Francis Meire Fávero

Mortality rates due to amyotrophic lateral sclerosis in São Paulo City from 2002 to 2006

OBJECTIVE To describe the mortality rates of amyotrophic lateral sclerosis (ALS) in the city of São Paulo as a function of demographics, year, and region. METHOD This was a retrospective descriptive study. Information was obtained from death certificates registered at the Program for the Improvement of Mortality Information, Municipal Health Department (PRO-AIM/SMS), coded as G12.2 according to International Classification of Diseases (ICD-10), from 2002 to 2006. RESULTS Over the studied time, were found 326 deaths (51.6% women, overall mean age of 64.1 years). Highest deaths percentages happened in those from 60 to 69 and 70 to 79 years and in white individuals. ALS mortality rates ranged 0.44/100,000 in 2002 and 0.76/100,000 in 2006. No significant changes overtime in administrative districts were found. CONCLUSION ALS mortality rates in São Paulo were lower in comparison to other countries, however any risk factor in our environment, lifestyle or genetic characteristics were found.

Relação entre degeneração do trato córtico-espinhal através de ressonância magnética e escala funcional (ALSFRS) em pacientes com esclerose lateral amiotrófica

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that affects the corticospinal tract. ALS functional rating scale (ALSFRS) is a questionnaire that quantifies motor deficits, while diffusion tensor imaging (DTI) evaluates the integrity of fibers through the fractional anisotropy (FA). In the present study, seven ALS patients were evaluated by ALSFRS and immediately submitted to DTI, getting FA values in the following regions: cerebral peduncle (PC), internal capsule (CI) and the white matter under the primary motor cortex (M1), secondary motor cortex (M2) and somesthetic cortex (SI). A control group was constituted by twelve healthy individuals. FA values in patients were significantly lower when compared with controls, with a tendency to higher reductions in the right hemisphere and more inferior regions. Interestingly, FA values were reduced in somesthetic area. No correlation was observed between symptoms duration and FA values. Despite the correlation observed between ALSFRS scores and degeneration in PC and CI, our results suggest that this subjective scale is not a good parameter for the evaluation of the structural damage in encephalic portions of the corticospinal tract.

The clinical relevance of timed motor performance in children with Duchenne muscular dystrophy

Abstract Background: The measurement of time and compensatory movements for functional tasks is not frequently used to evaluate children with Duchenne muscular dystrophy (DMD). As muscle weakness progresses, new synergies (compensatory movements) are selected to perform the tasks, demanding higher times. Objectives: The present study aimed to describe the timed motor performance of rising from the floor to standing, sitting down on the floor from standing, climbing up four steps and climbing down four steps 18 and 6 months prior to gait loss and to investigate possible relationships between these timed performances, the compensatory movements and the Vignos Scale (VS) scores. Method: Fourteen children with DMD (mean age: 9.6) were videotaped performing the tasks. Spearman correlation tests investigated the relationships between the times, compensatory movements (scored by FES-DMD) and VS. Results: The timed performance and the compensatory movements for rising from the floor, climbing up and climbing down steps varied broadly and were correlated to each other among patients with DMD at 18 and 6 months prior to gait loss. The relationship was not found for sitting on the floor. The timed performance and compensatory movements for climbing up and down steps also correlated to the VS. Conclusion: Rising from the floor, climbing up, and climbing down steps have some components in common, such as the demand for muscle strength and the recruitment of compensatory muscle synergies, as DMD progresses. To sit down on the floor, some children let themselves fall, resulting in a faster performance, but more compensatory movements.

