A 76-year-old man was admitted to our hospital because of sudden upper abdominal pain and shock status. The patient had undergone Miles' procedure with a colostomy on the left lower abdomen due to rectal cancer at the age of 70 years. CT scans revealed a thoracoabdominal aortic aneurysm. In view of the clinical findings, ruptured aneurysm requiring emergent operation was diagnosed. A left spiral skin incision was made, keeping away from the colostomy. An extraperitoneal approach was selected. The thoracoabdominal aorta was replaced with an artificial graft under partial extracorporeal circulation with femoral arterial and venous cannulation. The postoperative course was uneventful. No paraplegia occurred in spite of no reconstruction of the intercostal arteries due to severe atherosclerotic changes of the aortic wall. The fact that bleeding due to ruptured aneurysm was localized in the extrapleural and extrapritoneal spaces seemed to be an advantageous factor for the success in this case.

A 47-year-old woman complained of dyspnea on exertion. Ultrasonic cardiography revealed coronary sinus type atrial septal defect. At operation, the drainage veins to the left atrium from the coronary arteries were observed but no anomalies of the vena cave or any other veins were observed. The defect was closed with a pericardial patch under cardiopulmonary bypass. The post-operative course was uneventful. Coronary arteriography performed on the 14th post operative day confirmed that the coronary veins drained individually into the corresponding atria. Unroofed coronary sinus is rare and difficult to diagnose prior to operation. Ultrasonic cardiography and coronary arteriography are considered useful for preoperative diagnosis.

The patient was a 63-year-old man with a history of multiple mononeuritis with hypergammaglobulinemia since 1980. The symptoms gradually worsened, and he had been bed-ridden since 1992. On February 28, 1997, he had sudden dyspnea after defecation. Echocardiography demonstrated a large thrombus in the right atrium and the right ventricle. Enhanced chest computed tomography revealed thrombi in the bilateral pulmonary arteries. The patient was considered to have acute pulmonary thromboembolism, and an emergency operation was indicated. Thrombectomy was performed under extracorporeal circulation through a median sternotomy. No thrombi were found in the right atrium or the right ventricle, and thrombi in the bilateral pulmonary arteries were removed completely. Four days after the operation, a Greenfield filter was implanted in the vena cava inferior because venography detected a thrombus in the right common iliac vein. The postoperative course was uneventful. No pulmonary rethromboembolisms were noticed after the operation. The long duration of being bed-ridden seemed to be the chief cause of thrombosis in the deep veins, and hyperviscosity due to hypergammaglobulinemia may have caused hyperthrombogenicity.

This review summarizes improvements in understanding the pathophysiology and early clinical symptoms of multiple system atrophy (MSA) and advancements in diagnostic methods and disease-modifying therapies for the condition. In 2022, the Movement Disorder Society proposed new diagnostic criteria to develop disease-modifying therapies and promote clinical trials of MSA since the second consensus was proposed in 2008. Regarding pathogenesis, cutting-edge findings have accumulated on the interactions of α-synuclein, neuroinflammation, and oligodendroglia with neurons. In neuroimaging, introducing artificial intelligence, machine learning, and deep learning has notably improved diagnostic accuracy and individual analyses. Advancements in treatment have also been achieved, including immunotherapy therapy against α-synuclein and serotonin-targeted and mesenchymal stem cell therapies, which are thought to affect several aspects of the disease, including neuroinflammation. The accelerated progress in clarifying the pathogenesis of MSA over the past few years and the development of diagnostic techniques for detecting early-stage MSA are expected to facilitate the development of disease-modifying therapies for one of the most intractable neurodegenerative diseases.

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.
Biomarkers ; Brain ; Cerebellar Ataxia ; Cerebrospinal Fluid ; Dementia ; Diagnosis ; Early Diagnosis ; Hallucinations ; Humans ; Multiple System Atrophy ; Neurodegenerative Diseases ; Paralysis ; Parkinsonian Disorders ; Phenotype ; White Matter