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PURPOSE: This study aimed to assess reliable options for bedside diagnosis of silent aspiration in the intensive care unit by examining the use of default grayscale images (DGI) obtained using a mobile, general-purpose, radiography system capable of dynamic digital radiography (M-DDR) and inverted grayscale images (IGI) of DGI. METHODS: This cohort study (exploratory and preliminary) involved 18 adult patients (mean age, 89.0 years) for whom a swallowing assessment request was received from their primary physicians. Fifty-six IGI videoclips were evaluated by three specialists using the penetration-aspiration scale (PAS), with the gold standard being the consensus reading of all three specialists. Another three speech-language pathologists (SLPs) assessed 56 DGI and IGI videoclips using the PAS. PAS scores 1 and 2 were classified as normal range, PAS scores 3-5 as pathological laryngeal penetration, and PAS scores 6-8 as aspiration. The correct rates with IGI and DGI were then determined, and the level of agreement of IGI and DGI evaluations was evaluated. RESULTS: The correct rate of all evaluators was 100% for normal range, 80-100% for pathological laryngeal penetration, and 83-100% for aspiration with IGI and 100% for normal range, 90% for pathological laryngeal penetration, and 83% for aspiration with DGI. The kappa coefficient for IGI and DGI showed almost complete agreement for abnormal conditions. CONCLUSION: Dynamic imaging of swallowing 2-5 ml of liquid using M-DDR performed for elderly patients at the bedside showed that aspiration assessments by SLPs obtained from DGI videos immediately after imaging are acceptable.
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INTRODUCTION: We previously identified UDP-N-acetylglucosamine 2-epimerase (GNE) myopathy patients with sleep apnea and a past history of thrombocytopenia, but without disease-specific cardiac involvement. This study aimed to clarify the occurrence, severity, and serial changes of these complications. METHODS: Thirty-three genetically confirmed GNE myopathy patients who participated in a 5-y longitudinal observational history study underwent platelet count and platelet-associated immunoglobulin G (PA-IgG) measurements, a sleep study, and electrocardiography (ECG), Holter ECG, and echocardiogram examinations. RESULTS: Among the 33 patients, three had low platelet counts and 17 out of 26 were PA-IgG positive. No patient exhibited bleeding tendencies, and 3 out of 28 had low platelet counts. Muscle weakness was more pronounced, and summed MMT and grip power significantly lower, in PA-IgG-positive patients than in PA-IgG-negative patients. Of 19 patients, 7, 4, and 3 who underwent a sleep study had mild, moderate, and severe sleep apnea, respectively, and three started continuous positive airway pressure (CPAP). The respiratory disturbance index was not significantly correlated with physical evaluation items or forced vital capacity. All patients underwent ECG, 32 underwent cardiac ultrasound, and 25 underwent Holter ECG. No disease-specific cardiac involvement was noted, no serial changes during the follow-up period were observed for ECG and echocardiography, and none of the patients required therapy for cardiac abnormalities. DISCUSSION: PA-IgG is a potential disease biomarker in GNE myopathy patients, although its significance needs to be clarified. While none of the patients in this study experienced cardiomyopathy or arrythmia due to myopathy, sleep apnea was identified as a frequent complication.
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Miopatias Distais , Doenças Musculares , Síndromes da Apneia do Sono , Trombocitopenia , Humanos , Complexos Multienzimáticos , Doenças Musculares/diagnóstico , Síndromes da Apneia do Sono/diagnósticoRESUMO
Unilateral spatial neglect (USN) may lead to poor functional rehabilitation outcomes. However, studies investigating the rehabilitation outcomes of right-sided USN are lacking. We aimed to investigate (1) the clinical impacts of USN, including right-sided USN, for stroke patients in sub-acute rehabilitation, and (2) evaluate the differences in clinical characteristics and rehabilitation outcomes between right- and left-sided USN patients. We retrospectively screened the medical records of 297 inpatients at the Tokyo-Bay Rehabilitation Hospital who experienced a cerebrovascular accident with supratentorial lesions between January 1st, 2014 and December 31st, 2016. We performed independent multiple regression analysis in patients with left and right hemisphere damage. The Behavioral Inattention Test was a significant independent variable for predicting the motor, cognitive, and total functional independence measure (FIM), compared to the Stroke Impairment Assessment Set and Mini-Mental State Examination. USN affects motor FIM recovery more than cognitive FIM recovery regardless of the damaged hemisphere. Our study results confirm that both right- and left-sided USN influence the functional recovery of stroke patients. USN occurs, slightly less frequently, following a left hemisphere stroke. However, USN negatively affected rehabilitation outcomes, regardless of the neglected side. Therefore, USN treatment is necessary for patients with left and right hemisphere damage.
