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J Neurol. 2007 Oct;254(10):1384-9. Epub 2007 Oct 15.

Sporadic adult onset ataxia of unknown etiology : a clinical, electrophysiological and imaging study.

Author information

  • 1Dept. of Neurology, University of Bonn, Sigmund-Freud-Str. 25, 53105, Bonn, Germany. michael.abele@uni-bonn.de

Abstract

BACKGROUND:

The sporadic adult onset ataxias of unknown etiology (SAOA) denote the non-hereditary degenerative adult onset ataxias that are distinct from multiple system atrophy (MSA).

OBJECTIVE:

To define and characterize the clinical phenotype of sporadic adult onset ataxia of unknown etiology (SAOA).

DESIGN:

A survey of clinical features, nerve conduction and evoked potentials, autonomic tests, and magnetic resonance imaging (MRI)-based brain morphometry was conducted in patients with SAOA.

PATIENTS:

Study subjects were a consecutive sample of 27 patients (11 male, 16 female) who met the diagnostic criteria for SAOA (age 55 +/- 13 years; age at disease onset 47 +/- 14 years; disease duration 8 +/- 7 years).

RESULTS:

All patients presented with a cerebellar syndrome. The most frequent extracerebellar symptoms were decreased vibration sense in 70% and decreased or absent ankle reflexes in 33% of the patients. Nerve conduction studies revealed a polyneuropathy in 26% of the patients. Somatosensory evoked potentials were abnormal in 44%, and central motor conduction time in 17% of patients. Autonomic testing revealed an affected autonomic nervous system in 58% of patients. Voxel-based brain morphometry showed a predominant reduction of gray matter in the cerebellum which was significantly correlated with disease stages. A loss of white matter was found in both middle cerebellar peduncles and the outer edge of the pons.

CONCLUSIONS:

The data show that SAOA is a predominantly, but not exclusively cerebellar disorder. Clinical, electrophysiological, and imaging findings showed some similarities with multiple system atrophy which raises the question of an overlap of these two disorders.

PMID:
17934884
[PubMed - indexed for MEDLINE]
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