Clinical and Imaging Features of Multiple System Atrophy: Challenges for an Early and Clinically Definitive Diagnosis

Multiple system atrophy (MSA) is an adult-onset, progressive neurodegenerative disorder. Patients with MSA show various phenotypes during the course of their illness, including parkinsonism, cerebellar ataxia, autonomic failure, and pyramidal signs. Patients with MSA sometimes present with isolated autonomic failure or motor symptoms/signs. The median duration from onset to the concomitant appearance of motor and autonomic symptoms is approximately 2 years but can range up to 14 years. As the presence of both motor and autonomic symptoms is essential for the current diagnostic criteria, early diagnosis is difficult when patients present with isolated autonomic failure or motor symptoms/signs. In contrast, patients with MSA may show severe autonomic failure and die before the presentation of motor symptoms/signs, which are currently required for the diagnosis of MSA. Recent studies have also revealed that patients with MSA may show nonsupporting features of MSA such as dementia, hallucinations, and vertical gaze palsy. To establish early diagnostic criteria and clinically definitive categorization for the successful development of disease-modifying therapy or symptomatic interventions for MSA, research should focus on the isolated phase and atypical symptoms to develop specific clinical, imaging, and fluid biomarkers that satisfy the requirements for objectivity, for semi- or quantitative measurements, and for uncomplicated, worldwide availability. Several novel techniques, such as automated compartmentalization of the brain into multiple parcels for the quantification of gray and white matter volumes on an individual basis and the visualization of α-synuclein and other candidate serum and cerebrospinal fluid biomarkers, may be promising for the early and clinically definitive diagnosis of MSA.

Investigation of Inpatient Rehabilitation Outcomes in different Ischemic Stroke Disease Types : Relationships with Leukoaraiosis in MRI / The Japanese Journal of Rehabilitation Medicine

<b>Purpose</b> : The aim of this study is to investigate inpatient rehabilitation outcomes in different ischemic stroke disease types. Subjects and methods : Subjects were 178 patients with ischemic stroke transferred from stroke units or emergency units for inpatient rehabilitation at Kami-iida Rehabilitation Hospital. For all patients, National Institutes of Health Stroke Scale (NIHSS) scores were measured on admission. Functional Independence Measure (FIM) scores were also measured both on admission and discharge, and FIM-gain (FIM-g) and FIM-efficiency (FIM-e) values were calculated. The disease types of ischemic stroke were : lacunar (LI) in 16 patients ; atherothrombosis (AI) in 23 ; branch-atheromatous-disease (BAD) in 59 ; artery to artery embolism (A to A) in 18; cardiogenic embolism (CE) in 34 ; undetermined embolism (unable to differentiate from A to A and cardiogenic embolism) in 22 ; the 6 remaining patients were not categorized. Results : There were no significant differences in the NIHSS scores and FIM scores on admission between disease types except for the NIHSS scores in the LI patients. The FIM-e value in A to A patients was significantly lower than those in other types (<i>p</i><0.05). Moreover, A to A patients have a tendency of severe leukoaraiosis and their MRAs demonstrated high rates of stenosis (≥50%) or occlusion with intracranial arteries. Conclusion : In A to A embolism, significantly lower FIM-e values were found and FIMs at discharge were affected by leukoaraiosis on the basis of large-vessel arteriosclerosis. Our study revealed that inpatient rehabilitation outcomes differed for each ischemic stroke type and appeared to be influenced by leukoaraiosis.