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Background: Multiple system atrophy (MSA) is a sporadic neurodegenerative disease. According to the clinical presentation a parkinsonian type and a cerebellar type (MSA-C) are distinguished. Objective: To study the morphological alterations of MSA-C–affected brains in vivo using voxel-based morphometric analysis of magnetic resonance images. Setting: University hospital. Patients: Fourteen patients (5 men and 9 women) with MSA-C (mean age [SD], 59.4 [7.4] years; mean [SD] disease duration, 3.7 [1.4] years) and 13 healthy control subjects (5 men and 8 women) (mean [SD] age, 55.1 [6.9] years) were studied. Methods: T1-weighted magnetic resonance images were normalized to a common stereotaxic space and segmented into gray and white matter. Data were analyzed using statistical parametric mapping (SPM99). Results: Gray matter was reduced in the brainstem and the anterior lobe of the cerebellum. Reduction of white matter was observed in the middle cerebellar peduncles, cerebellar white matter, and brainstem. The inverted comparison revealed an increase of white matter density along the pyramidal tracts. Conclusions: Voxel-based morphometry revealed a significant loss of cerebellar and brainstem tissue in MSA-C. It allowed a precise anatomical localization and a distinction between gray and white matter densities. In addition, our data point to a particular involvement of the pyramidal tract in MSA-C. [ABSTRACT FROM AUTHOR]

Background:. Olfactory dysfunction has been reported in Parkinson’s (PD) and Alzheimer’s disease (AD). Objective:. We studied olfactory function in eight patients with multiple system atrophy of cerebellar type (MSA-C), eleven patients with sporadic cerebellar ataxia of unknown etiology and thirteen controls matched for age and gender. Subjects received tests for n-butanol odor thresholds, odor identification, and odor discrimination. Results:. Olfactory thresholds were abnormally high in 16% of the patients. Odor discrimination and odor identification were impaired in 44 % and 74% of the patients, respectively. There was no significant difference in olfactory function between patients with sporadic ataxia of unknown etiology and MSA-C patients. Conclusions:. The present data suggest that olfactory dysfunction is common to various neurodegenerative disorders and not specifically restricted to PD or AD. Cerebellar dysfunction affected suprathreshold olfactory function more severely than odor thresholds. Thus cerebellar lesions may affect the processing of odor-related information to a higher degree than the transport of odorants to the receptor through sniffing. [ABSTRACT FROM AUTHOR]

To study the frequency and severity of peripheral neuropathy in multiple system atrophy (MSA), we performed nerve conduction studies in 42 MSA patients suffering from either cerebellar MSA (MSA-C) or parkinsonian MSA (MSA-P). Abnormal nerve conduction was present in 24% of the patients. Abnormalities were significantly more frequent in MSA-P (43%) compared to MSA-C (14%). Motor nerve conduction velocities were reduced in 4% of the MSA-C and in 7% of the MSA-P patients. Abnormal compound muscle action potentials were more frequent in MSA-P (29% versus 7% in MSA-C) pointing to a more pronounced loss of motor axons in this subgroup. Sensory nerve conduction velocities were abnormal in 4% of the MSA-C and 14% of the MSA-P patients, and mean sensory nerve action potentials were normal in all MSA-C and reduced in 7% of the MSA-P patients. The data provide evidence that the peripheral nervous system is differentially affected in MSA-C and MSA-P.Copyright © 2000 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]