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Lateralidade Funcional , Transtornos da Percepção , Reabilitação do Acidente Vascular Cerebral , Acidente Vascular Cerebral , Lateralidade Funcional/fisiologia , Humanos , Transtornos da Percepção/etiologia , Transtornos da Percepção/fisiopatologia , Transtornos da Percepção/psicologia , Recuperação de Função Fisiológica , Estudos Retrospectivos , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/fisiopatologia , Acidente Vascular Cerebral/psicologiaRESUMO
BACKGROUND: Motor planning, imagery or execution is associated with event-related desynchronization (ERD) of mu rhythm oscillations (8-13 Hz) recordable over sensorimotor areas using electroencephalography (EEG). It was shown that motor imagery involving distal muscles, e.g. finger movements, results in contralateral ERD correlating with increased excitability of the contralateral corticospinal tract (c-CST). Following the rationale that purposefully increasing c-CST excitability might facilitate motor recovery after stroke, ERD recently became an attractive target for brain-computer interface (BCI)-based neurorehabilitation training. It was unclear, however, whether ERD would also reflect excitability of the ipsilateral corticospinal tract (i-CST) that mainly innervates proximal muscles involved in e.g. shoulder movements. Such knowledge would be important to optimize and extend ERD-based BCI neurorehabilitation protocols, e.g. to restore shoulder movements after stroke. Here we used single-pulse transcranial magnetic stimulation (TMS) targeting the ipsilateral primary motor cortex to elicit motor evoked potentials (MEPs) of the trapezius muscle. To assess whether ERD reflects excitability of the i-CST, a correlation analysis between between MEP amplitudes and ipsilateral ERD was performed. METHODS: Experiment 1 consisted of a motor execution task during which 10 healthy volunteers performed elevations of the shoulder girdle or finger pinching while a 128-channel EEG was recorded. Experiment 2 consisted of a motor imagery task during which 16 healthy volunteers imagined shoulder girdle elevations or finger pinching while an EEG was recorded; the participants simultaneously received randomly timed, single-pulse TMS to the ipsilateral primary motor cortex. The spatial pattern and amplitude of ERD and the amplitude of the agonist muscle's TMS-induced MEPs were analyzed. RESULTS: ERDs occurred bilaterally during both execution and imagery of shoulder girdle elevations, but were lateralized to the contralateral hemisphere during finger pinching. We found that trapezius MEPs increased during motor imagery of shoulder elevations and correlated with ipsilateral ERD amplitudes. CONCLUSIONS: Ipsilateral ERD during execution and imagery of shoulder girdle elevations appears to reflect the excitability of uncrossed pathways projecting to the shoulder muscles. As such, ipsilateral ERD could be used for neurofeedback training of shoulder movement, aiming at reanimation of the i-CST.
