1/13. 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 = demyelinating (Clic here for more details about this article) |
2/13. 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 = 6keywords = demyelinating (Clic here for more details about this article) |
3/13. 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 = demyelinating (Clic here for more details about this article) |
4/13. 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 = 1keywords = demyelinating (Clic here for more details about this article) |
5/13. Loss of aquaporin-4 in active perivascular lesions in neuromyelitis optica: a case report.neuromyelitis optica (NMO) is clinically characterized by severe optic neuritis and transverse myelitis. In japan, NMO has been named optic-spinal multiple sclerosis (OSMS) and it has been thought to be a subtype of multiple sclerosis (MS). However, several clinical and laboratory findings suggest NMO or OSMS is distinct from MS. Recently, the disease-specific antibody (NMO-IgG) was found in the serum from NMO patients, and its target antigen was identified as aquaporin-4 (AQP4) water channel protein which is mainly expressed in astroglial foot processes. However, the pathogenetic role of AQP4 in NMO remains unknown. We herein report a typical case of NMO in which immunohistochemical analysis showed a lack of AQP4 in the spinal cord lesions. The loss of AQP4 was evident in the central gray matter, especially in the perivascular lesions where immunoglobulins and complements were deposited, and glial fibrillary acidic protein (GFAP) staining was weak in those lesions. However, GFAP was strongly stained at the reactive astrogliosis surrounding the lesions. myelin basic protein (MBP)-stained myelinated fibers were relatively preserved in the lesions where AQP4 was lost. In contrast to these NMO lesions, AQP4 was expressed predominantly in the gray matter in control spinal cords, and AQP4 was preserved in demyelinating MS lesions. Our findings suggest that astrocytic impairment associated with humoral immunity against AQP4 may be primarily involved in the lesion formation of NMO, and that the pathomechanisms of NMO are different from those of MS in which demyelination is the primary pathology.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
6/13. neuromyelitis optica immunoglobulin g in a child.neuromyelitis optica or Devic's syndrome is an uncommon demyelinating disorder that preferentially attacks the spinal cord and optic nerves. Although it is well described in adults, childhood neuromyelitis optica has rarely been reported in the literature and is frequently misdiagnosed as severe multiple sclerosis. Recently, a serum immunoglobulin g test for neuromyelitis optica has become available which may clarify and accelerate the diagnosis. This report describes a child with recurrent myelitis and an elongated spinal cord lesion who was found to have positive neuromyelitis optica autoantibody. We believe that neuromyelitis optica autoantibody testing should be performed in cases of pediatric transverse myelitis with multiple vertical segments or recurrence.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
7/13. 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 = 1keywords = demyelinating (Clic here for more details about this article) |
8/13. neuromyelitis optica (Devic's syndrome): not always multiple sclerosis.A patient is presented with clinical signs and symptoms of neuromyelitis optica (Devic's syndrome). The clinical findings, the cerebrospinal fluid findings and the magnetic resonance imaging findings in our patient demonstrate that neuromyelitis optica is not always a form of multiple sclerosis. It can also be a manifestation of other demyelinating diseases, for example Disseminated encephalomyelitis.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
9/13. neuromyelitis optica in the old age: a clinico-pathological contribution.We present a case of neuromyelitis optica (NMO) in a previously healthy 64-year-old woman. While CT scan and MRI investigations of the brain as well as myelography were normal, CSF revealed a pathological increase of IgG. Notwithstanding an intensive steroid therapy the patient died after 4 1/2 months. Microscopic examination showed, besides extensive demyelination of the optic nerves and spinal cord (especially in its cervico-dorsal tract), several small demyelinating plaques in the white matter of the pons and near the right lateral ventricle. These findings confirm once again that NMO is a clinical variant of multiple sclerosis and that this disease may make it first appearance at an old age.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
10/13. neuromyelitis optica (Devic syndrome) in a 12-year-old male with complete recovery following steroids.neuromyelitis optica is one of the rarest forms of central nervous system demyelinating disease. It is particularly infrequent in Western countries and complete recovery is rare. We report a 12-year-old boy with neuromyelitis optica which occurred shortly after an upper respiratory infection. magnetic resonance imaging revealed several small areas of increased signal intensity within the cerebral and cerebellar white matter on T2-weighted images. The patient recovered completely following treatment with high-dose prednisone.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
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