1/18. filtration of cerebrospinal fluid for acute demyelinating neuropathy in systemic lupus erythematosus.We report a patient with systemic lupus erythematosus complicated by an acute demyelinating neuropathy. Conventional therapy with intravenous immunoglobulins and immunoadsorption complemented by pulse methylprednisolone and cyclophosphamide failed. Institution of filtration of the cerebrospinal fluid was followed by a rapid improvement of the paresis.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
2/18. Bilateral phrenic paralysis in a patient with systemic lupus erythematosus.Respiratory manifestations of systemic lupus erythematosus (SLE) are frequent. They include respiratory muscle abnormalities, which have been implicated in the pathogenesis of the "shrinking lung syndrome" (SLS). We report the case of a patient with this syndrome, in whom diaphragmatic paralysis due to demyelinating phrenic lesions was diagnosed at the same time as SLE. follow-up studies showed a favorable clinical and diaphragmatic outcome with corticosteroid therapy, but little change in spirometry. It is concluded that severe diaphragm palsy is possibly due to phrenic nerve lesions in SLE, and that the link between diaphragm dysfunction and the SLS is probably not a straightforward one.- - - - - - - - - - ranking = 0.2keywords = demyelinating (Clic here for more details about this article) |
3/18. Chronic inflammatory demyelinating polyneuropathy in patients with systemic lupus erythematosus: prognosis and outcome.OBJECTIVES: To identify clinical characteristics, laboratory features, approaches to management, and predictors of outcome of chronic inflammatory demyelinating polyneuropathy (CIDP) in patients with systemic lupus erythematosus (SLE). methods: An analysis of 6 adults with the concurrent diagnosis of CIDP and SLE seen at a SLE Clinic from 1994 to 2004 with a review of 13 patients with SLE and CIDP reported in the medical literature from 1950 through 2004. RESULTS: Among our 6 patients with SLE and CIDP, 3 (50%) achieved a substantial clinical response to intravenous immunoglobulin (IVIg) and the remainder had a minimal response. The improved patients were more likely to have received treatment earlier (within 1 year of CIDP onset) and to respond faster (<1 to 3 months) than minimally improved patients. They tended to have CIDP features of weakness of all extremities, hyporeflexia of the upper extremities, and slowed nerve conduction velocity of the motor median nerve. Compared with minimal responders, responders had more serious internal organ manifestations and multiple autoantibodies associated with SLE. review of the literature identified 13 previously reported CIDP patients with SLE. Many had neurological involvement of all extremities, nerve biopsies showing demyelination, and serious SLE internal organ manifestations. Most were treated with steroids, but the 1 treated with IVIg had similar characteristics to our subset of patients who improved with IVIg. CONCLUSIONS: CIDP is an uncommon, but not rare, manifestation of SLE. Certain characteristics including early CIDP diagnosis, involvement of all 4 extremities, hyporeflexia of the upper extremities, and slowed motor nerve conduction velocity of the median nerve in addition to SLE involvement of critical internal organs and the presence of multiple antibodies associated with SLE all appear to predict a good response to IVIg.- - - - - - - - - - ranking = 14.103605994082keywords = inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
4/18. Reversible posterior leukoencephalopathy in connective tissue diseases.OBJECTIVES: To describe a case of reversible posterior leukoencephalopathy (RPLS) involving a patient with systemic lupus erythematosus (SLE) and to review the medical literature to define the epidemiological, clinical, radiological, and therapeutic aspects of this syndrome in various connective tissue diseases. methods: Report of 1 case and review of the English literature using medline search from 1967 to 2005. RESULTS: Including our reported case, RPLS has been identified in 13 patients with connective tissue disease. In separate case reports, 9 SLE patients, 2 Wegener's granulomatosis (WG) patients, and 1 patient with SLE and systemic sclerosis presented with RPLS. Associated risk factors included malignant hypertension, acute renal failure, and recent treatment with cyclophosphamide, cyclosporine, or methylprednisolone. patients were treated with blood pressure control, hemodialysis, or withdrawal of the offending drug. In our patient, plasmapheresis and high-dose methylprednisolone resulted in a full recovery. In most cases, complete resolution of neurological symptoms occur within 2 weeks of presentation, along with improvement or resolution of imaging abnormalities. CONCLUSION: RPLS is a clinicoradiological entity, associated with reversible white matter edema involving most commonly the posterior central nervous system circulation. seizures and altered mental status in patients with SLE or WG can pose difficult diagnostic and therapeutic challenges. The differential diagnosis is broad and includes infection, uremia, hypertension, infarction, thrombosis, demyelinating disorders, and vasculitis. Accurate diagnosis of RPLS and its differentiation from other, more common causes of the central nervous system is essential to ensure the best possible outcome in this rare but life-threatening neurological disorder.- - - - - - - - - - ranking = 0.2keywords = demyelinating (Clic here for more details about this article) |