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Eletroencefalografia , Músculo Esquelético/fisiologia , Rede Nervosa/fisiologia , Ombro/fisiologia , Músculos Superficiais do Dorso/fisiologia , Adulto , Interfaces Cérebro-Computador , Sincronização de Fases em Eletroencefalografia , Eletromiografia , Potencial Evocado Motor/fisiologia , Feminino , Dedos/fisiologia , Lateralidade Funcional/fisiologia , Humanos , Imagens, Psicoterapia , Masculino , Córtex Motor/fisiologia , Músculo Esquelético/inervação , Ombro/inervação , Músculos Superficiais do Dorso/inervação , Estimulação Magnética Transcraniana , Adulto JovemRESUMO
Supraspinal excitability and sensory input may play an important role for the modulation of spinal inhibitory interneurons and functional recovery among patients with incomplete spinal cord injury (SCI). Here, we investigated the effects of anodal transcranial direct current stimulation (tDCS) combined with patterned electrical stimulation (PES) on spinal inhibitory interneurons in patients with chronic incomplete SCI and in healthy individuals. Eleven patients with incomplete SCI and ten healthy adults participated in a single-masked, sham-controlled crossover study. PES involved stimulating the common peroneal nerve with a train of ten 100 Hz pulses every 2 s for 20 min. Anodal tDCS (1 mA) was simultaneously applied to the primary motor cortex that controls the tibialis anterior muscle. We measured reciprocal inhibition and presynaptic inhibition of a soleus H-reflex by stimulating the common peroneal nerve prior to tibial nerve stimulation, which elicits the H-reflex. The inhibition was assessed before, immediately after, 10 min after and 20 min after the stimulation. Compared with baseline, simultaneous application of anodal tDCS with PES significantly increased changes in disynaptic reciprocal inhibition and long-latency presynaptic inhibition in both healthy and SCI groups for at least 20 min after the stimulation (all, p < 0.001). In patients with incomplete SCI, anodal tDCS with PES significantly increased the number of ankle movements in 10 s at 20 min after the stimulation (p = 0.004). In conclusion, anodal tDCS combined with PES could induce spinal plasticity and improve ankle movement in patients with incomplete SCI.
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Tornozelo/fisiologia , Reflexo H/fisiologia , Interneurônios/fisiologia , Córtex Motor/fisiologia , Inibição Neural/fisiologia , Plasticidade Neuronal/fisiologia , Nervo Fibular/fisiologia , Traumatismos da Medula Espinal/reabilitação , Estimulação Transcraniana por Corrente Contínua/métodos , Estimulação Elétrica Nervosa Transcutânea/métodos , Adulto , Humanos , Masculino , Pessoa de Meia-Idade , Nervo Fibular/fisiopatologiaRESUMO
BACKGROUND: Assessment of physical frailty is important among elderly with severe aortic stenosis (AS) when considering treatment. AIMS: We aimed to: (1) investigate the prevalence of physical frailty in older people with severe AS and (2) examine factors related to physical frailty. METHODS: A total of 125 consecutive elderly AS patients (mean age 84.6 ± 4.4 year) were enrolled. Physical frailty was defined as scoring ≤8 points on the short physical performance battery (SPPB). Factors likely related to physical frailty, including cardiac function, nutritional and metabolic status, kidney function, medical history, and comorbidities, were evaluated. Logistic regression analyses were used to examine which factors were related to physical frailty. RESULTS: Physical frailty was prevalent in 38.4 %. After sex and age adjusted, the following were significantly related to physical frailty: LVEF (adjusted OR per 10 % decrease: 1.39, p < 0.05), the Mini Nutritional Assessment-Short Form (adjusted OR per 1 point decrease: 1.21, p < 0.05), serum albumin (adjusted OR per 1 g/dL decrease: 2.64, p < 0.05), HDL-C (adjusted OR per 10 mg/dL decrease: 1.52, p < 0.01), eGFR (adjusted OR per 10 mL/min decrease: 1.59, p < 0.05), grip strength (adjusted OR per 10 kg decrease: 3.60, p < 0.01), coronary heart disease (adjusted OR: 2.78, p < 0.01), cerebrovascular disease (adjusted OR: 6.06, p < 0.01), and musculoskeletal disorders (adjusted OR: 3.28, p < 0.01). CONCLUSIONS: The prevalence of physical frailty is high and related to nutritional status, comorbidities, and cardiac status.
