Clinical characteristics of autoimmune GFAP astrocytopathy.

The clinical features of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy remain to be elucidated. We describe here the clinical features of 14 patients with GFAP astrocytopathy confirmed by detection of GFAP-IgG in cerebrospinal fluid (CSF). The novel findings of this study are as follows. First, over half of the patients presented with movement disorders (tremor, myoclonus, and ataxia), autonomic dysfunction (mainly urinary dysfunction), and hyponatremia. Second, most patients showed transient elevation of adenosine deaminase activity levels in CSF. Finally, some patients showed bilateral hyperintensities in the posterior part of the thalamus on brain magnetic resonance imaging.

Study protocol for single-center, open-label, randomized controlled trial to clarify the preventive efficacy of electrical stimulation for muscle atrophy after trauma.

BACKGROUND: Management of trauma involves long-term bed rest even when muscle strength in the lower extremities is preserved. Prolonged bed rest reduces muscle mass and causes muscle atrophy. A recent study reported the efficacy of rehabilitation using electrical muscle stimulation (EMS) for muscle strength maintenance in intensive care unit patients with disturbance of consciousness. However, despite the expected benefits of EMS in maintaining muscle strength, little is known about its efficacy in trauma patients. METHODS/DESIGN: A single-center, open-label, randomized controlled trial of 40 patients with pelvic fracture to test the effectiveness of 14 days of EMS. The primary outcome will be change in cross-sectional area of the thigh muscle between pre and post intervention, as measured on computed tomography images. We will analyze the primary endpoint by analysis of covariance (ANCOVA) and analyze the secondary endpoints in an exploratory manner. CONCLUSION: If our hypothesis is confirmed, this study will provide evidence that the use of EMS can be effective in preventing muscle atrophy. TRIAL REGISTRATION: UMIN registration number: UMIN000030190 . Registered on 1 December 2017.

Inorganic phosphorus (Pi) in CSF is a biomarker for SLC20A2-associated idiopathic basal ganglia calcification (IBGC1).

INTRODUCTION: Idiopathic basal ganglia calcification (IBGC), also called Fahr's disease or recently primary familial brain calcification (PFBC), is characterized by abnormal deposits of minerals including calcium mainly and phosphate in the brain. Mutations in SLC20A2 (IBGC1 (merged with former IBGC2 and IBGC3)), which encodes PiT-2, a phosphate transporter, is the major cause of IBGC. Recently, Slc20a2-KO mice have been showed to have elevated levels of inorganic phosphorus (Pi) in cerebrospinal fluid (CSF); however, CSF Pi levels in patients with IBGC have not been fully examined. METHODS: We investigated the cases of 29 patients with IBGC including six patients with SLC20A2 mutation and three patients with PDGFB mutation, and 13 controls. The levels of sodium (Na), potassium (K), chloride (Cl), calcium (Ca), and Pi in sera and CSF were determined by potentiometry and colorimetry. Moreover, clinical manifestations were investigated in the IBGC patients with high Pi levels in CSF. RESULTS: The study revealed that the average level of Pi in the CSF of the total group of patients with IBGC is significantly higher than that of the control group, and the levels of Pi in CSF of the IBGC patients with SLC20A2 mutations are significantly higher than those of the IBGC patients with PDGFB mutations, the other IBGC patients and controls. CONCLUSION: Results of this study suggest that the levels of CSF Pi will be a good biomarker for IBGC1.

Decreased regional cerebral blood flow in the bilateral thalami and medulla oblongata determined by an easy Z-score (eZIS) analysis of (99m)Tc-ECD-SPECT images in a case of MM2-thalamic-type sporadic Creutzfeldt-Jakob disease.

