Repeated Acute Exacerbations of Chronic Inflammatory Demyelinating Polyradiculoneuropathy Accompanied by Pain and Swelling in Distal Extremities.

An 81-year-old man experienced acute progression of weakness in the extremities accompanied by a fever, tenderness, and swelling in distal parts of the extremities. He had flaccid tetraparesis with fasciculations and general hyporeflexia. Nerve conduction studies indicated demyelinating sensorimotor neuropathy. A cerebrospinal fluid examination revealed elevated proteins without pleocytosis. Immunological treatments were effective, but his symptoms exhibited repeated relapse and remission phases. He was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with an acute onset. The highlight of this case is pain with inflammatory reaction recognized as red flags of CIDP, with the clinical course and electrophysiological findings compatible with CIDP.

Integrated Extrinsic and Intrinsic Self-Healing of Polysiloxane Materials by Cleavable Molecular Cages Encapsulating Fluoride Ions.

Self-healing ability is crucial to increasing the lifetime and reliability of materials. In this study, spatiotemporal control of the healing of a polysiloxane material is achieved using a cleavable cage compound encapsulating a fluoride ion (F- ), which triggeres the dynamic rearrangement of the siloxane (Si-O-Si) networks. A self-healing siloxane-based elastomer is prepared by cross-linking polydimethylsiloxane (PDMS) with a F- -encapsulating cage-type germoxane (Ge-O-Ge) compound. This material can self-heal repeatedly under humid conditions. The F- released by hydrolytic cleavage of the cage framework contributes to rejoining of the cut pieces by promoting the local rearrangement of the siloxane networks. The use of a molecular cage encapsulating a catalyst for dynamic bond rearrangement provides a new concept for designing self-healing polysiloxane materials based on integrated extrinsic and intrinsic mechanisms.

[An autopsy case of polyarteritis nodosa accompanied with multiple immune-specific autoantibodies and rhabdomyolysis].

A 72-year-old male complained of fever lasting 1 month and developed muscle weakness and paresthesia in the legs. He presented with muscle weakness, grasping pain, decreased deep tendon reflexes in the extremities, and reduction of tactile sensation in the distal parts of the left leg muscles. Blood tests revealed leukocytosis and inflammatory reactions. Collagen-disease-specific autoantibodies including anti-double-stranded DNA and anti-Scl-70 antibodies were positive, but antineutrophil cytoplastic antibodies were negative. Nerve conduction studies revealed asymmetric axonal degeneration, indicating multiple mononeuropathy. We started intravenous methylprednisolone pulse and plasma exchange therapies. However, the patient developed intestinal necrosis and perforation, and he died 44 days after the onset of fever. An autopsy revealed vasculitis in small- to medium-sized vessels in multiple organs as well as myoglobin casts in the renal tubules, which were suggestive polyarteritis nodosa (PAN) accompanied with rhabdomyolysis. Positivity for collagen-disease-specific autoantibodies and accompanying rhabdomyolysis are atypical findings with PAN. This patient was not clinically diagnosed as PAN, and so promptly starting immunotherapies should be considered when a case presents with evidence of vasculitis.

Bilateral asterixis in a patient with bilateral anterior cerebral artery infarction.

A 83-year-old woman complained of muscular weakness in the left leg and trembling in all extremities. She was apathetic and had left leg paresis and asterixis in all extremities. Magnetic resonance imaging revealed acute cerebral infarctions in the bilateral frontal lobes perfused by the anterior cerebral artery (ACA). Anticoagulant treatments improved ischemia-induced damage of the frontal lobes, and then her neurological symptoms including asterixis gradually disappeared. A unique point of this case is that acute stroke in the bilateral ACA territory induced bilateral asterixis resembling metabolic encephalopathy. Occurrence of the bilateral ACA territory infarction is extremely rare, but it should be considered in patients presenting with bilateral asterixis.

Predictors for Incident Mild Parkinsonian Signs in Older Japanese.

BACKGROUND: Mild parkinsonian signs are important clinical symptoms related to the decline of motor and cognitive functions. We aimed to identify predictors for the incidence of mild parkinsonian signs in older Japanese by conducting an 8-year longitudinal community-based cohort study. METHODS: Participants aged 65 years or older, living in Ama-cho, a rural island town in western Japan, underwent a baseline assessment of motor function, cognitive function, depression score, the Pittsburgh Sleep Quality Index (PSQI), the Tanner questionnaire, and cerebral white matter lesions on brain magnetic resonance imaging from 2008 to 2010, and then underwent a follow-up neurological examination from 2016 to 2017. Mild parkinsonian signs were defined according to a modified Unified Parkinson's Disease Rating Scale score. RESULTS: Of the 316 participants without mild parkinsonian signs at baseline, 94 presented with incident mild parkinsonian signs at follow-up. In addition to an absence of exercise habits, a higher score on the Tanner questionnaire, PSQI, and deep white-matter hyperintensity Fazekas scores were significant independent predictors for incidence of mild parkinsonian signs. CONCLUSION: We suggest multiple factors related to incidence of mild parkinsonian signs. Vascular lesions and sleep disorders are associated with a pathogenesis of mild parkinsonian signs, the Tanner questionnaire is useful for early detection of subclinical mild parkinsonian signs, and exercise has a possibility of being associated with preventing onset of mild parkinsonian signs.

Association between exercise habits and subcortical gray matter volumes in healthy elderly people: A population-based study in Japan.