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Estenose da Valva Aórtica , Idoso Fragilizado/estatística & dados numéricos , Idoso de 80 Anos ou mais , Estenose da Valva Aórtica/diagnóstico , Estenose da Valva Aórtica/epidemiologia , Estenose da Valva Aórtica/fisiopatologia , Transtornos Cerebrovasculares/epidemiologia , Comorbidade , Feminino , Testes de Função Cardíaca/métodos , Humanos , Japão/epidemiologia , Testes de Função Renal/métodos , Masculino , Doenças Musculoesqueléticas/epidemiologia , Estado Nutricional/fisiologia , Prevalência , Índice de Gravidade de DoençaRESUMO
We developed a computerised test to evaluate unilateral spatial neglect (USN) using a touchscreen display, and estimated the spatial and temporal patterns of visual search in USN patients. The results between a viewer-centered USN patient and a stimulus-centered USN patient were compared. Two right-brain-damaged patients with USN, a patient without USN, and 16 healthy subjects performed a simple cancellation test, the circle test, a visuomotor search test, and a visual search test. According to the results of the circle test, one USN patient had stimulus-centered neglect and a one had viewer-centered neglect. The spatial and temporal patterns of these two USN patients were compared. The spatial and temporal patterns of cancellation were different in the stimulus-centered USN patient and the viewer-centered USN patient. The viewer-centered USN patient completed the simple cancellation task, but paused when transferring from the right side to the left side of the display. Unexpectedly, this patient did not exhibit rightward attention bias on the visuomotor and visual search tests, but the stimulus-centered USN patient did. The computer-based assessment system provided information on the dynamic visual search strategy of patients with USN. The spatial and temporal pattern of cancellation and visual search were different across the two patients with different subtypes of neglect.
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Atenção , Transtornos da Percepção/classificação , Percepção Espacial , Acidente Vascular Cerebral/fisiopatologia , Idoso , Estudos de Casos e Controles , Diagnóstico por Computador , Feminino , Lateralidade Funcional , Humanos , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos , Transtornos da Percepção/diagnóstico , Transtornos da Percepção/etiologia , Transtornos da Percepção/fisiopatologia , Análise Espaço-Temporal , Acidente Vascular Cerebral/complicações , Percepção VisualRESUMO
Unilateral spatial neglect (USN) is a symptom of unilateral brain damage resulting in failure to report sensory phenomena in the contra-lesional space. It is associated with motor impairment as well as sensory deficits. Recent research suggests that USN, may be caused by a disruption in the interhemispheric balance of the visual attention network. Based on this hypothesis, non-invasive brain stimulation (NIBS), such as repetitive transcranial magnetic stimulation (rTMS) and transcranial direct current stimulation (tDCS), is utilized in the rehabilitation of USN patients. Presently, inhibitory stimulation by continuous theta burst stimulation (cTBS) on contra-lesional parietal cortex are believed to be the most promising method. Conversely, compensation by attentional network of the non-lesioned hemisphere plays an important role in the recovery of USN. Recent imaging studies revealed that functional and structural connectivity of attentional networks within a lesioned hemisphere and between lesioned and non-lesioned hemispheres affects spontaneous recovery and effectiveness of rehabilitation approach such as prism adaptation therapy. These findings are useful in elucidating the pathophysiology of USN and predicting functional outcome. Furthermore, we hope that understanding the pathophysiology will enable the development of new rehabilitation strategies and appropriate treatment selection.
