Demonstration of CameraMouseAI: A Head-Based Mouse-Control System for People with Severe Motor Disabilities

Using a transcranial Doppler blood flowmeter, the blood flow velocity (BFV) ratio of the middle cerebral artery (MCA) to the basilar artery (BA) was investigated in 12 patients with severe motor and intellectual disability syndrome. The BFV of the MCA was also investigated in 58 handicapped children, classified according to the severity of their motor and intellectual disability. The ratio of the MCA to the BA was lower by 2 SD from the mean of our previously reported standard value in 8 out of the 12 cases with severe motor and intellectual disability syndrome, suggesting a more profound decrease in the level of brain activity in the MCA area than that of the BA area. The BFV of the MCA mainly decreased in cases belonging to the category of the most severe motor disability (bed-ridden). Hence, it is suggested that motor disability is the main factor related to the decrease in the BFV of the MCA.

ObjectiveTo determine bone quality in adults with severe motor and intellectual disabilities.DesignA retrospective cohort study.PatientsBone quality of 60 patients with severe motor and intellectual disabilities (28 men, 32 women; mean age 57 years) at a long-term care facility for adults was examined retrospectively.MethodsQuantitative ultrasonography was used to measure the stiffness index, T-score and Z-score of the calcaneus. A multiple linear regression model, including sex, age, anti-epileptic drug use, tube-feeding status, and current and peak physical abilities, was used to identify significant predictors of T-scores.ResultsQuantitative ultrasonography revealed that all patients had lower bone quality (based on T-scores, Z-scores, and stiffness index), and all patients had T-scores with standard deviations (SD) below 1.8. Current physical ability, age, and anti-epileptic drug use were significant factors in T-score determination, while tube-feeding and peak physical ability were not. The ability to walk without assistance was the most significant predictor in quantitative ultrasonography.ConclusionSeverely low bone quality is observed in patients with severe motor and intellectual disabilities; and it is strongly associated with current physical activity. It is important that patients with severe motor and intellectual disabilities preserve their physical abilities to prevent osteoporosis- related fractures.LAY ABSTRACTFractures are common in adults with severe motor and intellectual disabilities. This study assessed the bone quality of 60 individuals from young adult to advanced age. All patients had low bone quality from a young age, and current physical ability, age, and anti-epileptic drug use were factors affecting bone quality, while tube-feeding and peak physical ability were not. Severely low bone quality in patients with severe motor and intellectual disabilities is strongly associated with current physical activity levels. It is very important for patients with severe motor and intellectual disabilities to increase and preserve their physical activities and abilities, respectively, to prevent osteoporosis-related fractures.

The utility of bioelectrical impedance analysis to assess nutritional status of patients with severe motor and intellectual disabilities.

BackgroundChildren with profound intellectual and multiple disabilities (PIMD) or severe motor and intellectual disabilities (SMID) only communicate through movements, vocalizations, body postures, muscle tensions, or facial expressions on a pre- or protosymbolic level. Yet, to the best of our knowledge, there are few systems developed to specifically aid in categorizing and interpreting behaviors of children with PIMD or SMID to facilitate independent communication and mobility. Further, environmental data such as weather variables were found to have associations with human affects and behaviors among typically developing children; however, studies involving children with neurological functioning impairments that affect communication or those who have physical and/or motor disabilities are unexpectedly scarce.ObjectiveThis paper describes the design and development of the ChildSIDE app, which collects and transmits data associated with children’s behaviors, and linked location and environment information collected from data sources (GPS, iBeacon device, ALPS Sensor, and OpenWeatherMap application programming interface [API]) to the database. The aims of this study were to measure and compare the server/API performance of the app in detecting and transmitting environment data from the data sources to the database, and to categorize the movements associated with each behavior data as the basis for future development and analyses.MethodsThis study utilized a cross-sectional observational design by performing multiple single-subject face-to-face and video-recorded sessions among purposively sampled child-caregiver dyads (children diagnosed with PIMD/SMID, or severe or profound intellectual disability and their primary caregivers) from September 2019 to February 2020. To measure the server/API performance of the app in detecting and transmitting data from data sources to the database, frequency distribution and percentages of 31 location and environment data parameters were computed and compared. To categorize which body parts or movements were involved in each behavior, the interrater agreement κ statistic was used.ResultsThe study comprised 150 sessions involving 20 child-caregiver dyads. The app collected 371 individual behavior data, 327 of which had associated location and environment data from data collection sources. The analyses revealed that ChildSIDE had a server/API performance >93% in detecting and transmitting outdoor location (GPS) and environment data (ALPS sensors, OpenWeatherMap API), whereas the performance with iBeacon data was lower (82.3%). Behaviors were manifested mainly through hand (22.8%) and body movements (27.7%), and vocalizations (21.6%).ConclusionsThe ChildSIDE app is an effective tool in collecting the behavior data of children with PIMD/SMID. The app showed high server/API performance in detecting outdoor location and environment data from sensors and an online API to the database with a performance rate above 93%. The results of the analysis and categorization of behaviors suggest a need for a system that uses motion capture and trajectory analyses for developing machine- or deep-learning algorithms to predict the needs of children with PIMD/SMID in the future.