BACKGROUND AND AIMS: The relationship between exercise and subcortical gray matter volume is not well understood in the elderly population, although reports indicate that exercise may prevent cortical gray matter atrophy. To elucidate this association in the elderly, we measured subcortical gray matter volume and correlated this with volumes to exercise habits in a community-based cohort study in Japan. METHODS: Subjects without mild cognitive impairment or dementia (n = 280, 35% male, mean age 73.1 ± 5.9 years) were evaluated using the Mini-Mental State Examination (MMSE), an exercise habit questionnaire, and brain magnetic resonance imaging. Subcortical gray matter volume was compared between groups based on the presence/absence of exercise habits. The MMSE was re-administered 3 years after the baseline examination. RESULTS: Ninety-one subjects (32.5%) reported exercise habits (exercise group), and 189 subjects (67.5%) reported no exercise habits (non-exercise group). Volumetric analysis revealed that the volumes in the exercise group were greater in the left hippocampus (p = 0.042) and bilateral nucleus accumbens (left, p = 0.047; right, p = 0.007) compared to those of the non-exercise group. Among the 195 subjects who received a follow-up MMSE examination, the normalized intra-cranial volumes of the left nucleus accumbens (p = 0.004) and right amygdala (p = 0.014)showed significant association with a decline in the follow-up MMSE score. CONCLUSION: Subjects with exercise habits show larger subcortical gray matter volumes than subjects without exercise habits in community-dwelling elderly subjects in Japan. Specifically, the volume of the nucleus accumbens correlates with both exercise habits and cognitive preservation.

Differences in clinical characteristics when REM sleep behavior disorder precedes or comes after the onset of Parkinson's disease.

OBJECTIVE: Rapid eye movement (REM) sleep behavior disorder (RBD) is important not only as a preclinical symptom of Parkinson's disease (PD), but also as an aggravating symptom of PD. However, it is not known whether the onset of RBD in relation to PD affects the clinical characteristics of PD. A cross-sectional study comparing clinical characteristics of PD between patients with RBD occurring before and after the onset of PD was conducted. METHODS: Interviews regarding RBD symptoms were conducted and polysomnography was performed on 136 patients with PD. Patients with PD were divided into a group with RBD and a group without RBD. Moreover, the group with RBD was subdivided into those with RBD before the onset of PD (RBD→PD) and those with RBD after the onset of PD (PD→RBD). Clinical characteristics of the patients with and without RBD, and PD with RBD→PD and PD→RBD were compared. RESULTS: Patients with RBD (47 PD patients) had more severe parkinsonian symptoms, autonomic dysfunction, and cognitive impairment than those without RBD (89 PD patients). Moreover, 38 PD patients with PD→RBD had greater cognitive impairment including Mini-metal examination than 9 with RBD→PD in spite of similar motor and autonomic dysfunction with similar dopaminergic agents. CONCLUSION: The occurrence of RBD after the onset of PD might be an important factor aggravating cognitive function.

Longitudinal course of mild parkinsonian signs in elderly people: A population-based study in Japan.

We aimed to clarify the longitudinal course of mild parkinsonian signs (MPS) and their association with dementia and functional disability by conducting a comprehensive epidemiological study, including brain MRI, and assessments of cognition, depression, and sleep, in people aged ≥65years living in Ama-cho. We diagnosed MPS and parkinsonism (PS) using a modified Unified Parkinson's Disease Rating Scale. The phase I study was conducted between 2008 and 2010 (n=729) and the phase II between 2011 and 2013 (n=436). By phase II, 8.5% of the phase I participants without PS had developed PS. In addition to older age, a lower Mini-Mental State Examination (MMSE) score, and lower body mass index, the MPS rigidity subtype was a significant independent predictor of PS onset. By phase II, 10.1% of the participants without dementia or PS at phase I had developed dementia. Older age, lower MMSE score, and the axial dysfunction and tremor MPS subtypes were significant independent predictors of dementia development. By phase II, 38.8% of participants with MPS at phase I showed no motor symptoms. Younger age and adequate sleep were significant predictors for this reversion. Periventricular and deep white matter hyperintensity Fazekas scores increased with the evolution of parkinsonian signs. MPS is therefore critically, although sometimes reversibly, associated with PS and dementia development in elderly people.

Screening tools for clinical characteristics of probable REM sleep behavior disorder in patients with Parkinson's disease.

INTRODUCTION: The REM sleep behavior disorder (RBD) screening questionnaire (RBDSQ) has been used as a screening tool for RBD. We investigated the clinical characteristics of probable RBD (pRBD) using the RBDSQ in patients with Parkinson's disease (PD). METHODS: Seventy patients with PD (age: 69.2 ± 8.9 years old, 31 males and 49 females, length of PD morbidity: 7.4 ± 6.4 years, Hoehn and Yahr: 2.7 ± 0.8) underwent examination including the RBDSQ and Mini-Mental State Examination (MMSE) in both 2011 and 2013. We assessed the changes and characteristics of pRBD associated with PD during the two year interval. RESULTS: Nineteen patients (27.1%) in 2011 and 27 patients (38.6%) in 2013 were diagnosed as having pRBD because they scored 6 or higher on the RBDSQ. During the 2 year interval, twelve patients showed persistent pRBD, 15 developed pRBD, and 7 showed improved pRBD. In 2013, PD patients with pRBD took higher amounts of levodopa equivalents and scored lower on the MMSE than those without pRBD. Also, more PD patients with pRBD had dementia than those without pRBD. Similarly, more patients with persistent and developed pRBD had dementia than those without. CONCLUSION: We found that RBD symptoms might fluctuate during the clinical course of PD, and RBD symptoms might temporarily affect cognitive impairment.