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Transtornos da Percepção , Estimulação Magnética Transcraniana , Humanos , Transtornos da Percepção/reabilitação , Transtornos da Percepção/fisiopatologia , Transtornos da Percepção/terapia , Transtornos da Percepção/etiologia , Estimulação Transcraniana por Corrente Contínua , Atenção/fisiologiaRESUMO
Background: Interventions and care that can evoke positive emotions and reduce apathy or agitation are important for people with dementia. In recent years, socially assistive robots used for better dementia care have been found to be feasible. However, the immediate responses of people with dementia when they are given multiple sensory modalities from socially assistive robots have not yet been sufficiently elucidated. Objective: This study aimed to quantitatively examine the immediate emotional responses of people with dementia to stimuli presented by socially assistive robots using facial expression analysis in order to determine whether they elicited positive emotions. Methods: This pilot study adopted a single-arm interventional design. Socially assistive robots were presented to nursing home residents in a three-step procedure: (1) the robot was placed in front of participants (visual stimulus), (2) the robot was manipulated to produce sound (visual and auditory stimuli), and (3) participants held the robot in their hands (visual, auditory, and tactile stimuli). Expression intensity values for "happy," "sad," "angry," "surprised," "scared," and "disgusted" were calculated continuously using facial expression analysis with FaceReader. Additionally, self-reported feelings were assessed using a 5-point Likert scale. In addition to the comparison between the subjective and objective emotional assessments, expression intensity values were compared across the aforementioned 3 stimuli patterns within each session. Finally, the expression intensity value for "happy" was compared between the different types of robots. Results: A total of 29 participants (mean age 88.7, SD 6.2 years; n=27 female; Japanese version of Mini-Mental State Examination mean score 18.2, SD 5.1) were recruited. The expression intensity value for "happy" was the largest in both the subjective and objective assessments and increased significantly when all sensory modalities (visual, auditory, and tactile) were presented (median expression intensity 0.21, IQR 0.09-0.35) compared to the other 2 patterns (visual alone: median expression intensity 0.10, IQR 0.03-0.22; P<.001; visual and auditory: median expression intensity 0.10, IQR 0.04-0.23; P<.001). The comparison of different types of robots revealed a significant increase when all stimuli were presented by doll-type and animal-type robots, but not humanoid-type robots. Conclusions: By quantifying the emotional responses of people with dementia, this study highlighted that socially assistive robots may be more effective in eliciting positive emotions when multiple sensory stimuli, including tactile stimuli, are involved. More studies, including randomized controlled trials, are required to further explore the effectiveness of using socially assistive robots in dementia care.
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Demência , Robótica , Humanos , Feminino , Idoso de 80 Anos ou mais , Projetos Piloto , Emoções/fisiologia , FelicidadeRESUMO
We estimated the severity of cerebellar ataxia by analyzing gait rhythm. We measured the step times in patients with pure cerebellar ataxia and healthy controls and then analyzed the distribution of the ratios of adjacent times. Gait rhythm displayed the best adaptation when expressed as the sum of the power law and lognormal distributions in both groups, and the groups could be distinguished by the exponent of the power law distribution, reflecting the fractal property of gait rhythm. Gait rhythm might reflect different features of impairment in patients with cerebellar ataxia, making it a useful continuous scale for cerebellar ataxia.
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Objectives: Some upper-limb function assessments can evaluate treatments in the non-ambulatory stage of Duchenne muscular dystrophy (DMD). The Functional Classification of the Upper Extremities (FCUE) was developed for DMD in Japan. The FCUE is easier to use than the Performance of Upper Limb (PUL) and is more detailed than the Brooke Upper Extremity Scale. This study aimed to determine the concurrent validity of FCUE with other methods of assessment for DMD. Methods: This retrospective study reviewed the medical records of 39 boys with DMD from the National Center of Neurology and Psychiatry to evaluate the concurrent validity of the FCUE and PUL using non-parametric Spearman rank correlation (ρ). We also determined the concurrent validity of the Brooke Upper Extremity Scale and PUL for comparison. Results: The ρ value between the FCUE and PUL was -0.914 (P<0.001). The FCUE showed robust concurrent validity with the PUL. That correlation between the FCUE and Brooke Upper Extremity Scale gave a ρ value of -0.854 (P<0.001). Conclusions: The FCUE had a higher concurrent validity with the PUL than with the Brooke Upper Extremity Scale. The FCUE is considered a valid assessment tool of upper-limb function in boys with DMD. Selecting the best assessment method depends on the severity of the patient's condition and a balance between assessment accuracy and evaluation time.
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A sensory trick is a specific maneuver that temporarily improves focal dystonia. We describe a case of musician's dystonia in the right-hand fingers of a patient, who showed good and immediate improvement after using an electrical stimulation-mimicking sensory trick. A 49-year-old professional guitarist presented with chronic involuntary flexion of the right-hand third and fourth fingers that occurred during guitar performances. Electrical stimulation with a frequency of 40 Hz and an intensity of 1.5 times the sensory threshold was administered on the third and fourth fingernails of the right hand, which facilitated fluent guitar playing. While he played guitar with and without electrical stimulation, we measured the surface electromyograms (sEMG) of the right extensor digitorum and flexor digitorum superficialis muscles to evaluate the sensory-trick-like effects of electrical stimulation. This phenomenon can offer clues for developing electrical stimulation-based treatment devices for focal dystonia. Electrical stimulation has the advantage that it can be turned off to avoid habituation. Moreover, the device is easy to use and portable. These findings warrant further investigation into the use of sensory stimulation for treating focal dystonia.
