Pitfalls of the E-Ref Procedure: Tie Values and the Proportion of the Abnormal Data.

Introduction: Extrapolated reference values (E-Ref) procedure is a new method for determining the cutoff value without collecting the control data. We tried to apply this method to determine the cutoff value for the distal motor latency of the median nerve (median DML). During this process, we found two pitfalls of the E-Ref method.

A Two-week, Hands-on Educational Program for Primary Care Pediatricians Aimed at Equalization of Pediatric Allergy Practice across Institutions and Regions.

Introduction: Similar to other countries, in Japan, the demand for primary care pediatricians has increased due to the surge in pediatric allergic diseases, and with the change in a paradigm shift regarding the prevention of pediatric allergic disease in the last 20 years, they have had an increased need for retraining. To offer better support to children and their caregivers, educational needs for bridging the gap between knowledge and practice must be met. Therefore, we developed an educational program including practical and interactive approaches for pediatricians in 2012.

Japanese version of the food allergy quality of life questionnaire 10: An easy-to-use instrument.

Background: The health-related quality of life (HRQL) of people with food allergies should be evaluated to provide high-quality medical care. Currently, there is no available easy-to-use and reliable instrument for assessing HRQL clinically in Japan.

Methods: The Food Allergy Quality of Life Questionnaire 10 (FAQLQ10) in English was translated into Japanese, and this was referred to as the Japanese version of the Food Allergy Quality of Life Questionnaire 10 (FAQLQ10-J).

Hereditary spastic paraplegia and extensive leukoencephalopathy: a case report of a unique phenotype associated with a GJB1/Cx32 p.Pro174Ser variant.

Background: Pathogenic variants in Gap junction protein beta 1 (GJB1), which encodes Connexin 32, are known to cause X-linked Charcot-Marie-Tooth disease (CMTX), the second most common form of CMT. CMTX presents with the following five central nervous systems (CNS) phenotypes: subclinical electrophysiological abnormalities, mild fixed abnormalities on neurological examination and/or imaging, transient CNS dysfunction, cognitive impairment, and persistent CNS manifestations.

Case Presentation: A 40-year-old Japanese male showed CNS symptoms, including nystagmus, prominent spastic paraplegia, and mild cerebellar ataxia, accompanied by subclinical peripheral neuropathy.

The speed of completion of the decremental responses on repetitive nerve stimulation.

Objective: It is generally believed that the decremental response in repetitive nerve stimulation (RNS) stabilizes at the fourth or fifth response. We have a preliminary impression that the decremental response approaches a plateau earlier in proximal muscles than in distal muscles. We investigated the speed of the completion of the decremental response in different muscles.