Multiple system atrophy: A review of 203 pathologically proven cases

Abstract

We report the clinicopathological features of 203 cases of pathologically proven multiple system atrophy (MSA) from 108 publications up to February 1995. The majority of patients showed symptoms in their early fifties, and men were more commonly affected than women (ratio of 1.3:1). Most patients suffered from some degree of autonomic failure (74%). Parkinsonism was the most common motor disorder (87%), followed by cerebellar ataxia (54%) and pyramidal signs (49%). The response to levodopa was poor in most patients, but there was a subgroup with a good response, who also often developed axial levodopa-induced dyskinesias. Other characteristic features included severe dysarthria, stridor, and, in a few patients, contractures and dystonia (antecollis). Mild or moderate intellectual impairment occurred in some cases, but severe dementing illness was most unusual. The main pathological change comprised cell loss and gliosis in the putamen, caudate nucleus, external pallidum, substantia nigra, locus ceruleus, inferior olives, pontine nuclei, cerebellar Purkinje cells, and intermediolateral cell columns of the spinal cord. However, other neuronal populations were also involved to varying degrees, such as the thalamus, vestibular nucleus, dorsal vagal nucleus, corticospinal tracts, and anterior horn cells. Characteristic glial and/or neuronal cytoplasmic inclusions were identified in all cases in which they were sought, irrespective of clinical presentation. Akinesia correlated with the degree of nigral and putaminal cell loss, whereas rigidity was related only to the later. Tremor was unrelated to cell loss at any site. Ataxia correlated with the degree of olivopontocerebellar atrophy. Pyramidal signs were associated with pyramidal tract pallor. Our analysis also confirmed an association of postural hypotension with intermediolateral cell column degeneration.

MSA-C is the predominant clinical phenotype of MSA in Japan: Analysis of 142 patients with probable MSA

“…In this series, the frequency of neruogenic bladder at an early stage and impotence was relatively low compared with several previous reports [12,14]. However, this result may have been biased because it was determined by asking questions and by retrospective review of each patient's medical record.…”

“…Other disorders associated with parkinsonism, such as Parkinson's disease with autonomic failure, progressive supranuclear palsy (PSP), and diffuse Lewy body disease (DLBD), may often be misdiagnosed as MSA-P [7,8,9,10,11,12]. A neuropathological study showed that Table 1.…”

Morning Glory Sign: A Particular MR Finding in Progressive Supranuclear Palsy

“…23 On the other hand, in MSA degeneration of the olivopontocerebellum (the transverse pontineˆbers and pontine neurons), striatonigral and autonomic systems are seen; however, degeneration of the gray matter around the aqua duct and superior colliculus does not occur. 26,27 The midbrain usually shrinks not only in PSP, but also in MSA. However, in the present study, the lateral margin of the midbrain in four of theˆve PSP patients was concave (the morning glory sign) and that of almost all patients with MSA remained convex.…”

Differential Diagnosis of Parkinsonism Using Dual-Phase F-18 FP-CIT PET Imaging

“…However, the differential diagnosis of parkinsonism continues to be challenging with a high misdiagnosis rate, particularly in the early stage [6], because parkinsonian patients show similar symptoms and specific symptoms do not appear in early stage [7]. It is of importance to make an accurate differential diagnosis of parkinsonism to decide on treatment regimens [8], provide a prognosis [9], investigate etiology and pathogenesis, and to develop new therapeutic strategies [10].…”