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Objectives: This study investigated the transition patterns of activities of daily living (ADL) status based on the Functional Independence Measure (FIM) motor and cognitive items in patients who experienced subacute stroke. Methods: In this single-site, retrospective investigation, 1592 FIM samples were collected during the hospitalization of 373 stroke patients who were admitted between April 2018 and March 2020. FIM item levels were transformed from seven to three (FIM1-2, Complete Dependence; FIM3-5, Modified Dependence; FIM6-7, Independence). FIM samples were classified by latent class modeling into six latent ADL states based on the independence levels of FIM motor and cognitive items. We created an ADL status transition diagram based on the FIM sample's probability of belonging to each status at different hospitalization timepoints. Results: Transition diagrams for each ADL status at admission revealed distinct patterns. In two ADL statuses for which patients required full assistance in FIM motor items, the patients remained motor-dependent without achieving independence on discharge. In contrast, patients in transition from the other four ADL statuses largely achieved independence in motor items by the time of discharge. The time required to reach higher ADL status varied according to the initial ADL status at admission; the slowest improvement was observed in statuses initially classified as needing the most assistance, whereas many patients achieved transition within 3 months from admission. Conclusions: Based on the characteristics of patient ADL status and timing of its changes, the classification of ADL status and visualization of ADL transition can contribute to improved treatment.
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BACKGROUND: Real-world data have shown variability in treatment responses to nusinersen in spinal muscular atrophy (SMA). We investigated whether the magnitude of muscle impairment assessed by magnetic resonance imaging (MRI) at baseline can predict the treatment response. METHODS: We retrospectively assessed the clinical data in relevance to the thigh and pelvic MRI taken before the nusinersen treatment. A total of 16 patients with SMA types 2 and 3 (age = mean [SD]; 9.2 [4.6] year) receiving nusinersen treatment were enrolled. The T1-weighted MRI images of the pelvis and thigh were scored for muscle fatty infiltration and atrophy. The minimally clinically important difference (MCID) was considered as gaining at least 3 points of Hammersmith Functional Motor Scale-Expanded (HFMSE) from baseline. RESULTS: Of these 16 individuals, 14 had been treated for at least 15 months with baseline data. At 15 months, seven individuals obtained MCID in HFMSE. Baseline muscle MRI score could not differentiate the two groups; however, individuals who obtained MCID had significantly less severe scoliosis. In addition, there was a significant and negative relationship between baseline MRI score and the change of score in HFMSE after 15 months of treatment. Further, baseline Cobb angle along with MRI score also indicated the correlation to the degree of change in motor function. CONCLUSION: The degree of muscle damage may confer the variability in response to nusinersen in SMA types 2 and 3. Muscle MRI score along with the severity of scoliosis assessed at baseline may help to predict the motor function change.
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Atrofia Muscular Espinal , Escoliose , Atrofias Musculares Espinais da Infância , Humanos , Estudos Retrospectivos , Atrofia Muscular Espinal/diagnóstico por imagem , Atrofia Muscular Espinal/tratamento farmacológico , Músculos , Imageamento por Ressonância MagnéticaRESUMO
Genu recurvatum (knee hyperextension) is a common problem after stroke. It is important to promote the coordination between knee and ankle movements during gait; however, no study has investigated how multi-joint assistance affects genu recurvatum. We are developing a gait training technique that uses robotized knee-ankle-foot orthosis (KAFO) to assists the knee and ankle joints simultaneously. This report aimed to investigate the safety of robotized KAFO-assisted gait training (Experiment 1) and a clinical trial to treat genu recurvatum in a patient with stroke (Experiment 2). Six healthy participants and eight patients with chronic stroke participated in Experiment 1. They received robotized KAFO-assisted gait training for one or 10 sessions. One patient with chronic stroke participated in Experiment 2 to investigate the effect of robotized KAFO-assisted gait training on genu recurvatum. The patient received the training for 30 min/day for nine days. The robot consisted of KAFO and an attached actuator of four pneumatic artificial muscles. The assistance parameters were adjusted by therapists to prevent genu recurvatum during gait. In Experiment 2, we evaluated the knee joint angle during overground gait, Fugl-Meyer Assessment of lower extremity (FMA-LE), modified Ashworth scale (MAS), Gait Assessment and Intervention Tool (G.A.I.T.), 10-m gait speed test, and 6-min walk test (6MWT) before and after the intervention without the robot. All participants completed the training in both experiments safely. In Experiment 2, genu recurvatum, FMA-LE, MAS, G.A.I.T., and 6MWT improved after robotized KAFO-assisted gait training. The results indicated that the multi-joint assistance robot may be effective for genu recurvatum after stroke.