Computer task performance by subjects with Duchenne muscular dystrophy

Aims Two specific objectives were established to quantify computer task performance among people with Duchenne muscular dystrophy (DMD). First, we compared simple computational task performance between subjects with DMD and age-matched typically developing (TD) subjects. Second, we examined correlations between the ability of subjects with DMD to learn the computational task and their motor functionality, age, and initial task performance. Method The study included 84 individuals (42 with DMD, mean age of 18±5.5 years, and 42 age-matched controls). They executed a computer maze task; all participants performed the acquisition (20 attempts) and retention (five attempts) phases, repeating the same maze. A different maze was used to verify transfer performance (five attempts). The Motor Function Measure Scale was applied, and the results were compared with maze task performance. Results In the acquisition phase, a significant decrease was found in movement time (MT) between the first and last acquisition block, but only for the DMD group. For the DMD group, MT during transfer was shorter than during the first acquisition block, indicating improvement from the first acquisition block to transfer. In addition, the TD group showed shorter MT than the DMD group across the study. Conclusion DMD participants improved their performance after practicing a computational task; however, the difference in MT was present in all attempts among DMD and control subjects. Computational task improvement was positively influenced by the initial performance of individuals with DMD. In turn, the initial performance was influenced by their distal functionality but not their age or overall functionality.

Technological Tools for Observational Evaluation - the Experience with the Software for Functional Evaluation Scale for Duchenne Muscular Dystrophy – A Pilot Study Software for Observational Evaluation

Systematic observation is an indispensable tool in clinical neurology evaluation, but data organization and record are extensive and time-consuming, requiring method and training. Reliable scales facilitate this task and technology can be decisive in the implementation of observational data routines. In this study, we aimed to show the experience of a software development to Original Research Article Albuquerque et al.; BJMMR, 16(11): 1-7, 2016; Article no.BJMMR.27482 2 optimize the application of a clinical observational scale. For this proposal it was necessary to consider the needs of the target population, text and image storing and reports generation, using computers with basic configuration, questionnaires and the measurement of time. The software allowed significant reduction in evaluation time and favored the cost-benefit of the task. The proposed methodology was adequate for this type of study.

Hand Function in Muscular Dystrophies: Relationship Between Performance of Upper Limb and Jebsen–Taylor Tests

The aim of this study was to investigate the relationship between Performance of Upper Limb (PUL) and Jebsen–Taylor Test (JTT) to assess and monitor upper limb function progression in patients with muscular dystrophy. Thirty patients diagnosed with Duchenne muscular dystrophy, limb-girdle muscular dystrophy, Becker muscular dystrophy, myotonic dystrophy Type 1, and fascioscapulohumeral dystrophy were submitted to the shoulder, elbow, and wrist domains of PUL, and to JTT subtests. Spearman tests investigated the relationships between PUL and JTT total scores and domains. Correlations were classified as strong (r ≥ 0.70), moderate (0.40 ≤ r < 0.70), or weak (r ≤ 0.40). There were strong correlations between the PUL and JTT total scores (r = −0.706). Although JTT measures time and PUL provides kinesiologic scores, these measures were related. Therefore, muscle synergies, which control the compensatory movements and motor functions involving mainly shoulder, elbow, wrist, and finger movements, are related to timed performance in patients with muscular dystrophies.

Relationship between muscle strength and motor function in Duchenne muscular dystrophy.

OBJECTIVE To investigate the relationship between muscle strength and motor function and between these variables and age. METHOD Muscle strength was measured by Medical Research Council (MRC) scale and motor function, by Motor Function Measure (MFM), in 40 non-ambulatory patients. Spearman tests investigated the relationships between muscle strength, motor function and age. RESULTS Total MRC and MFM scores were strongly related to each other (r = 0.94; p < 0.001), but not to age (r = -0.19, r = -0.31, respectively; p > 0.05). Strong and moderate relationships between partial muscle strength and motor function scores were found. Higher correlation coefficients were found between total scores and Dimensions 2 (axial/ proximal control) and 3 (distal control) of MFM. CONCLUSION Muscle strength and motor function are strongly correlated and seem to decrease proportionally in DMD.