BackgroundOwing to recurrent aspiration pneumonia and airway stenosis secondary to thoracic deformities, tracheostomy or laryngotracheal separation are often necessary in children with severe motor and intellectual disabilities. However, these procedures are associated with the risks of tracheal stenosis due to tracheal granulation and tracheoinnominate artery fistula formation. We report a case of a child with severe motor and intellectual disabilities treated with an anterior mediastinal tracheostomy.Case presentationThe patient was a 15-year-old boy with severe motor and intellectual disabilities due to intractable epilepsy. Due to thoracic deformity and tracheomalacia, the patient had a flattened and narrowed trachea. Accordingly, laryngotracheal separation was performed 4 months before admission to avoid aspiration pneumonia. Due to a common cold, the patient required frequent sputum suctioning, which exacerbated the tracheal stenosis. Bronchoscopy revealed tracheal stenosis 4–5 cm caudal to the tracheostomy site, tracheal mucosal ulcers, and pulsation of the innominate artery on the anterior wall of the trachea. We performed an anterior mediastinum tracheostomy to release the tracheal stenosis and prevent tracheoinnominate artery fistula formation.ConclusionsAnterior mediastinal tracheostomy has several advantages. Including sufficient release of bony compression, release of tracheal hyperextension, and relief of tracheal and innominate artery contact ensures a cannula-free tracheostomy, and there is no need to dissect the brachiocephalic artery. It is the procedure of choice in cases of head and neck malignancies requiring extensive tracheal resection and could be a good surgical option for severe tracheal stenosis and tracheoinnominate artery fistula in children with severe motor and intellectual disabilities.

To investigate the longitudinal incidence of kidney/urinary stones in patients with severe motor and intellectual disabilities and explore health burden events in patients with stone formation. This was a retrospective, observational study. We identified patients with severe motor and intellectual disabilities who had the following: 1) admission to our hospital wards for >10 years; 2) two or more assessments for stone formation by ultrasonography or computed tomography; and 3) absence of kidney/urinary stones in the first imaging study. The Kaplan-Meier method was used to analyze the cumulative incidence of kidney/urinary stones. Recurrent urinary tract infections, hydronephrosis, renal dysfunction, and death were identified as health burdens. Among the 41 patients (19 men, 22 women; median age, 28 years; range, 8-50 years), stone formation was detected in 11 (27%) patients during the observation period. The cumulative incidence rate of stone formation was 9.8% (95% confidence interval, 3.8-23.9) and 18.7% (95% confidence interval, 9.2-35.7) at five and 10 years, respectively. Death was frequently observed in patients with stone formation; six (55%) of the 11 patients with stone formation died during the follow-up period; two (15%) died among the other 30 patients without stone formation. However, only one patient with stone formation died in a renal event; the causal relationship between the stone formation and the deaths was not clarified. The longitudinal incidence of kidney/urinary stones was higher in patients with severe motor and intellectual disabilities than in the general population. Considering the difficulty of patients with severe motor and intellectual disabilities in conveying their symptoms, regular assessment of the kidney using abdominal imaging may be recommended.

