Leucoencephalopathy with neuroaxonal spheroids (LENAS) presenting as the cerebellar subtype of multiple system atrophy
- 1Department of Neurology, University Hospitals of Cleveland and Case Western Reserve University School of Medicine, 11100 Euclid Avenue, Cleveland, Ohio 44106, USA
- 2Department of Pathology, University Hospitals of Cleveland and Case Western Reserve University School of Medicine
- Correspondence to: Dr D Riley Department of Neurology, University Hospitals of Cleveland and Case Western Reserve University School of Medicine, 11100 Euclid Avenue, Cleveland, Ohio 44106, USA; David.Rileyuhhs.com
- Received 16 September 2003
- Accepted 29 November 2003
- Revised 25 November 2003
Abstract
Leucoencephalopathy with neuroaxonal spheroids (LENAS) is a rare disease of cerebral and cerebellar white matter. LENAS usually presents as a disorder of cognition and behaviour, or with gait dysfunction and ataxia. This report describes a patient who had a 14 year course of progressive neurological decline consistent with a clinical diagnosis of probable multiple system atrophy, with prominent cerebellar dysfunction and dysautonomia. Formal autonomic laboratory testing was consistent with global autonomic dysfunction of central origin. However, magnetic resonance imaging showed extensive white matter signal abnormalities, in addition to moderate cerebral and cerebellar atrophy. On postmortem microscopic examination, there were numerous axonal spheroids throughout the white matter of both regions. This case of LENAS presented unique clinical characteristics, and typical pathological findings.
- LENAS, eucoencephalopathy with neuroaxonal spheroids
- MMSE, Mini Mental Status examination
- MRI, magnetic resonance imaging
- MSA, multiple system atrophy
- MSA-C, type C multiple system atrophy
- NAD, neuroaxonal dystrophy
Footnotes
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Conflicting interests: none declared