5/18. Rheumatic manifestations in myelodysplastic syndromes.The myelodysplastic syndromes are characterized by ineffective hematopoiesis with possible transformation to acute nonlymphocytic leukemia. We describe a patient with refractory anemia with excess blasts with unusual rheumatic manifestations of vasculitis, migratory synovitis, arthralgias, and myalgias. A retrospective review over a 6-month period of 162 patients with myelodysplastic syndromes found 16 patients (10%) with several rheumatic manifestations. We divided these manifestations into 4 different categories: cutaneous vasculitis, "lupus-like syndrome," neuropathy, and patients with both a rheumatic disease and a myelodysplastic syndrome. There were 7 with cutaneous vasculitis including leukocytoclastic vasculitis and other individual cases of urticarial vasculitis and panniculitis; 3 with lupus-like manifestations with histological evidence of an inflammatory process; 3 with neuropathic manifestations including peripheral neuropathy, mononeuritis with foot drop, and chronic inflammatory demyelinating polyneuropathy; and 3 patients in which their myelodysplastic syndrome was diagnosed after their rheumatic disease was known, including rheumatoid arthritis. sjogren's syndrome and mixed connective tissue disease. The class with refractory anemia with excess blasts had 9 patients with rheumatic manifestations but also had the largest number of patients in the study (46/162 or 29%). Three of the 16 patients died, all from the refractory anemia with excess blasts class, reflecting the known mortality in this group of patients. We believe there is a significant association between myelodysplastic syndromes and rheumatic manifestations.- - - - - - - - - - ranking = 2.8207211988163keywords = inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
6/18. serum antibodies to GM1 and GM3-gangliosides in systemic lupus erythematosus with chronic inflammatory demyelinating polyradiculoneuropathy.Acute symmetric demyelinating polyneuropathy of the Guillain-Barre type is known in systemic lupus erythematosus (SLE). Chronic idiopathic demyelinating polyneuropathy (CIDP) has been reported rarely with SLE. A case is reported of CIDP accompanying SLE with autoantibodies against GM1- and GM3-gangliosides. There was no historical evidence to suggest SLE, and CIDP was the first manifestation of SLE. The 38-year-old patient had elevated CSF-protein, slow nerve conduction velocities, sural nerve biopsy revealed mixed axon loss with demyelination and CIDP white matter lesions were observed in magnetic resonance imaging, the GM1- and GM3-autoantibodies may play a role in the pathogenesis of CIDP in SLE.- - - - - - - - - - ranking = 42.432247430744keywords = polyradiculoneuropathy, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
7/18. A case of systemic lupus erythematosus presenting as guillain-barre syndrome.Acute demyelinating polyneuropathy has been reported only twice as a presenting feature of systemic lupus erythematosus (SLE) in female patients. We report a male presenting with an acute demyelinating polyneuropathy who subsequently was found to have SLE.- - - - - - - - - - ranking = 0.4keywords = demyelinating (Clic here for more details about this article) |
8/18. Systemic lupus erythematosus presenting with recurrent acute demyelinating polyneuropathy.We report a patient who in the course of 3 years developed 3 distinct episodes of an acute demyelinating neuropathy, each fulfilling the criteria set up by an ad hoc committee of the National Institute of Neurological and Communicative Disorders and stroke for acute guillain-barre syndrome. Each episode was associated with strong serological evidence for lupus erythematosus. The episodes were separated by 1-2 years and each was followed by near complete clinical recovery. The only systemic feature was a pleural effusion during the last 2 episodes.- - - - - - - - - - ranking = 1keywords = demyelinating (Clic here for more details about this article) |
9/18. optic neuritis and myelopathy in systemic lupus erythematosus.The optic neuritis of systemic lupus erythematosus (S.L.E.) more frequently results in the persistence of a central scotoma or complete blindness after a single attack than demyelinating optic neuritis, although the initial clinical presentations may be identical. A significant number of patients, however, recover normal vision. optic neuritis may be the presenting symptom of S.L.E. and as myelopathy may also occur in the course of the disease, confusion with multiple sclerosis may result, especially if there are no arthritic, cutaneous nor visceral manifestations. We report a case of lupus optic neuritis associated with anticardiolipin antibodies and a circulating lupus anticoagulant and suggest these may be a marker for vasculitic optic neuritis and play a role in its aetiology.- - - - - - - - - - ranking = 0.2keywords = demyelinating (Clic here for more details about this article) |
10/18. Cerebral involvement in systemic lupus erythematosus.Four patients with central nervous system lupus erythematosus (SLE), were studied with nuclear magnetic resonance imaging (NMRI) and positron emission tomography (PET) to assess cerebral blood flow and glucose metabolism. The NMRI showed brain atrophy and demyelinating defects. The PET scan showed cerebral blood flow and metabolic abnormalities that improved in one patient after neuropsychiatric recovery. These preliminary findings demonstrate cerebral derangements in patients with SLE cerebritis.- - - - - - - - - - ranking = 0.2keywords = demyelinating (Clic here for more details about this article) |
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