Long-term Observation in Patients with Duchenne Muscular Dystrophy with Early Introduction of a Standing Program Using Knee-ankle-foot Orthoses.

Objectives: This study investigated the outcomes of the early introduction of a standing program for patients with Duchenne muscular dystrophy (DMD).

Methods: This was a retrospective observational study of 41 outpatients with DMD aged 15-20 years. We introduced the standing program using knee-ankle-foot orthoses (KAFO) to slow the progression of scoliosis when ankle dorsiflexion became less than 0° in the ambulatory period.

Common Motor Drive Triggers Response of Prime Movers When Two Fingers Simultaneously Respond to a Cue.

This study investigated whether the motor execution process of one finger movement in response to a start cue is influenced by the participation of another finger movement and whether the process of the finger movement is dependent on the movement direction. The participants performed a simple reaction time (RT) task, the abduction or flexion of one (index or little finger) or two fingers (index and little fingers). The RT of the prime mover for the finger abduction was significantly longer than that for the flexion, indicating that the time taken for the motor execution of the finger response is dependent on the movement direction.

Viltolarsen in Japanese Duchenne muscular dystrophy patients: A phase 1/2 study.

Objective: The novel morpholino antisense oligonucleotide viltolarsen targets exon 53 of the dystrophin gene, and could be an effective treatment for patients with Duchenne muscular dystrophy (DMD). We investigated viltolarsen's ability to induce dystrophin expression and examined its safety in DMD patients.

Methods: In this open-label, multicenter, parallel-group, phase 1/2, exploratory study, 16 ambulant and nonambulant males aged 5-12 years with DMD received viltolarsen 40 or 80 mg/kg/week via intravenous infusion for 24 weeks.

Early phase 2 trial of TAS-205 in patients with Duchenne muscular dystrophy.

Objective: Duchenne muscular dystrophy (DMD) is a progressive muscular disease characterized by chronic cycles of inflammatory and necrotic processes. Prostaglandin D (PGD ) is produced by hematopoietic PGD synthase (HPGDS), which is pathologically implicated in muscle necrosis. This randomized, double-blind, placebo-controlled early phase 2 study (NCT02752048) aimed to assess the efficacy and safety of the novel selective HPGDS inhibitor, TAS-205, with exploratory measures in male DMD patients aged ≥5 years.

Cumulative jerk as an outcome measure in nonambulatory Duchenne muscular dystrophy.

Objectives: Quantitative or semiquantitative outcome measures for patients with Duchenne muscular dystrophy (DMD) are important, as they can be objective indicators of the natural history of DMD; these measures also aid in the evaluation of the efficacy of various treatments. However, the most widely used standard outcome measures in patients with DMD, such as the North Star Ambulatory Assessment and the 6-min walk test, cannot be applied after patients have become nonambulatory. We evaluated the utility and reliability of accelerometric analysis of motor activity in nonambulatory patients with DMD.

Processes of anticipatory postural adjustment and step movement of gait initiation.

The purpose of this study was to elucidate whether the anticipatory postural adjustment (APA) and focal step movement of gait initiation are produced as a single process or different processes and whether the APA receives an inhibitory drive from the ongoing stop process of gait initiation. Healthy humans initiated gait in response to a first visual cue that instructed the initial swing leg. In some trials, a switch or stop cue was also provided after the first cue.

Interhemispheric Inhibition Induced by Transcranial Magnetic Stimulation Over Primary Sensory Cortex.

The present study investigated whether the long-interval interhemispheric inhibition (LIHI) is induced by the transcranial magnetic stimulation over the primary sensory area (S1-TMS) without activation of the conditioning side of the primary motor area (M1) contributing to the contralateral motor evoked potential (MEP), whether the S1-TMS-induced LIHI is dependent on the status of the S1 modulated by the tactile input, and whether the pathways mediating the LIHI are different from those mediating the M1-TMS-induced LIHI. In order to give the TMS over the S1 without eliciting the MEP, the intensity of the S1-TMS was adjusted to be the sub-motor-threshold level and the trials with the MEP response elicited by the S1-TMS were discarded online. The LIHI was induced by the S1-TMS given 40 ms before the test TMS in the participants with the attenuation of the tactile perception of the digit stimulation (TPDS) induced by the S1-TMS, indicating that the LIHI is induced by the S1-TMS without activation of the conditioning side of the M1 contributing to the contralateral MEP in the participants in which the pathways mediating the TPDS is sensitive to the S1-TMS.

Change in Excitability of Corticospinal Pathway and GABA-Mediated Inhibitory Circuits of Primary Motor Cortex Induced by Contraction of Adjacent Hand Muscle.

The present study examined whether the excitability of the corticospinal pathway and the GABA-mediated inhibitory circuits of the primary motor cortex that project onto the corticospinal neurons in the tonically contracting hand muscle are changed by tonic contraction of the adjacent hand muscle. The motor evoked potential (MEP) and cortical silent period (CSP) in the tonically contracting hand muscle were obtained while the adjacent hand muscle was either tonically contracting or at rest. The MEP and CSP of the first dorsal interosseous (FDI) muscle elicited across the scalp sites where the MEP is predominantly elicited in the FDI muscle were decreased by tonic contraction of the abductor digiti minimi (ADM) muscle.

Monaural Auditory Cue Affects the Process of Choosing the Initial Swing Leg in Gait Initiation.

The authors investigated the effect of an auditory cue on the choice of the initial swing leg in gait initiation. Healthy humans initiated a gait in response to a monaural or binaural auditory cue. When the auditory cue was given in the ear ipsilateral to the preferred leg side, the participants consistently initiated their gait with the preferred leg.

Long-term effect of repeated lidocaine injections into the external oblique for upper camptocormia in Parkinson's disease.

Background: Parkinson's disease (PD) is occasionally complicated by camptocormia. In a previous study, we classified camptocormia into upper and lower types based on the inflection point, and reported that lidocaine injection into the external oblique muscle, but not into the internal oblique or rectus abdomen, improved upper camptocormia in PD. The effect of a single lidocaine injection disappeared over a period of few days.

The effect of advance information about the sequence of a to-be-signaled motor response on corticospinal excitability during the foreperiod.

We investigated the effect of advance information about the sequence of a to-be-signaled motor response on corticospinal excitability during the foreperiod in healthy humans. Advance information about the sequence of a to-be-signaled motor response was provided by a precue signal 600 ms before a response signal during a warned choice reaction task. The precue signal indicated the sequence of one of three to-be-signaled motor responses: simple, repetitive, or sequential motor responses.

Long latency electromyographic response induced by transcranial magnetic stimulation over the cerebellum preferentially appears during continuous visually guided manual tracking task.

We investigated whether long latency motor response induced by transcranial magnetic stimulation over the cerebellum (C-TMS) preferentially appears during a continuous visually guided manual tracking task, and whether it originates in a concomitantly evoked neck twitch. C-TMS or magnetic stimulation over the neck (N-MS) was delivered during one of four tasks: a continuous or discrete visually guided manual tracking task, or phasic or tonic contraction of the first dorsal interosseous muscle. The probability of long latency fluctuation of index finger movement induced by C-TMS was not significantly different from that induced by N-MS, but the probability of long latency fluctuation induced by C-TMS and that induced by N-MS was significantly higher than that induced by sham TMS during all the tasks.