Muscle histopathology in upper motor neuron-dominant amyotrophic lateral sclerosis
- 1 January 2008
- journal article
- Published by Taylor & Francis Ltd in Amyotrophic Lateral Sclerosis
- Vol. 9 (5), 287-293
- https://doi.org/10.1080/17482960802206801
Abstract
The distinction between primary lateral sclerosis (PLS) and amyotrophic lateral sclerosis (ALS) still remains debated. Recently, PLS patients displaying lower motor neuron (LMN) signs have been defined as 'upper motor neuron (UMN)-dominant ALS', using 'clinically pure PLS' diagnosis to those with no LMN signs. To further characterize the LMN involvement in UMN-dominant ALS we investigated the presence and the extent of neurogenic abnormalities in the skeletal muscle of patients affected with a pyramidal syndrome consistent with UMN-dominant ALS. A total of nine patients affected with UMN-dominant ALS were analysed. In all cases, muscle biopsies showed the presence of scattered or clustered atrophic angulated fibres in small groups, and a mild to moderate fibre type-grouping. Target and targetoid fibres were detected in two cases only. Three patients had a second muscle biopsy which demonstrated a roughly unchanged pattern of chronic denervation with still moderate reinnervation phenomena. This study suggests that in UMN-dominant ALS muscle denervation may be characterized by an early chronic impairment of a restricted number of LMNs. The extent rather than the presence of LMN signs may allow to categorize patients with motor neuron disease involving mainly UMN into distinct entitiesKeywords
This publication has 19 references indexed in Scilit:
- Primary lateral sclerosisMuscle & Nerve, 2007
- The natural history of primary lateral sclerosisNeurology, 2006
- Primary Lateral SclerosisJournal of Clinical Neuromuscular Disease, 2005
- Primary lateral sclerosis: clinical, neurophysiological, and magnetic resonance findingsJournal of Neurology, Neurosurgery & Psychiatry, 2001
- The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory functionJournal of the Neurological Sciences, 1999
- Clinicopathological features of primary lateral sclerosis are different from amyotrophic lateral sclerosisBrain Research Bulletin, 1993
- PRIMARY LATERAL SCLEROSIS: CLINICAL FEATURES, NEUROPATHOLOGY AND DIAGNOSTIC CRITERIABrain, 1992
- Denervation in hemiplegic muscles.Stroke, 1990
- The central nervous system in motor neurone diseaseJournal of Neurology, Neurosurgery & Psychiatry, 1970
- The histographic analysis of human muscle biopsies with regard to fiber typesNeurology, 1969