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.