Medullo-ponto-cerebellar white matter degeneration altered brain network organization and cortical morphology in multiple system atrophy

The cerebellum involves diverse functions from motor coordination to higher cognitive functions. Impairment of the cerebellum can cause ataxia and cerebellar cognitive affective syndrome. Multiple system atrophy of the cerebellar type (MSA-C) is a neurodegenerative disorder with atrophy of medullo-ponto-cerebellar (MPC) white matter (WM). To understand the role of the cerebellum from the perspective of the local structure to the global function in the presence of MPC WM degeneration, we acquired T1-weighted and diffusion tensor images for 17 patients with MSA-C and 19 normal controls. We concurrently used the measures of local morphology, including MPC WM volume and inner surface area, and properties of global network organization based on graph theory for the MSA-C and control groups. The results showed that MPC WM degeneration caused the destruction of cerebello-ponto-cerebral loops, resulting in reduced communication efficiency between regions in the whole-brain network. In addition, the sulcal area of the inner cortical surface in the cerebellum decreased linearly with the MPC WM volume, and the inferoposterior lobe exhibited a steeper atrophy rate than that of vermis regions. We concluded that the integrity of MPC WM is critical in sustaining the local morphology and the global functions of the whole-brain fiber network.     

Co-activation of primary motor cortex ipsilateral to muscles contracting in a unilateral motor task

ObjectiveThis study aims to investigate the role of the primary motor cortex ipsilateral to the movement (ipsilateral M1) in unilateral motor execution.MethodsFifteen right-handed healthy subjects underwent functional magnetic resonance imaging (fMRI) and transcranial magnetic stimulation (TMS) experiments. Motor tasks were performed with the right-side limb. Subjects followed visual cues to execute movements in the scanner and independent component analysis (ICA) was applied to analyse the data. Interhemispheric inhibition (IHI), short-interval intracortical inhibition (SICI) and recruitment curves (RCs) of motor-evoked potentials (MEPs) in right M1 were measured by TMS and responses were recorded from the left flexor carpi radialis (FCR) and left anterior deltoid (AD).ResultsGroup ICA showed activations of bilateral M1s highly related to motor tasks. Additionally, TMS results showed significant increases of MEP RCs on the left FCR and left AD during right wrist flexion and right shoulder flexion. Prominent decreases of IHI and SICI were also observed under the same conditions.ConclusionsDuring unilateral muscle contraction, co-activation of the ipsilateral M1 involves additional processes modulated by intra- and interhemispheric interactions and its size of activations is specifically enhanced on the homotopic representation.SignificanceThe ipsilateral M1 plays a central role in unilateral motor executions.     

Comparison of population pyramid and demographic characteristics between people with an intellectual disability and the general population

The purposes of this study were to measure disparities of age structure between people with an intellectual disability and general population, and to explore the difference of demographic characteristics between these two populations by using data from a population based register in Taiwan. Data were analyzed by SPSS 20.0 statistical software. Results found that the gender and mean age were significantly different between people with an intellectual disability and general population (mean age: 28.86 years vs. 35.26 years; p < 0.001). The shape of the pyramid in general population tended to be fatty in the middle age, and intellectual disability population was more populous in the younger age. Furthermore, there were very few people with an intellectual disability can live more than 65 years old (3%) while there were nearly 10% of the general population were the elderly. The results also showed that two groups were different in marital status, educational levels, family status of veteran and aborigine (p < 0.001). As the premature aging and short life span of people with an intellectual disability, this study suggested that the government authority should initiate necessary assistance for this group of people in the future.     

