کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
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1914001 | 1535143 | 2012 | 6 صفحه PDF | دانلود رایگان |
![عکس صفحه اول مقاله: Clinical and pathological heterogeneity in a series of 31 patients with IgM-related neuropathy Clinical and pathological heterogeneity in a series of 31 patients with IgM-related neuropathy](/preview/png/1914001.png)
BackgroundIgM-related neuropathy generally presents as a late-onset demyelinating polyneuropathy with predominant sensory loss and ataxia. Sporadic cases with atypical presentation have been described.Patients and methodsWe report clinical and pathological findings from 31 patients with IgM-related neuropathy followed in our Institute of Neurology over a 20-year period.ResultsTypical presentation with predominant sensory ataxic neuropathy was observed in 18/31 patients. In the remaining 13/31 (42%), we observed an atypical phenotype, characterized by multiple mononeuropathy or polyneuropathy with predominant motor impairment; one patient had polyneuropathy with predominant small-fibre involvement. Uncommon pathological findings consisting in inflammatory infiltrates, focal axonal loss or light chain deposition were observed in 8 patients, all with atypical clinical phenotype. Almost all patients with atypical phenotype improved with immunosuppressive therapy.ConclusionsA significant proportion of patients with IgM-related neuropathy presents with atypical clinical features. In these patients, sural nerve biopsy helps clarify heterogeneous disease mechanisms and identify patients who might benefit from immunosuppressive therapy.
Journal: Journal of the Neurological Sciences - Volume 319, Issues 1–2, 15 August 2012, Pages 75–80