We presented a detailed clinical evaluation with electrophysiological confirmation of ADCME syndrome in a Turkish family. This rare clinical picture might be misdiagnosed as JME and should be kept in mind to ensure correct diagnosis and to provide a homogenous group for genetic studies.

Introduction The objective of this study was to determine compound muscle action potential (CMAP) scan parameters and MScanFit motor unit number estimation (MUNE) in patients with amyotrophic lateral sclerosis (ALS) and to compare the results in the abductor pollicis brevis (APB) to those in the abductor digiti minimi (ADM). Methods CMAP scans were recorded from the APB and ADM in 35 patients with ALS and 21 controls. MScanFit MUNE, neurophysiological index (NI), step%, returner%, and D50 were calculated. Results CMAP scan parameters including the returner%, MScanFit MUNE, and NI can distinguish ALS with high sensitivity and specificity. The electrophysiological parameters, with the exception of D50 (the number of largest consecutive differences of recorded responses generating 50% of maximum CMAP), showed more pronounced changes in the APB than in the ADM, even though most of the patients had normal APB/ADM amplitude ratios. Discussion CMAP scan parameters and MScanFit MUNE can be used in the evaluation of denervation and reinnervation and may herald the “split hand” in ALS.

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Introduction: The prevalence of migraine was found to be more than three-fold higher in women as compared with men, and in addition to differences in prevalence rates, the characteristics and associated features might also differ between the sexes. The aim of this study was to compare sex-specific features of migraine and demographic parameters in a nationwide population-based study in Turkey.Methods: Among 5323 subjects, a total of 871 patients who were diagnosed as having definite migraine according to the diagnostic criteria of the International Classification of Headache Disorders-III (ICHD-III) were included in our study. The demographic characteristics, associated features, and triggers of migraine were examined with regard to sex. Results:The study group comprised 640 women (73.5%) and 231 men (26.5%), with a female to male ratio of 2.8:1. Attack duration, mean migraine disability assessment scores (MIDAS), frequencies of nausea, vomiting, osmophobia, vertigo/dizziness, and allodynia were found significantly different between women and men. When we compared these parameters between men and postmenopausal women, all these parameters were still significant except nausea. Odor was statistically more frequent as a reported trigger in women, whereas excessive sleep was a statistically more frequent triggering factor in men. The rates of depression and allergy were significantly higher in women when compared with men. Conclusion:Longer attack duration, higher MIDAS scores, and the frequencies of nausea, vomiting, osmophobia, vertigo/dizziness, and allodynia were more significant in women and this variance in sex persisted after menopause. Also, some trigger factors and comorbidities differed between the sexes. These findings might result from complex genetic factors besides sociocultural influences, biologic, and sociocultural roles. Future studies should continue to explore biologic and genetic factors with respect to sex in migraine.