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.


OBJECTIVE
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.


CONCLUSIONS
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.
Gebere-Michael
Sisay Gizaw
SG
Department of Neurology, Addis Ababa University, Addis Ababa, Ethiopia.
Johnston
James C
JC
Metaferia
Guta Zenebe
GZ
Wuhib
Mehila Zebenigus
MZ
Fernandez
Hubert Henery
HH
D001132:Arm / Q000294:innervation / Q000473:pathology* / Q000503:physiopathology; D002574:Cervical Vertebrae / Q000473:pathology / Q000503:physiopathology; D018450:Disease Progression; D007839:Functional Laterality / Q000502:physiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000473:pathology; D018482:Muscle, Skeletal / Q000294:innervation / Q000473:pathology* / Q000503:physiopathology; D009133:Muscular Atrophy / Q000473:pathology* / Q000503:physiopathology; D013115:Spinal Canal / Q000473:pathology / Q000503:physiopathology; D013116:Spinal Cord / Q000473:pathology* / Q000503:physiopathology; D013117:Spinal Cord Compression / Q000473:pathology* / Q000503:physiopathology; D013126:Spinal Nerve Roots / Q000473:pathology / Q000503:physiopathology; D055009:Spondylosis / Q000473:pathology* / Q000503:physiopathology