The prevalence of advanced medical treatment has led to global population aging, resulting in increased numbers of dementia patients. One of the most intractable symptoms of dementia is apathy, or lack of interest and enthusiasm, which can accompany memory and cognitive deterioration. Development of a novel method to ameliorate apathy is desirable. In this feasibility trial, we propose a series of digital art installations as a candidate dementia intervention approach. Seven, three-minute scenes of digital images and sounds were presented to visitors either passively or in response to their reactions (motion and sound). We evaluated the potential of this application as an intervention against apathy in an elderly living home. We collected the dementia global standard Mini-Mental State Examination (MMSE) and questionnaire scores of sensory pleasure and motivation along with behavioral motion data in twenty participants. We further compared responses between the first and second experiences in the thirteen participants that were present for both days. Overall, we found a significant increase in participants’ motivation. In these subjects, head and right hand motion increased over different scenes and MMSE degrees, but was most significant during passive scenes. Despite a small number of subjects and limited evaluations, this new digital art technology holds promise as an apathy intervention in the elderly and can be improved with use of wearable motion sensors.

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Background Recessive mutations in SLC12A6 have been linked to hereditary motor sensory neuropathy with agenesis of the corpus callosum. Patients with early‐onset peripheral neuropathy associated with SLC12A6 heterozygous variants were reported in 2016. Only five families and three variants have been reported to date, and the spectrum is unclear. Here, we aim to describe the clinical and mutation spectra of SLC12A6‐related Charcot–Marie–Tooth (CMT) disease in Japanese patients. Methods We extracted SLC12A6 variants from our DNA microarray and targeted resequencing data obtained from 2598 patients with clinically suspected CMT who were referred to our genetic laboratory by neurological or neuropediatric departments across Japan. And we summarized the clinical and genetic features of these patients. Results In seven unrelated families, we identified one previously reported and three novel likely pathogenic SLC12A6 heterozygous variants, as well as two variants of uncertain significance. The mean age of onset for these patients was 17.5 ± 16.1 years. Regarding electrophysiology, the median motor nerve conduction velocity was 39.6 ± 9.5 m/sec. For the first time, we observed intellectual disability in three patients. One patient developed epilepsy, and her brain MRI revealed frontal and temporal lobe atrophy without changes in white matter and corpus callosum. Conclusions Screening for the SLC12A6 gene should be considered in patients with CMT, particularly those with central nervous system lesions, such as cognitive impairment and epilepsy, regardless of the CMT subtype.

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We read with great interest the published article titled "Anti-PM/ Scl antibody-positive dermatomyositis in a Japanese patient: A case report and review of the literature." 1 The article suggested that the frequencies of anti-PM/Scl antibody-positive patients vary by ethnicity, and their emergence is considered rare in the Asian/Japanese population. Anti-PM/Scl antibody is one of the myositis-associated autoantibodies. In Europe, it was identified in 2.2%, 11.8%, and 63.9% of the adult patients with polymyositis, systemic sclerosis (SSc), and polymyositis/SSc overlap syndrome, respectively. 2 By contrast, it has been detected in 5% of patients with juvenile myositis, with the majority of cases classified as juvenile dermatomyositis (JDM) overlap with SSc. 3 Adult patients with anti-PM/Scl antibodypositive myositis report more severe weakness in their arm abductors than that in their hip flexors, and extramuscular symptoms, such as arthralgia and Raynaud's phenomenon, are prominent. 4 Conversely, the clinical features of juvenile patients with anti-PM/ Scl antibody-positive myositis remain unknown. Herein, we report on a novel case of magnetic resonance imaging (MRI) and pathological findings in anti-PM/Scl antibody-positive juvenile myositis, and provide a literature review of the associated clinical features.A previously healthy 15-year-old Japanese girl was referred to our hospital with joint pain, muscle pain, difficulty in grasping, and malaise. She had pain in both her wrists and finger joints that lasted for 1 year. Moreover, she had muscle pain in her buttocks and limbs, as well as weakness in the cervical flexors. Despite observing Raynaud's phenomenon, heliotrope rash, Gottron's papules, and skin sclerosis were absent. Laboratory tests revealed hemoglobin level, 11.0 g/dL; white blood cell count, 6.46 × 10 9 /L; platelet count, 380×10 9 /L; erythrocyte sedimentation rate, 34 mm/h (reference range [RR], 5-15 mm/h); C-reactive protein level, 0.85 mg/dL (RR,