ALS的电诊断.pdfVIP

Electrodiagnostics Supplement Electrodiagnosis in Persons With Amyotrophic Lateral Sclerosis Nanette C. Joyce, DO, Gregory T. Carter, MD Abstract: Electrophysiology remains an important tool in the evaluation of patients presenting with signs and symptoms of motor neuron disease. The electrodiagnostic study should include peripheral nerve conduction studies and needle electromyography to both exclude treatable disease and gather evidence regarding a diagnosis of amyotrophic lateral sclerosis (ALS). The recent changes in the revised El Escorial criteria, recommended by the Awaji-shima consensus group, have increased the diagnostic significance of fasciculation potentials to equal that of fibrillation and positive sharp-wave potentials in the needle electromyography examination of patients suspected of having ALS. In addition, electro- physiologic evidence is now considered equivalent to clinical signs and symptoms in reaching a diagnostic certainty of ALS. These changes, strategies for the design, and implementation of an effective electrodiagnostic evaluation, in addition to electrophysi- ologic techniques and their relationship to the evaluation of a patient with ALS, are reviewed and discussed. PM R 2013;5:S89-S95 INTRODUCTION Amyotrophic lateral sclerosis (ALS) is a progressive and inevitably fatal neurodegenerative disease belonging to a heterogeneous group of disorders known as motor neuron diseases (MNDs). ALS is the most common adult MND worldwide and is considered the prototypical disorder of this class, as demonstrated by the often-interchangeable use of the terms ALS and MND [1]. However, although ALS is characterized by the rapid degeneration of motor neurons throughout the neuraxis and by definition requires both upper motor neuron (UMN) and lower motor neuron (LMN) signs to confirm the clin