |
10/19. neuromyelitis optica: two new cases and review of the literature.The clinical features of two recent cases of neuromyelitis optica are reviewed, along with 43 cases from the literature. Severe bilateral visual impairment, thoracic myelitis, prodromal symptoms suggesting a viral syndrome, and moderate pleocytosis of the cerebrospinal fluid (CSF) were characteristic. Respiratory failure developed in 22% of the cases. Seventy percent of patients improved neurologically, 14% had a poor neurological outcome, and 16% died in the acute stages. Predictors of a poor outcome were older age, marked CSF pleocytosis, and severe myelitis. Forty-two percent of patients had a recurrence of demyelinating disease after initial recovery, suggesting a diagnosis of multiple sclerosis. Fifty-eight percent of patients had a self-limited monophasic illness, consistent with a post-infectious encephalomyelitis. No clear predictors of patients at risk for recurrence were identified. CSF oligoclonal bands were absent in three patients with information available.- - - - - - - - - - ranking = 20.805608534202keywords = neuromyelitis optica, neuromyelitis, optica (Clic here for more details about this article) |
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