Effects of Ethyl Loflazepate for Children with Refractory Epilepsy Accompanied by Severe Motor and Intellectual Disabilities. 1 Hideaki Kanemura, 1 Masao Aihara, and 1 Shinpei Nakazawa ( 1 Department of Pediatrics, Faculty of Medicine, University of Yamanashi, Yamanashi, Japan ). Purpose: Ethyl lofrazepate has been used mainly as an antianxiety agent. Recently, ethyl lofrazepate has been reported to demonstrate good efficacy for acute epilepsy, seizure clusters, and serial seizures. Hypersecretion and hypersalivation are problematic adverse reactions in infants and children treated with benzodiazepine drugs. However, ethyl lofrazepate may have a lower incidence of hypersecretion and hypersalivation compared to other benzodiazepine drugs. We report the efficacy of ethyl lofrazepate in three children with refractory epilepsies accompanied by severe motor and intellectual disabilities. Methods: The subjects comprised three symptomatic localization-related epileptic patients aged 2–8 years with severe motor and intellectual disabilities. All patients had some difficulties in seizure control with standard antiepileptic drugs (AEDs). They had multiple seizure types, including simple partial seizures, complex partial seizures, tonic, myoclonic, and generalized tonic–clonic seizures. Prior to starting ethyl lofrazepate, they had difficulty in breathing due to hypersecretion and hypersalivation induced by benzodiazepine drugs. Parents and caregivers, including nursing staff, were requested to keep seizure diaries for seizure counts. The basic strategy was add-on therapy, in which ethyl lofrazepate was additionally given with standard doses of the AEDs. During the titration phase, concomitant benzodiazepine, if any, was gradually withdrawn and discontinued. Ethyl lofrazepate was started at a dose of 0.015 mg/kg and was increased to 0.05 mg/kg in the second week. Ethyl lofrazepate was taken twice a day in the morning and at night. Clinical laboratory tests including antiepileptic drug levels, blood chemistry, and hematology were performed at baseline, and 2, 4, 8, and 12 weeks after reaching the maximum dose of ethyl lofrazepate. EEGs were obtained from all patients at the same time points. Informed consent was obtained from the parents. Results: Seizures were reduced during the first 12 weeks of treatment with ethyl lofrazepate in all three patients (100%). Two patients became seizure free, and one patient demonstrated at least a 90% reduction. All seizure types were reduced by ethyl lofrazepate. The sample size was too small to determine if any particular seizure type was more affected than any other. The response to ethyl lofrazepate was observed within 6 weeks of initiating treatment. One patient had complete cessation of seizures at 2 weeks and another patient became seizure free at 4 weeks after reaching the maximum dose of ethyl lofrazepate. Epileptic discharges were suppressed in two cases and demonstrated at least 75% reduction in one case. No alteration in blood chemistry or hepatic function was observed in any patient. Blood concentrations of other AEDs were unaffected before and after therapy. No adverse reactions such as hypersecretion or hypersalivation were observed. Respiratory status improved markedly in all patients. There were no other adverse effects that required dose reduction of ethyl lofrazepate in all cases. None had a recurrence of seizures or adverse effects such as hypersecretion or hypersalivation at 6-, 8- and 9-month follow-up of ethyl lofrazepate therapy. Conclusion: The results of our prospective study indicate that ethyl lofrazepate is an effective and safe benzodiazepine for the treatment of refractory epilepsy with severe motor and intellectual disabilities. Dramatic improvement (over 90% reduction in seizure frequency, including complete cessation) was observed in all cases. Unlike other benzodiazepines, adverse effects, such as hypersecretion and hypersalivation, were less noticeable. Based on this study, we conclude that ethyl lofrazepate is an effective adjunctive antiepileptic drug for refractory epileptic children with severe motor and intellectual disabilities complicated by respiratory failure. Further studies are necessary to determine whether these effects are transient or permanent.

