11/81. Asymmetrical polyneuropathy associated with churg-strauss syndrome.A 26-year-old man who presented with fever, leg edema, weakness and numbness of both hands and feet and painless palpable purpura was reported. He had had a history of asthma and arthalgia before this admission. physical examination revealed distal asymmetrical polyneuropathy. Electrodiagnostic study was consistent with polyneuropathy. skin biopsy revealed vasculitis with prominent eosinophilic infiltration. His motor power was markedly improved by prednisolone treatment.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
12/81. Motor dominant neuropathy in sjogren's syndrome: report of two cases.Most of the peripheral neuropathies in sjogren's syndrome (SS) are sensory- or autonomic-dominant. In this report, we present two cases of a rare type of neuropathy, motor dominant neuropathy, in SS. One showed signs similar to those of guillain-barre syndrome, and the other showed signs characteristic of chronic inflammatory demyelinating polyradiculoneuropathy. These patients received i.v. immunoglobulin therapy. To our knowledge, this is the first report indicating that i.v. immunoglobulin has beneficial effects on motor dominant neuropathy in SS.- - - - - - - - - - ranking = 1.5keywords = syndrome (Clic here for more details about this article) |
13/81. Intravascular lymphomatosis.Intravascular lymphomatosis, also known as endovascular lymphoma or angiotropic large cell lymphoma, is a rare malignancy typically diagnosed at autopsy. We describe a case in which the diagnosis was made at an early stage. Specific electrodiagnostic findings suggested concomitant polyneuropathy and myopathy. Suspicion of a paraneoplastic syndrome during electrodiagnostic testing prompted muscle biopsy, which revealed myopathy due to the direct infiltration of neoplastic cells. Resultant treatment with a standard chemotherapy regimen not only increased the patient's survival, but also improved neurological function.- - - - - - - - - - ranking = 0.25keywords = syndrome (Clic here for more details about this article) |
14/81. Severe sensory ataxia and demyelinating polyneuropathy with IgM anti-GM2 and GalNAc-GD1A antibodies.Several polyneuropathy syndromes have been described with polyclonal serum immunoglobulin g (IgG) or immunoglobulin m (IgM) binding to gangliosides GM2 and GalNAc-GD1a that contain the terminal trisaccharide moiety GalNAc(beta1-4)Gal(alpha2-3)NeuAc. We describe the clinical and electrodiagnostic features in two patients with serum IgM monoclonal anti-GM2 and anti-GalNAc-GD1a antibodies. These patients had slowly progressive, panmodal sensory loss with severe sensory ataxia. Electrodiagnostic testing showed demyelinating features. Prominent improvement in gait ataxia occurred after treatment with human immune globulin but not after other immunomodulating therapies. Enzyme-linked immunoabsorbent assay and thin-layer chromatography demonstrate that the patient's serum monoclonal IgM bound to gangliosides GM2 and GalNac-GD1a but not to gangliosides without the GalNAc(beta1-4)Gal(alpha2-3)NeuAc moiety. This neuropathy differs from previously reported neuropathy syndromes associated with polyclonal GM2 and GalNAc-GD1a antibodies and from other chronic demyelinating polyneuropathies. We conclude that a distinct syndrome of chronic demyelinating neuropathy with sensory ataxia, unresponsive to corticosteroids, is associated with monoclonal IgM binding to gangliosides with a terminal GalNAc(beta1-4)Gal(alpha2-3)NeuAc trisaccharide moiety. diagnosis of this syndrome is important to direct appropriate treatment.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
15/81. Autoimmune enteropathy in an adult with autoimmune multisystemic involvement.We describe the case of a 58-year-old woman with autoimmune enteropathy associated with thyroiditis, gastritis, transitory neutropenia, sicca syndrome and severe axonal polyneuropathy of autoimmune origin. Enterocyte autoantibodies were not detected. However, predisposition to autoimmune disease was indicated by the presence of high titres of anti-gastric parietal cell, anti-thyroglobulin, anti-thyroid peroxidase and anti-neutrophil antibodies. CD4 and CD8 lymphocytes were equally distributed in the lamina propria of the small intestine, but CD8 cells were highly represented among intraepithelial lymphocytes.- - - - - - - - - - ranking = 0.25keywords = syndrome (Clic here for more details about this article) |
16/81. Polyneuritis cranialis with contrast enhancement of cranial nerves on magnetic resonance imaging.The disorder of multiple cranial nerve palsies without spinal cord involvement is referred to as polyneuritis cranialis (PC) and is rare. It is thought to be an acute post-infective polyneuropathy or a variant of guillain-barre syndrome. Electrophysiological evidence of demyelination has been reported, but no radiological abnormalities of the affected cranial nerves have been noted. We report a case of PC where contrast enhanced magnetic resonance imaging (MRI) showed enhancement of the peripheral segments of the oculomotor and abducens nerves. This case illustrates the utility of MRI in the assessment of cranial nerve palsies.- - - - - - - - - - ranking = 0.25keywords = syndrome (Clic here for more details about this article) |
