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
Objective: To evaluate specific patterns of locomotion in Huntington’s disease (HD) and notably the respective roles of hypokinesia (i. e. a decrease in the amplitude of movement) and bradykinesia (i. e. difficulty i...Objective: To evaluate specific patterns of locomotion in Huntington’s disease (HD) and notably the respective roles of hypokinesia (i. e. a decrease in the amplitude of movement) and bradykinesia (i. e. difficulty in executing a movement, slowness) in gait disturbance. Methods: Kinematic, spatial (stride length, speed), temporal (cadence, speed, and stride time) and angular gait parameters (jo int ankle range) were recorded in 15 early-stage HD patients by means of a video motion analysis system and then compared with 15 controls and 15 Parkinson’s disease (PD) patients. Hypokinesia was studied in terms of both spatial (decrease in stride length) and angular gait parameters (decrease in joint ankle range), whereas hyperkinesia was characterized by an increase in joint ankle range. Bradykinesia (defined by a decrease in gait velocity) was also assessed in terms of temporal parameters (cadence, stride time). We studied the influence of clinical symptoms (motor dysfunction, chorea, overall disability and cognitive impairment) and the CAG repeat number on gait abnormalities. Results: we observed a clear decrease in gait speed, a decrease in cadence and an increase in stride time (i. e. bradykinesia) for HD, with significant intra-individual variability. Cadence remained normal in PD. In HD, there was no evidence for a clear decrease in stride length, although the latter is a characteristic feature of hypokinetic gait (such as that observed in PD). Angle analysis revealed the coexistence of hyperkinesia and hypokinesia in HD, which thus participate in gait abnormalities. Gait speed in HD was correlated to the motor part of the UHDRS. Conclusion: Gait in HD is mainly characterized by a timing disorder: bradykinesia was present, with severe intra-individual variability in temporal gait parameters.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Objective: To evaluate specific patterns of locomotion in Huntington’s disease (HD) and notably the respective roles of hypokinesia (i. e. a decrease in the amplitude of movement) and bradykinesia (i. e. difficulty in executing a movement, slowness) in gait disturbance. Methods: Kinematic, spatial (stride length, speed), temporal (cadence, speed, and stride time) and angular gait parameters (jo int ankle range) were recorded in 15 early-stage HD patients by means of a video motion analysis system and then compared with 15 controls and 15 Parkinson’s disease (PD) patients. Hypokinesia was studied in terms of both spatial (decrease in stride length) and angular gait parameters (decrease in joint ankle range), whereas hyperkinesia was characterized by an increase in joint ankle range. Bradykinesia (defined by a decrease in gait velocity) was also assessed in terms of temporal parameters (cadence, stride time). We studied the influence of clinical symptoms (motor dysfunction, chorea, overall disability and cognitive impairment) and the CAG repeat number on gait abnormalities. Results: we observed a clear decrease in gait speed, a decrease in cadence and an increase in stride time (i. e. bradykinesia) for HD, with significant intra-individual variability. Cadence remained normal in PD. In HD, there was no evidence for a clear decrease in stride length, although the latter is a characteristic feature of hypokinetic gait (such as that observed in PD). Angle analysis revealed the coexistence of hyperkinesia and hypokinesia in HD, which thus participate in gait abnormalities. Gait speed in HD was correlated to the motor part of the UHDRS. Conclusion: Gait in HD is mainly characterized by a timing disorder: bradykinesia was present, with severe intra-individual variability in temporal gait parameters.