We report a case of autopsy-verified MM2-thalamic-type sporadic Creutzfeldt-Jakob disease (sCJD) in a 46-year-old patient with a 16-month history of abnormal behavior, progressive dementia, insomnia, and speech disturbances without family history. Neurological examination revealed progressive dementia, frontal signs, insomnia, speech disturbance, gait disturbance and bilaterally exaggerated tendon reflexes. Both brain MRI and cerebrospinal fluid examinations, including 14-3-3 protein, yielded normal results. An easy Z-score (eZIS) analysis for (99m)Tc-ethyl cysteinate dimer-single photon emission computed tomography ((99m)Tc-ECD-SPECT) revealed decreased regional cerebral blood flow in the bilateral thalami and medulla oblongata. PRNP gene analysis revealed methionine homozygosity at codon 129 without mutation. Neuropathological examinations revealed severe neuronal loss, gliosis, and hypertrophic astrocytosis in the medial thalamus and inferior olivary nucleus. A slight depletion of Purkinje cells was observed. PrP immunostaining showed no obvious PrP deposits in the basal ganglia, thalamus, cerebellum, or brainstem; however, mild synaptic-type PrP deposits with some smaller plaque-like structures were only partially observed in the localized region of the frontal lobe with the spongiform change. Western blot analyses of protease-resistant PrP showed a type 2 pattern. In conclusion, eZIS analysis of (99m)Tc-ECD-SPECT images is useful for detecting both thalamic and medullary lesions. This is the first case of medullary lesions detected in a live patient with MM2-thalamic-type sCJD using SPECT.

Daily training with realistic visual feedback improves reproducibility of event-related desynchronisation following hand motor imagery.

OBJECTIVE: Few brain-computer interface (BCI) studies have addressed learning mechanisms by exposure to visual feedback that elicits scalp electroencephalogram. We examined the effect of realistic visual feedback of hand movement associated with sensorimotor rhythm. METHODS: Thirty-two healthy participants performed in five daily training in which they were shown motor imagery of their dominant hand. Participants were randomly assigned to 1 of 4 experimental groups receiving different types of visual feedback on event-related desynchronisation (ERD) derived over the contralateral sensorimotor cortex: no feedback as a control, bar feedback with changing bar length, anatomically incongruent feedback in which the hand open/grasp picture on screen was animated at eye level, and anatomically congruent feedback in which the same hand open/grasp picture was animated on the screen overlaying the participant's hand. RESULTS: Daily training with all types of visual feedback induced more robust ERD than the no feedback condition (p < 0.05). The anatomically congruent feedback produced the highest reproducibility of ERD with the smallest inter-trial variance (p < 0.05). CONCLUSION: Realistic feedback training is a suitable method to acquire the skill to control a BCI system. SIGNIFICANCE: This finding highlights the possibility of improvement of reproducibility of ERD and can help to use BCI techniques.

Immediate effects of electrical stimulation combined with passive locomotion-like movement on gait velocity and spasticity in persons with hemiparetic stroke: a randomized controlled study.

OBJECTIVE: Research to examine the immediate effects of electrical stimulation combined with passive locomotion-like movement on gait velocity and spasticity. DESIGN: A single-masked, randomized controlled trial design. SUBJECTS: Twenty-seven stroke inpatients in subacute phase (ischemic n = 16, hemorrhagic n = 11). INTERVENTIONS: A novel approach using electrical stimulation combined with passive locomotion-like movement. MAIN MEASURES: We assessed the maximum gait speed and modified Ashworth scale before and 20 minutes after the interventions. RESULTS: The gait velocity of the electrical stimulation combined with passive locomotion-like movement group showed the increase form 0.68 ± 0.28 (mean ± SD, unit: m) to 0.76 ± 0.32 after the intervention. Both the electrical stimulation group and passive locomotion-like movement group also showed increases after the interventions (from 0.76 ± 0.37 to 0.79 ± 0.40, from 0.74 ± 0.35 to 0.77 ± 0.36, respectively). The gait velocity of the electrical stimulation combined with passive locomotion-like movement group differed significantly from those of the other groups (electrical stimulation combined with passive locomotion-like movement versus electrical stimulation: P = 0.049, electrical stimulation combined with passive locomotion-like movement versus passive locomotion-like movement: P = 0.025). Although there was no statistically significant difference in the modified Ashworth scale among the three groups, six of the nine subjects (66.6%) in the electrical stimulation combined with passive locomotion-like movement group showed improvement in the modified Ashworth scale score, while only three of the nine subjects (33.3%) in the electrical stimulation group and two of the nine subjects (22.2%) improved in the passive locomotion-like movement group. CONCLUSION: These findings suggest electrical stimulation combined with passive locomotion-like movement could improve gait velocity in stroke patients.