17/81. Sensory neuropathy with monoclonal IgM binding to a trisulfated heparin disaccharide.We studied clinical and serological features of five patients with polyneuropathy and serum immunoglobulin m (IgM) binding to the trisulfated disaccharide IdoA2S-GlcNS-6S (TS-HDS), the most abundant disaccharide component of heparin oligosaccharides. The patients all had painful, predominantly sensory polyneuropathies. Sensory loss was distal and panmodal. Electrophysiological and pathological studies were consistent with axonal loss, especially of unmyelinated axons. immunohistochemistry showed IgM and kappa light chains deposited around the rim of intermediate-sized veins in the perimysium and epineurium. serum IgM binding to TS-HDS was selective, present in high titer (>12,000), and limited to kappa light chains. We conclude that TS-HDS is a newly identified target carbohydrate antigen of some IgM M-proteins. Monoclonal IgM binding to TS-HDS is associated with a painful, predominantly sensory, polyneuropathy syndrome with axonal loss and deposition of IgM in veins. The role of IgM binding to TS-HDS in the pathogenesis of the neuropathy remains to be determined.- - - - - - - - - - ranking = 0.25keywords = syndrome (Clic here for more details about this article) |
18/81. Motor-axonal polyneuropathy associated with hepatitis c virus.The association between hepatitis c virus (HCV) infection, the presence of mixed cryoglobulinemia and peripheral neuropathy is well-documented (Apartis et al., 1996). HCV is the chief cause of essential mixed cryoglobulinemia (type II cryoglobulinemia) with cryoglobulins present in up to half of patients with HCV infection (Akriviadis et al., 1997). More recently it has been stated that peripheral polyneuropathy may be associated with HCV chronic infection without mixed cryoglobulinemia (Lidove et al., 2001). patients usually present with a clinical and electrophysiology--predominantly sensory axonopathies (Apartis et al., 1996; Heckmann et al., 1999) or less frequently with fulminating vasculitis and mononeuropathy multiplex syndrome (David et al., 1996)--especially when associated with cryoglobulinemia. We report, for the first time, the association between pure motor-axonal polyneuropathy and HCV infection without cryoglobulinemia.- - - - - - - - - - ranking = 0.25keywords = syndrome (Clic here for more details about this article) |
19/81. Late onset polyneuropathy due to organophosphate (DDVP) intoxication.BACKGROUND: Organophosphate intoxication can cause some well-known life threatening acute neurological complications such as seizures, paralysis, neuromuscular and cardiac conduction disorders. Less often, a predominantly motor and delayed axonal neuropathy can occur. This syndrome is due to inhibition of neuropathy target esterase. CASE REPORT: A 30-year-old woman attempted suicide by drinking approximately 1,000mg/kg dimethyl-2,2-dichloro vinyl phosphate (DDVP). After a muscarinic and cholinergic syndrome lasting four days, she developed a purely motor distal axonal polyneuropathy on the fifth week after ingestion confirmed by electroneuromyography and sural nerve biopsy. Neurological examination and electroneuromyography revealed a slight recovery at the end of the 21st month. CONCLUSION: This case of late onset polyneuropathy caused by organophosphate intoxication had unusual features such as intact sensory nerves and worse prognosis when compared to previously reported cases.- - - - - - - - - - ranking = 0.5keywords = syndrome (Clic here for more details about this article) |
20/81. Reversible acute axonal polyneuropathy associated with Wernicke-korsakoff syndrome: impaired physiological nerve conduction due to thiamine deficiency?Acute axonal polyneuropathy and Wernicke-Korsakoff encephalopathy developed simultaneously in three patients. Nerve conduction studies (NCS) detected markedly decreased compound muscle action potentials (CMAPs) and sensory nerve action potentials (SNAPs) with minimal conduction slowing; sympathetic skin responses (SSRs) were also notably decreased. sural nerve biopsies showed only mild axonal degeneration with scattered myelin ovoid formation. The symptoms of neuropathy lessened within two weeks after an intravenous thiamine infusion. CMAPs, SNAPs, and SSRs also increased considerably. We suggest that this is a new type of peripheral nerve impairment: physiological conduction failure with minimal conduction delay due to thiamine deficiency.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
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