BackgroundRecognizing the predictors of disease relapses in patients with anti-aquaporin-4 antibody (AQP4-ab)-positive neuromyelitis optica spectrum disorder (NMOSD) is essential for individualized treatment strategy. We aimed to identify the factors that predicted relapses among patients with AQP4-ab-positive NMOSD, develop outcome prediction models, and validate them in a multicenter validation cohort.MethodsBetween January 2015 and December 2020, 820 patients with NMOSD were registered at Huashan Hospital. We retrospectively reviewed their medical records, and included 358 AQP4-ab-positive patients with 1135 treatment episodes. Univariate and multivariate analyses were used to explore the predictors of relapse, severe visual or motor disability during follow-up. A model predicting the 1- and 2-year relapse-free probability was developed and validated in an external validation cohort of 92 patients with 213 treatment episodes.ResultsLower serum AQP4-ab titer (< 1:100), higher Expanded Disability Status Scale (EDSS) score at onset (≥ 2.5), and use of intravenous methylprednisolone (IVMP) at the first attack predicted an overall lower annualized relapse rate. Older age (> 48 years), optic neuritis at onset, and higher onset EDSS score (≥ 2.5) significantly increased the risk for blindness, while IVMP at the first attack and maintenance therapy reduced the risk for blindness. Myelitis at onset increased the possibility of motor disability (EDSS ≥ 6.0), severe motor disability or death (EDSS ≥ 8.0), while maintenance therapy reduced these possibilities. Anderson and Gill model identified that the risk factors predicting recurrent relapses under certain treatment status were female gender, high AQP4-ab titer (≥ 1:100), previous attack under same therapy, lower EDSS score at treatment initiation (< 2.5), and no maintenance therapy or oral prednisone lasting less than 6 months. A nomogram using the above factors showed good discrimination and calibration abilities. The concordance indexes in the primary and validation cohort were 0.66 and 0.65, respectively.ConclusionThis study reports the demographic, clinical and therapeutic predictors of relapse, and severe visual or motor disability in NMOSD. Early identification of patients at risk of unfavorable outcomes is of paramount importance to inform treatment decisions.

Background: “Children with severe motor and intellectual disabilities” refers to children with markedly limited activity due to severe overlapping of physical and intellectual disabilities. The physical and mental burden placed on families raising severely disabled children, particularly the primary caregivers, is great in home settings. For families to effectively utilize services and over-come child rearing problems, the families themselves need the “strength” to cooperate with others for the purpose of raising a severely disabled child. The ultimate goal of family support is to enable such families to achieve satisfaction and self-growth in child rearing. Methods: We used a questionnaire to survey 75 primary caregivers to empirically elucidate the empowerment and positive feelings towards child rearing of families raising children with severe motor and intellectual disabilities and the related factors. The t-test and Spearman’s rank correlation coefficient were used to examine the association with bivariates. A multiple regression analysis was conducted for empowerment and positive feelings. Results: Results revealed that life events, livelihood, awareness of social support and the child’s sleep problems were factors related to empowerment. Of these, awareness of social support from outside of the family was found to contribute the most to empowerment. Furthermore, improvement and maintenance of positive feelings towards child rearing reaffirmed the existence of empowerment in addition to reducing negative feelings towards child rearing and ensuring social support. Conclusions: Raising children with severe motor and intellectual disabilities requires specialist knowledge and skills. Support from professionals to empower the entire family is therefore important in order to strengthen positive feelings towards child rearing.

BackgroundThere are few epidemiological data to support rehabilitation programs for cerebral palsy (CP). Scarce international studies described the developmental anomalies (DAs) among children with CP. To our knowledge, the Arab countries did not publish data regarding this topic. This study aimed to describe the percentage of DAs among children with CP and detect the association between clinical subtypes and impairment severity in children with various DAs. We collected registry data of 679 children with cerebral palsy, between 2014 and 2019, from Armed Forces Hospitals, Taif, Kingdom of Saudi Arabia (KSA). We recorded demographic, perinatal, postnatal, developmental anomalies, subtypes, and impairment characteristics. We utilized the chi-square test to calculate the differences between groups.ResultsWe reported significant differences between the children with and without anomalies regarding the percentages of consanguinity, preterm labor, low birth weight, and neonatal intensive care unit admission (P = 0.001, 0.002, 0.003, 0.005, respectively). Congenital dysplasia of the hip and hydrocephalus was the most frequent skeletal and nervous anomalies among children with DAs (19.1% and 12.8%, respectively). The spastic bilateral pattern was significantly higher among children with skeletal anomalies than the central nervous system/other groups (P < 0.001). The nervous anomalies group had higher frequencies of severe intellectual, motor, speech, and visual disabilities and a higher percentage of seizures than all other groups.ConclusionsThe frequency of children with anomalies in this study was comparable to previous studies. Children with CP and nervous system anomalies had more severe motor disabilities and associated impairments.

