Introduction. Polyneuropathies associated with IgM monoclonal gammopathy were recently recognized. Antibodies can react with glycoproteins such as myelin asso ciated glycoprotein (MAG), or gangliosides containing one ...Introduction. Polyneuropathies associated with IgM monoclonal gammopathy were recently recognized. Antibodies can react with glycoproteins such as myelin asso ciated glycoprotein (MAG), or gangliosides containing one sialosyl epitope such as GM1 or several sialosyl epitopes (polysialyted gangliosides) including GD2, G D3, GT1b, GT1a, GQ1b. Methods. We report on three patients presenting oculomotor dysfunction, chronic sensitive ataxic polyneuropathy, high sedimentation rate, IgM monoclonal paraprotein of unknown signification and antidisialosyl IgM antib odies and for two of them cold agglutinins. Such features have been previously d escribed under the acronym “CANOMAD”(chronic ataxic neuropathy with ophthalmop legia, M protein, agglutination and disialosyl antibodies). Results. One of the patients presents extra membranous glomerulopathy and severe motor disability as sociated with this syndrome. The pathophysiology of the glomerulopathy seems to be linked with the polyneuropathy. Patients were treated either by intravenous i mmunoglobulin, corticosteroids or cyclophosphamid. Response to treatment differs in the three cases and there is currently no consensus. Conclusion. Our study d emonstrates that spectrum of polyneuropathy associated with monoclonal polyneuro pathy may be larger than originally described.展开更多
Introduction. Guillain-Barré Syndrome (GBS) is generally related to perip heral nervous system involvement, but certain variants with central nervous syst em manifestations have been described. Case report. In th...Introduction. Guillain-Barré Syndrome (GBS) is generally related to perip heral nervous system involvement, but certain variants with central nervous syst em manifestations have been described. Case report. In the present study we repo rt 2 patients with GBS associated with hallucinations and onirism. Two men (age 64 and 49 years) presented GBS without proven infectious origin who required int ensive care because of respiratory problems. The disease progressed and manifest ations of encephalitis (hallucinations and onirism) appeared. The sensorimotor s igns and encephalitis manifestations evolved in parallel with full recovery in t he first patient and death after 11 months of intensive care in the second. Conc lusion. GBS may be associated with stereotypic central nervous system symptoms, mimicking delirium tremens. The manifestations would be related to the severity of the initial period, but not to long-term prognosis.展开更多
Introduction. Acute or sub- acute pure dysautonomia is uncommon.We report a case of sub- acute pure pandysautonomia with favorable outcome after intravenous immunoglobulin therapy.Case report. A 29- year- old right- h...Introduction. Acute or sub- acute pure dysautonomia is uncommon.We report a case of sub- acute pure pandysautonomia with favorable outcome after intravenous immunoglobulin therapy.Case report. A 29- year- old right- handed student, with an uneventful medical history presented, for one month, bilateral loss of visual acuity and digestive disorders, associating diarrhea,vomiting and anorexia. Physical examination revealed bilateral intrinsec oculomotor nerve palsy, a dryness syndrome and severe orthostatic hypotension. Ophthalmologic examination showed bilateral diffuse parasympathic impairment associating an Argyll Robertson pupil and full pupil light reflex abolition. Elevated protein level (0.93g/l)was the only cerebrospinal fluid anomaly. Serum tests were negative for anti- gangliosides antibodies. The patient improved slowly after two series of intravenous immunoglobulin infusions. Conclusion. Clinical course and laboratory findings suggest that acute or sub- acute pure pandysautonomia events are likely to be related to acute polyradiculoneuritis. Therefore intravenous polyvalent immunoglobulin infusions should be attempted, even if their efficacy needs to be confirmed.展开更多
文摘Introduction. Polyneuropathies associated with IgM monoclonal gammopathy were recently recognized. Antibodies can react with glycoproteins such as myelin asso ciated glycoprotein (MAG), or gangliosides containing one sialosyl epitope such as GM1 or several sialosyl epitopes (polysialyted gangliosides) including GD2, G D3, GT1b, GT1a, GQ1b. Methods. We report on three patients presenting oculomotor dysfunction, chronic sensitive ataxic polyneuropathy, high sedimentation rate, IgM monoclonal paraprotein of unknown signification and antidisialosyl IgM antib odies and for two of them cold agglutinins. Such features have been previously d escribed under the acronym “CANOMAD”(chronic ataxic neuropathy with ophthalmop legia, M protein, agglutination and disialosyl antibodies). Results. One of the patients presents extra membranous glomerulopathy and severe motor disability as sociated with this syndrome. The pathophysiology of the glomerulopathy seems to be linked with the polyneuropathy. Patients were treated either by intravenous i mmunoglobulin, corticosteroids or cyclophosphamid. Response to treatment differs in the three cases and there is currently no consensus. Conclusion. Our study d emonstrates that spectrum of polyneuropathy associated with monoclonal polyneuro pathy may be larger than originally described.
文摘Introduction. Guillain-Barré Syndrome (GBS) is generally related to perip heral nervous system involvement, but certain variants with central nervous syst em manifestations have been described. Case report. In the present study we repo rt 2 patients with GBS associated with hallucinations and onirism. Two men (age 64 and 49 years) presented GBS without proven infectious origin who required int ensive care because of respiratory problems. The disease progressed and manifest ations of encephalitis (hallucinations and onirism) appeared. The sensorimotor s igns and encephalitis manifestations evolved in parallel with full recovery in t he first patient and death after 11 months of intensive care in the second. Conc lusion. GBS may be associated with stereotypic central nervous system symptoms, mimicking delirium tremens. The manifestations would be related to the severity of the initial period, but not to long-term prognosis.
文摘Introduction. Acute or sub- acute pure dysautonomia is uncommon.We report a case of sub- acute pure pandysautonomia with favorable outcome after intravenous immunoglobulin therapy.Case report. A 29- year- old right- handed student, with an uneventful medical history presented, for one month, bilateral loss of visual acuity and digestive disorders, associating diarrhea,vomiting and anorexia. Physical examination revealed bilateral intrinsec oculomotor nerve palsy, a dryness syndrome and severe orthostatic hypotension. Ophthalmologic examination showed bilateral diffuse parasympathic impairment associating an Argyll Robertson pupil and full pupil light reflex abolition. Elevated protein level (0.93g/l)was the only cerebrospinal fluid anomaly. Serum tests were negative for anti- gangliosides antibodies. The patient improved slowly after two series of intravenous immunoglobulin infusions. Conclusion. Clinical course and laboratory findings suggest that acute or sub- acute pure pandysautonomia events are likely to be related to acute polyradiculoneuritis. Therefore intravenous polyvalent immunoglobulin infusions should be attempted, even if their efficacy needs to be confirmed.
