Clinical Analysis Of Multiple System Atrophy (MSA)

Background:Multiple system atrophy (MSA) is a sporadic neurodegenerative disorder of unknown etiology which is involved with multiple systems. The main clinical symptoms include autonomic dysfunction, Parkinson’s symptoms and/or cerebellar ataxia etc. MSA is mainly divided into two subtypes:MSA-P with the outstanding performance of Parkinson’s symptoms and MSA-C with the outstanding performance of cerebellar ataxia. No matter in which subtype, autonomic dysfunctions are common. With its variable clinical presentations, multiple system atrophy presents a major diagnostic challenge not only in neurology but also in other specialties, including cardiology, gastroenterology, urology, and otolaryngology, which is susceptible to misdiagnosis and missed diagnosis at the early stage.Objective:To explore the clinical features, imaging findings, urine residual volume by ultrasound of patients with MSA, and to improve the clinical diagnosis of MSA.Methods:58 MSA cases fulfilled the Gilman diagnostic criteria (2008) were recruited. Clinial presentations, neuroimaging, urine residual volume by ultrasound and lying-standing blood pressure data of patients were retrospectively analyzed. According to their clinical manifestations, subtypes were classified.Results:Of 58 MSA patients, there were 37 men and 21 women, and the male-to-female ratio was 1.8:1. Mean age of onset (at time of first reported symptom) was 55.0±8.2 (range from 32 to 72). The mean clinical course up to this study was 3.1±1.9 years (range from 1 to 9 years).30 cases (21 male and 10 female) were diagnosed with MSA-C,28 (16 male and 13 female) with MSA-P. The mean onset age of MSA-C patients was 54.3±7.2 (range from 36 to 65), and the mean clinical course was 3.1±2.0 years (range from 1 to 9 years). The mean onset age of MSA-P patients was 55.7±9.2 (range from 32 to 72), and the mean clinical course was3.1±1.8 years (range from 1 to 8 years). Of all the patients, the onset symptoms included dizziness, urinary dysfunction, tremor, rigidity, bradykinesia, gait ataxia, dysarthria and limb weakness, with gait ataxia(50.0%) and dizziness (20.0%) as the main onset manifestations of MSA-C and with rigidity, tremor, bradykinesia (28.6%,17.9%,14.3%) as the main onset symptoms of MSA-P. The most common clinical presentations included urogenital dysfunction, gait ataxia, rigidity, bradykinesia, dizziness and dysarthria. The presentations were different in two subtypes:gait ataxia was more common in MSA-C and rigidity, bradykinesia were more common in MSA-P. Of the 55 cases with MRI abnormalities, MSA-C accounted for 29 and MSA-P accounted for 27. The most common lesions of MSA-C were cerebellar/pontine atrophy and ventricle expansion(71.4%,57.1%) and those of MSA-P were cerebral atrophy, putaminal rim sign and hot-cross-bun sign(29.6%,14.8%,18.5%). Among 58 patients,32 cases took the ultrasound examination of urine residual volume. Among these patients, there were 11 cases (34.3%) exceeding 100ml of the urine residual volume, in which MSA-C had 6 and MSA-P had 5. Comparing MSA-C with MSA-P, there was not obvious difference between them (P=0.907) o 53 cases did lying-standing blood pressure examination and 23 cases fulfilled the criteria of orthostatic hypotension. Among them MSA-C accounted for 60.0%(18 cases) and MSA-P accounted for 17.9%(5 cases). There was statistic difference between them (P=0.001).Conclusions:MSA is a group of multisystem-involved neurodegenerative diseases. Because of variable clinical presentations, it is susceptible to misdiagnosis and missed diagnosis at the early stage. Consequently, its clinical diagnosis should be detailed medical history and neurological examination, with auxiliary examinations such as magnetic resonance imaging (MRI) needed.