Bilaterally symmetric cervical spondylotic amyotrophy: A novel presentation and review of the literature

Background: Cervical spondylotic amyotrophy (CSA) is considered a syndrome of (1) unilateral upper extremity weakness and atrophy, (2) affecting either the proximal or distal musculature, (3) without sensory impairment or lower extremity dysfunction. Aims of study: The authors report a novel case of bilaterally symmetric CSA with blurring of the proximal-distal distinction, discuss the pathophysiology, and review the literature. Methods: A 45 year old man presented with a several year history of insidiously progressive bilaterally symmetric upper extremity weakness and wasting, profound in the proximal musculature and moderate to severe in the distal muscle groups. Results: Based on the clinical, neuroimaging and electrodiagnostic features, this patient harbors a more severe phenotype of the classical syndrome. Conclusion: The authors propose expanding the generally accepted definition of CSA to include this bilaterally symmetric form of disease, thereby minimizing diagnostic error or delay. Additionally, based on this case and a review of the literature, adherence to the proximal-distal distinction should be avoided since it is commonly blurred. Accurate diagnosis is crucial since this presentation mimics the motor neuron disease variant Vulpian-Bernhardt syndrome. The importance of early diagnosis is underscored by reports of significant improvement with timely surgical decompression. (C) 2009 Elsevier B.V. All rights reserved.