The purpose of this study was to investigate cortical interaction between brain regions in people with and without severe motor disability during brain–computer interface (BCI) operation through coherence analysis. Eighteen subjects, including six patients with cerebral palsy (CP) and three patients with amyotrophic lateral sclerosis (ALS), participated. The results showed (1) the existence of BCI performance difference caused by severe motor disability; (2) different coherence patterns between participants with and without severe motor disability during BCI operation and (3) effects of motor disability on cortical connections varying in the brain regions for the different frequency bands, indicating reduced cortical differentiation and specialisation. Participants with severe neuromuscular impairments, as compared with the able-bodied group, recruited more cortical regions to compensate for the difficulties caused by their motor disability, reflecting a less efficient operating strategy for the BCI task. This study demonstrated that coherence analysis can be applied to examine the ways cortical networks cooperate with each other during BCI tasks. Practitioner Summary: Few studies have investigated the electrophysiological underpinnings of differences in BCI performance. This study contributes by assessing neuronal synchrony among brain regions. Our findings revealed that severe motor disability causes more cortical areas to be recruited to perform the BCI task, indicating reduced cortical differentiation and specialisation.

Children with severe motor and intellectual disabilities experience chronic pain but cannot communicate verbally. However, no Japanese tool currently exists for assessing pain in this population. This study aimed to develop and evaluate the reliability and validity of a Japanese version of the Paediatric Pain Profile, which is a behavioral rating scale to assess pain in children with severe neurological disabilities. The sample comprised 30 children with severe motor and intellectual disabilities at three hospitals in Japan. Three specialist nurses rated low and high pain video scenes of the children (twice at 1-week intervals) using the Face, Legs, Activity, Cry, Consolability behavioral scale and a translated Japanese version of the Paediatric Pain Profile. On the basis of their ratings, we calculated the internal consistency, test–retest reliability, and intra- and inter-observer reliabilities of the Paediatric Pain Profile. Additionally, we assessed concurrent validity using the Face, Legs, Activity, Cry, Consolability behavioral scale and construct validity using low versus high pain scenes. Both internal consistency (low pain: alpha = 0.735; high pain: alpha = 0.928) and test–retest reliability (r = 0.846) of the Japanese version of the Paediatric Pain Profile were good. Intra-observer reliability was substantial (r = 0.748), whereas inter-observer reliability was only moderate (r = 0.529). However, the concurrent validity with Face, Legs, Activity, Cry, Consolability scores was good (r = 0.629) and construct validity was confirmed (p < 0.001). We confirmed the validity of the Japanese version of the Paediatric Pain Profile, but reliable pain assessment may require repeated ratings by the same person. To accurately assess pain in children with severe motor and intellectual disabilities, healthcare staff must be properly trained and become more skilled in using the Japanese version of the Paediatric Pain Profile.

Children with severe motor and intellectual disabilities experience chronic pain but cannot communicate verbally. However, no Japanese tool currently exists for assessing pain in this population. This study aimed to develop and evaluate the reliability and validity of a Japanese version of the Paediatric Pain Profile, which is a behavioral rating scale to assess pain in children with severe neurological disabilities. The sample comprised 30 children with severe motor and intellectual disabilities at three hospitals in Japan. Three specialist nurses rated low and high pain video scenes of the children (twice at 1-week intervals) using the Face, Legs, Activity, Cry, Consolability behavioral scale and a translated Japanese version of the Paediatric Pain Profile. On the basis of their ratings, we calculated the internal consistency, test-retest reliability, and intra- and inter-observer reliabilities of the Paediatric Pain Profile. Additionally, we assessed concurrent validity using the Face, Legs, Activity, Cry, Consolability behavioral scale and construct validity using low versus high pain scenes. Both internal consistency (low pain: alpha = 0.735; high pain: alpha = 0.928) and test-retest reliability (r = 0.846) of the Japanese version of the Paediatric Pain Profile were good. Intra-observer reliability was substantial (r = 0.748), whereas inter-observer reliability was only moderate (r = 0.529). However, the concurrent validity with Face, Legs, Activity, Cry, Consolability scores was good (r = 0.629) and construct validity was confirmed (p < 0.001). We confirmed the validity of the Japanese version of the Paediatric Pain Profile, but reliable pain assessment may require repeated ratings by the same person. To accurately assess pain in children with severe motor and intellectual disabilities, healthcare staff must be properly trained and become more skilled in using the Japanese version of the Paediatric Pain Profile.

Clinically important improvements in motor function are achievable during inpatient rehabilitation by stroke patients with severe motor disability: a prospective observational study.

Measuring waist circumference in disabled adults