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.

Considering the Utilization of “Discharge Support Protocol” on Acute Care Ward / 日本農村医学会雑誌

  In our hospital, we have appointed some nurses to full time support and adjust patient discharges since May 2009. We have an electronic “Discharge Support Protocol”, which is a tool that includes discharge adjustment screening, discharge support assessment, discharge adjustment planning, and discharge support planning. However, only a few ward nurses have used the tool. We performed a survey of nurses in the acute care ward, where 32% of the inpatients require discharge support input to consider how the “Discharge Support Protocol” could be fully utilized. The main reasons given for the low input rate were the difficulty in finding time from routine ward duties, and the vagueness of the protocol. We, therefore, made it part of the routine ward duty to fill out the discharge support protocol, and also made the work flow clearer. As a result, we found an increase in input rate of the first part of the protocol which deals with discharge adjustment screening, but did not find any change in input rate from the second part onwards. The challenge from now on will be to take further measures to improve the overall input rate. In addition, we have suggested the usability of the “Discharge Support Protocol” to educate ward nurses, and to share information about patients between nurses and other departments.