Polyneuropathy and monoclonal gammopathy of undetermined significance (MGUS); update of a clinical experience.

Background And Purpose: Polyneuropathies associated with monoclonal gammopathy of undetermined significance (MGUS) encompass a group of phenotypically and immunologically heterogeneous neuropathies. While the best characterized is that associated with anti-myelin glycoprotein (MAG) antibodies, there are phenotypical and immunological neuropathy variants that still lack a clear classification. We analyzed a significant number of patients, in order to better evaluate the distribution of neuropathy phenotypes and to look for some common characteristics.

A case of Takotsubo syndrome during a multiple sclerosis brainstem relapse.

Background: We report a case of a woman affected by relapsing remitting Multiple Sclerosis who developed acute left ventricular dysfunction in the context of a severe brainstem relapse, one month after an attempt of in-vitro fertilization.

Discussion: The characteristics of our case are consistent with the hypothesis of a possible causal association between an acute medulla oblongata lesion and the occurrence of Takotsubo cardiomyopathy.

Conclusion: Our case adds to previous reports in this field and calls for awareness of this exceptional extra-neurological manifestation of MS.

Fragile X premutation with atypical symptoms at onset.

Objective: To evaluate the presence of carriers of the fragile X premutation among male patients with sporadic ataxia without expansion into known spinocerebellar ataxia genes.

Design: Clinical and genetic examinations were performed on patients with sporadic pure ataxia and patients with ataxia associated with extracerebellar features such as pyramidal and extrapyramidal signs, dementia, or peripheral neuropathy.

Setting: University department of neurology.