Strength-Duration Time Constant in Peripheral Nerve: No Abnormality in Multiple Sclerosis

Objectives. To investigate the properties of the strength-duration time constant (SDTC) in multiple sclerosis (MS) patients. Methods. The SDTC and rheobase in 16 MS patients and 19 healthy controls were obtained following stimulation of the right median nerve at the wrist. Results. SDTC and rheobase values were 408.3 ± 60.0 μs and 4.0 ± 1.8 mA in MS patients, versus 408.0 ± 62.4 μs and 3.8 ± 2.1 mA in controls. The differences were not significant in SDTC or rheobase values between the patients and controls (P = 0.988 for SDTC and P = 0.722 for rheobase). Conclusion. Our study showed no abnormality in relapsing remitting MS patients in terms of SDTC, which gives some indirect information about peripheral Na+ channel function. This may indicate that alterations in the Na+ channel pattern in central nervous system (CNS) couldnot be shown in the peripheral nervous system (PNS) in the MS patients by SDTC. The opinion that MS can be a kind of channelopathy might be proven by performing other axonal excitability tests or SDTC in progressive forms of MS

P300 auditory event-related potentials in children with obesity: is childhood obesity related to impairment in cognitive functions?

Objective: To investigate alterations in P300 auditory event-related potentials in children with obesity to detect changes in cognitive functions

Genotypic and phenotypic presentation of transthyretin-related familial amyloid polyneuropathy (TTR-FAP) in Turkey.

Transthyretin-related familial amyloid polyneuropathy (TTR-FAP) is an autosomal dominant disorder caused by mutations of the transthyretin (TTR) gene. The mutant amyloidogenic transthyretin protein causes the systemic accumulation of amyloid fibrils that result in organ dysfunction. TTR-associated FAP is a progressive and fatal disease, if left untreated, and should be considered in the differential diagnosis of any person presenting with a progressive polyneuropathy, particularly with accompanying autonomic involvement. The clinical, electrophysiological, histopathological, and genetic characteristics of 17 patients from Turkey (5 female, 13 male) from nine families with polyneuropathy and mutations in TTR were evaluated. Sequence analysis of the TTR gene revealed five mutations (Va130Met, Glu89Gln, Gly53Glu, Glu54Gly and Gly47Glu). Mean age at disease onset was 40.4 +/- 13.9 years (range 21-66 years). The most commonly reported initial complaint was paresthesia in the feet (asymmetric in three patients). Three patients (2 male) with the Glu89Gln mutation presented with carpal tunnel syndrome. Two patients with the Gly53Glu mutation showed episodes of dysarthria and hemiparesis, consistent with this genotype. Seven patients died during the period of follow-up as a result of systemic involvement. Our study suggests that a cohort of patients from Turkey with TTR-FAP exhibits clinical and genetic heterogeneity. (C) 2016 Elsevier B.V. All rights reserved