Abstract:
INTRODUCTION:The distribution of clinical and neurophysiological abnormalities in patients with early amyotrophic lateral sclerosis (ALS) was investigated in an attempt to delineate patterns of disease spread. METHODS:Clinical and electrodiagnostic data were collected from 150 ALS patients and analyzed based on the clinical region of onset. RESULTS:Asymmetry of clinical and neurophysiological abnormalities was more marked in upper limb-onset than lower limb-onset disease. Significant rostral-caudal gradients of clinical weakness were identified in bulbar- and lower limb-onset disease. Neurophysiological evidence of the ALS "split-hand" pattern was evident irrespective of the region of disease onset. Limbs with and without evidence of clinical weakness demonstrated similar rates of abnormality on electromyography. CONCLUSIONS:These findings suggest a pattern of disease spread in ALS. This study may serve to guide ongoing development of disease quantitation biomarkers and the targeting of future neuroprotective strategies.
journal_name
Muscle Nervejournal_title
Muscle & nerveauthors
Simon NG,Lomen-Hoerth C,Kiernan MCdoi
10.1002/mus.24244subject
Has Abstractpub_date
2014-12-01 00:00:00pages
894-9issue
6eissn
0148-639Xissn
1097-4598journal_volume
50pub_type
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