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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. Results: Thirty-two patients with DMD were offered the standing program with KAFO; 12 continued the program until the age of 15 years (complete group) and 20 discontinued the program before the age of 15 years (incomplete group). The non-standing program group included 9 patients. The standing program with KAFO was significantly associated with the Cobb angle at the age of 15 years after adjustment for the duration of corticosteroid use and DMD mutation type (P=0.0004). At the age of 15 years, significant correlations were found between the ankle dorsiflexion range of motion (ROM) and non-ambulatory period (P=0.0010), non-ambulatory period and Cobb angle (P<0.0001), Cobb angle and percent predicted forced vital capacity (P=0.0004), and ankle dorsiflexion ROM and Cobb angle (P=0.0066). In the complete group, the age at ambulation loss (log-rank P=0.0015), scoliosis progression (log-rank P=0.0032), and pulmonary dysfunction (log-rank P=0.0006) were significantly higher than in the non-standing program group. Conclusions: The early introduction of a standing program for DMD patients may prolong the ambulation period and slow the progression of scoliosis and pulmonary dysfunction.
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BACKGROUND: Motor recovery varies across post-stroke individuals, some of whom require a better rehabilitation strategy. We hypothesized that macrostructural neuroplasticity of the motor control network including the cerebellum might underlie individual differences in motor recovery. Objectives. To gain insight into the macrostructural neuroplasticity after stroke, we examined 52 post-stroke individuals using both the Fugl-Meyer assessment and structural magnetic resonance imaging. METHODS: We performed voxel-based lesion symptom mapping and cross-sectional voxel-based morphometry to correlate the motor scores with the lesion location and the gray matter volume (GMV), respectively. Longitudinal data were available at ~8 and/or 15 weeks after admission from 43 individuals with supratentorial lesions. We performed a longitudinal VBM analysis followed by a multiple regression analysis to correlate between the changes of the motor assessment scores and those of GMV overtime. RESULTS: We found a cross-sectional correlation of residual motor functioning with GMV in the ipsilesional cerebellum and contralesional parietal cortex. Longitudinally, we found increases in GMV in the ipsilesional supplementary motor area, and the ipsilesional superior and inferior cerebellar zones, along with a GMV decrease in the ipsilesional thalamus. The motor recovery was correlated with the GMV changes in the superior and inferior cerebellar zones. The regaining of upper-limb motor functioning was correlated with the GMV changes of both superior and inferior cerebellum while that of lower-limb motor functioning with the GMV increase of the inferior cerebellum only. CONCLUSIONS: The present findings support the hypothesis that macrostructural cerebellar neuroplasticity is correlated with individual differences in motor recovery after stroke.