Effects of modified Restricted Environmental Stimulation Therapy on relaxation, heart rate, blood pressure and flexibility

Background : Restricted Environmental Stimulation Therapy (REST) promotes physiological and behavioral changes that reduce the deleterious effects of stress. However, it requires expensive equipment and is accessible to a limited number of professionals and patients. We aimed to evaluate the physiological and behavioral effects of modified REST (mREST) in healthy young women. Method: Twenty-one healthy young women (20-25 yrs) participated. mREST consists of positioning the patient floating in the pool with 32 o C for about 15 minutes, for twelve sessions, with blindfolded and wearing earplugs. The evaluation was performed before and after the intervention. The analysis of the state of relaxation was investigated by a questionnaire and the self-reports were categorized. Measures of heart rate and blood pressure were used as indicators of the cardiovascular response. Flexibility, measured by the finger-to-floor test, was used as an indicator of muscle relaxation. Results : Heart rate and blood pressure significantly decreased while flexibility and relaxation increased after the sessions (p<0.001 for all comparisons). Self-reports of relaxation were consistent with the blood pressure measures and indicated that the participants showed states of relaxation associated with the decrease of blood pressure and the increase of flexibility (p<0.001 for all comparisons). Conclusion : Healthy women undergoing mREST reported relaxation, with effects on muscular and cardiovascular systems. mREST is a simple, practical and affordable option for therapy in the aquatic environment.

Boys With Duchenne Muscular Dystrophy: 1-Year Locomotor Changes in Relation to a Control Group

We compared the timed performance and compensatory movements of 32 boys (mean age = 10.0 years) with Duchenne muscular dystrophy (DMD) and 38 healthy boys (mean age = 9.2 years) on 10 -meter walking and four-step stair work (climbing and descending). Dependent measures were digital scores on the Functional Evaluation Scale for DMD obtained by watching the boys on film. Groups were compared initially and after 6 and 12 months, using General Linear Models analysis (for two groups and three assessments). Results showed no significant timed performance differences between groups on 10 -m walking at the initial assessment; but boys with DMD showed longer times after 6 and 12 months, and boys with DMD engaged in more compensatory movements while walking. For stair climbing, boys with DMD were significantly slower than healthy controls when both climbing and descending steps in all assessments.

Independência funcional de crianças de um a quatro anos com mielomeningocele

Corresponding address: Mariana Callil Voos – Universidade Ibirapuera – Interlagos Avenue, 1329 – São Paulo (SP), Brazil – Zip Code: 04661100 – E-mail: ftmarivoos@gmail.com – Financing source: Nothing to declare – Conflict of interests: Nothing to declare – Presentation: Sept. 16th, 2017 – Accepted for publication: Mar. 4th, 2018 – Approved by the Ethics Committee of Universidade Santa Cecília under protocol no 13555913.7.0000.5513. Functional independence of oneto four-year-old children with myelomeningocele Independência funcional de crianças de um a quatro anos com mielomeningocele Independencia funcional de niños de un a cuatro años con mielomeningocele Fabiane Ramos Ferreira1, Fernanda Pinheiro Bexiga2, Vivian Vargas de Moraes Martins3, Francis Meire Favero4, Cristina Dallemole Sartor4, Mariana Cunha Artilheiro4, Mariana Callil Voos4| Myelomeningocele is caused by neural tube closure defects and represents the second cause of locomotion disability in children. Functional independence depends on level of spine injury and assessment is important to determine proper therapeutic approaches. We aimed to describe functional Independence and level of injury in 15 children, aged one to four years and with complete spinal cord injury caused by myelomeningocele. This is an observational transversal study developed in the Ibirapuera University and Santa Cecília University. The Pediatric Evaluation of Disability Inventory (PEDI) was used to ask parents about the functional independence of children in daily life activities. The International Standards for Neurological Classification of Spinal Cord Injury of the American Spinal Injury Association was used to determine the motor and sensory levels. Nine girls and six boys were assessed (27.0 ±11.8 months of age). Three children showed thoracic level, nine showed high lumbar level, two were classified as low lumbar, and one as sacral level. PEDI scores varied from 15 to 60% on the self-care area, from 10 to 15% on mobility, and from 19 to 58% on social function. High variability was observed on the functional independence of children with myelomeningocele, detected by self-care and social function areas of PEDI. Mobility was the most affected domain. Ferreira et al. Independência em crianças com mielomeningocele 197