Effects of integrated volitional control electrical stimulation (IVES) on upper extremity function in chronic stroke.

We evaluated the efficacy of a novel electromyogram (EMG)-controlled electrical stimulation system, called the integrated volitional control electrical stimulator (IVES), on the recovery of upper extremity motor functions in patients with chronic hemiparetic stroke. Ten participants in the chronic stage (more than 12 months post-stroke with partial paralysis of their wrist and fingers) received treatment with IVES to the extensor carpi radialis and extensor digitorum communis 6 h/day for 5 days. Before and after the intervention, participants were assessed using upper-extremity Fugl-Meyer motor assessment (FMA), the active range of motion (A-ROM), the nine-hole peg test (NHPT), and surface EMG recordings. The upper extremity FMA showed a statistically significant increase from 50.8 ± 5.8 to 56.8 ± 6.2 after the intervention (P < 0.01). The A-ROM of wrist extension was also significantly improved from 36.0° ± 15.4° to 45.0° ± 15.5° (P < 0.01). The NHPT significantly decreased from 85.3 ± 52.0 to 63.3 ± 29.7 (P = 0.04). EMG measurements demonstrated statistically significant improvements in the coactivation ratios for the wrist flexor and extensor muscles after the intervention. This study suggested that 5 days of IVES treatment yields a noticeable improvement in upper extremity motor functions in patients with chronic hemiparetic stroke.

Effects of neurofeedback training with an electroencephalogram-based brain-computer interface for hand paralysis in patients with chronic stroke: a preliminary case series study.

OBJECTIVE: To explore the effectiveness of neurorehabilitative training using an electroencephalogram-based brain- computer interface for hand paralysis following stroke. DESIGN: A case series study. SUBJECTS: Eight outpatients with chronic stroke demonstrating moderate to severe hemiparesis. METHODS: Based on analysis of volitionally decreased amplitudes of sensory motor rhythm during motor imagery involving extending the affected fingers, real-time visual feedback was provided. After successful motor imagery, a mechanical orthosis partially extended the fingers. Brain-computer interface interventions were carried out once or twice a week for a period of 4-7 months, and clinical and neurophysiological examinations pre- and post-intervention were compared. RESULTS: New voluntary electromyographic activity was measured in the affected finger extensors in 4 cases who had little or no muscle activity before the training, and the other participants exhibited improvement in finger function. Significantly greater suppression of the sensory motor rhythm over both hemispheres was observed during motor imagery. Transcranial magnetic stimulation showed increased cortical excitability in the damaged hemisphere. Success rates of brain-computer interface training tended to increase as the session progressed in 4 cases. CONCLUSION: Brain-computer interface training appears to have yielded some improvement in motor function and brain plasticity. Further controlled research is needed to clarify the role of the brain-computer interface system.

The potential for essential trauma care to empower communities and tackle inequities.

In an attempt to address rapidly increasing injuries in low-income and middle-income countries (LMICs), the Essential Trauma Care Project was started with the goal of standardizing trauma care systems. This project, although part of "vertical" essential health services, has the potential to strengthen the health care system as a whole and to empower communities. Improved diagnosis, triage, referral, communication, and transport benefit the integrated health care systems weakened by vertical approaches. This project mobilizes existing resources, including lay people, to establish a "local model of prehospital care," which can raise community capability and foster trust in health systems. This article describes how this project can be an intersection between vertical and horizontal approaches.