Sudden and unexpected and near death during the early neonatal period: A multicenter study

BackgroundTo investigate the incidence, clinical presentation and possible etiologies or risk factors of early onset of sudden and unexpected death or near-miss.MethodsFrom 2001 to 2005, a retrospective analysis of observational database of neonates who were younger than one week old without any risk factors at five tertiary medical centers. The demographic data, clinical manifestations, laboratory data and possible etiologies were retrospectively collected and analyzed.ResultsSeventeen neonates presumed to be healthy at birth encountered early near death in five medical centers in Taipei city. The mean gestation age (GA) was 38.5 ± 1.2 weeks, mean birth body weight (BBW) was 2948.2 ± 327.8 gm. The median age at event was 26 hours old. Eleven patients were rooming-in babies with exclusive breast feeding. Seven patients (41.2%) died; seven patients (41.2%) survived with neurological sequelas, and the remaining three patients (17.6%) survived without complication. Possible causative factors included infection in two cases, urea cycle disorder in one case, hypertrophic cardiomyopathy in one case, hypocalcemia only in one case, hypocalcemia plus airway obstruction in one case, dehydration-related diseases in seven cases and unknown in 4 cases; there was no autopsy case.ConclusionMore effort on promotion of autopsy to discover the underlying disease is necessary and helpful. To build up an alarm system or protocol for education and early detection is the basis to prevent this tragedy.     

Medical care services for people with intellectual disabilities living in the general community: A cross-sectional survey of inpatient care utilization in Taiwan, 2001

Background. The medical care needs and problems of persons with intellectual disabilities (ID) living in the general community have received limited attention in previous studies. The aim of this article is to describe aspects of medical care utilization among people with ID living in the general community, with particular emphasis on examining the type and determinants of inpatient care utilization in Taiwan.

Methods. A cross-sectional survey of people with intellectual disabilities was employed. A total of 997 respondents who provided fully completed data concerning inpatient care utilization were recruited into the analysis.

Results. A total of 12.4% of individuals with intellectual disabilities had used inpatient care in the 7 months prior to the survey. The average number of inpatient care visits in that time was 1.43, with an average hospital stay of 16.91 days. Surgery, fever, gastrointestinal disorders, psychiatric disorders, and accident were the main causes of inpatient care utilization. A stepwise logistic model showed that the factors of holding a Major Illness Card, regular medicine-taking and self-perceived health status were statistically significant to inpatient care utilization of people with intellectual disabilities.

Conclusions. Medical care providers and policy makers need to be aware that many people with intellectual disabilities have increased medical care needs that may require modification of standard medical care practices and service models in society.     

Physical and mental health status of staff working for people with intellectual disabilities in Taiwan: measurement with the 36-Item Short-Form (SF-36) health survey

Little explicit attention has been given to the generic health profile of staff working for people with intellectual disability in institutions. This study aimed to provide a profile of physical and mental health of staff working in disability welfare institutions, and to examine the possible demographic and organizational factors that explain an association with their health. A cross-sectional questionnaire survey was conducted to analyze 1243 staff (76% response rate) working in 24 institutions in Taiwan. The 36-Item Short-Form (SF-36) Taiwan version was used to measure their generic health status. The mean of Physical component scores (PCS) was slightly higher than Mental component scores (MCS) (50.83 vs. 45.12). With regard to each dimension among PCS, the mean score of Physical functioning (PF) was 57.14 (S.D.=5.93), Role limitations-physical (RP) was 49.88 (S.D.=9.69), Bodily pain (BP) was 52.14 (S.D.=8.09) and General medical health (GH) was 51.50 (S.D.=8.28). Among the MCS, Vitality (VT) was 46.19 (S.D.=6.71); Social functioning (SF) was 46.44 (S.D.=7.58); Role limitations-emotional (RE) was 47.30 (S.D.=11.89) and Mental health (MH) was 43.58 (S.D.=8.81). We found the generic health of staff working for people with intellectual disabilities were significantly lower in PCS and MCS than the Taiwan general population. Influences of staff's demographic and organizational characteristics on their health were also analyzed in the content. This study highlights the authorities and service providers need to continue to develop their awareness and understanding of the experiences that their staff encounters in the organizations, so that they can receive resources to support their positive health in working for people with intellectual disabilities.