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Acidente Vascular Cerebral , Humanos , Estudos Transversais , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/patologia , Cerebelo/diagnóstico por imagem , Substância Cinzenta/diagnóstico por imagem , Substância Cinzenta/patologia , Imageamento por Ressonância Magnética/métodosRESUMO
The prevailing theory concerning the pathophysiology of unilateral spatial neglect is that it is caused by an interhemispheric imbalance in attention networks. Previous studies have demonstrated that repetitive transcranial magnetic stimulation or transcranial direct current stimulation (tDCS) delivered over the right posterior parietal cortex can induce transitory neglect-like deficits in healthy individuals. We examined whether right cathodal and left anodal tDCS delivered over the posterior parietal cortex could produce neglect-like deficits and change the resting-state functional connectivity (rsFC) of attention networks. We found that the reaction time for targets in the left hemifield was significantly prolonged during two different types of visual search tasks, and rsFC of the attention networks was altered by tDCS. Furthermore, the change in the reaction times for the left visual target in the two different tasks significantly correlated with the change in the rsFC of either the right dorsal attention network (DAN) or right ventral attention network (VAN) based on the tasks. These results suggest that tDCS delivered to the posterior parietal cortex bilaterally induced neglect-like deficits by altering the connectivity of the attentional networks through excitability changes in the cortical area under the electrode. The results of this study are consistent with the hypothesis that the cause of neglect is the interhemispheric imbalance of attention networks. This is the first study to demonstrate that local cortical stimulation can induce changes not only in the local brain function but also in the cortical networks in healthy individuals.
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Transtornos da Percepção , Estimulação Transcraniana por Corrente Contínua , Humanos , Estimulação Transcraniana por Corrente Contínua/efeitos adversos , Lateralidade Funcional/fisiologia , Lobo Parietal/fisiologia , Transtornos da Percepção/etiologia , Estimulação Magnética TranscranianaRESUMO
Background: A number of clinical trials targeting GNE myopathy patients have been conducted. However, useful clinical parameters for postmarketing surveillance and long-term clinical observation have not yet been established. Objective: We conducted a 5-year observational follow-up natural history study to identify evaluation parameters, which may be useful for the long-term observation of GNE myopathy patients. Methods: Thirty-three genetically confirmed GNE myopathy patients were recruited and evaluated at study entry (baseline) and yearly in a 5-year follow-up. Hand-held dynamometer measurements of knee extension strength, grip power and pinch power, summed Manual Muscle Testing (MMT) score of 17 muscles, Gross Motor Function Measure (GMFM), 6 min walk test, percent vital capacity and percent forced vital capacity (%FVC), lean body mass (whole body, arms and legs), creatine kinase, Barthel Index, modified Rankin Scale and 36-item Short Form Survey national standard scores were examined. Results: Of the 33 patients, 22 (66%) completed evaluations for the entire 5-year follow-up period. These patients had a significant reduction in summed MMT score (p=0.005), GMFM (p=0.005), pinch power (p<0.001) and %FVC (p<0.001) at the fifth year evaluation relative to baseline. Among these parameters, summed MMT score, GMFM, pinch power and %FVC showed significant changes even in non-ambulant patients. Conclusions: MMT, GMFM, pinch power and %FVC are useful parameters for the long-term evaluation of GNE myopathy patients.
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Objectives: Using Functional Independence Measure (FIM) records, this study used latent class analysis (LCA) to clarify the structure of activities of daily living (ADL) status in patients following stroke. Methods: In this retrospective, single-center study, we extracted the medical records of patients with stroke who were admitted to a rehabilitation hospital in Japan between April 2018 and March 2020. LCA was used to determine classes of ADL status based on response patterns in FIM items converted from the original seven levels to three levels: Complete Dependence, FIM1-2; Modified Dependence, FIM3-5; Independence, FIM6-7. We compared the length of stay and discharge destinations among subgroups of patients with different ADL status at admission. Results: From 373 patients, 1592 FIM records were analyzed. These were classified into six ADL status classes based on "Complete Dependence," "Modified Dependence," and "Independence" in the motor and cognitive domains. Significant differences were observed among the six admission ADL subgroups for the length of stay (median values in patient subgroups based on admission ADL status: 126, 146, 90, 65, 44, and 29 days in the Motor Complete/Cognitive Complete, Motor Complete/Cognitive Modified, Motor Modified/Cognitive Modified, Motor Modified/Cognitive Independent, Motor Independent/Cognitive Modified, and Motor Independent/Cognitive Independent groups, respectively) and discharge destinations (patients discharged home: 27%, 62%, 81%, 92%, 95%, and 98%, respectively, and to acute care hospitals: 18%, 14%, 8%, 8%, 2%, and 2%, respectively). Conclusions: LCA successfully stratified ADL status in patients with stroke undergoing rehabilitation and may aid in determining an appropriate treatment regimen.