Cervical spondylotic amyotrophy (CSA) is characterized by upper limb muscle weakness and atrophy, without sensory deficits. The pathophysiology of CSA has been attributed to selective injury to the ventral nerve root and/or anterior horn of the spinal cord. This review aimed to delineate the history of CSA and to describe the epidemiology, etiology, pathophysiology, classification, clinical features, radiological and electrophysiological assessment, diagnosis, differential diagnosis, natural history and treatment of CSA.
Methods
A comprehensive search of PubMed, EMBASE, Cochrane library and Web of Science databases was conducted, from their inception to April 3, 2018.
Results
Clinically, CSA is classified into three types: a proximal-type (involving the scapular muscles, deltoid and biceps), a distal-type (involving the triceps and muscles of the forearm and hand) and a diffuse-type (involving features of both the distal- and proximal-type). Diagnosis requires documentation of muscle atrophy, without significant sensory deficits, supported by careful neurological, radiological and neurophysiological assessments, with amyotrophic lateral sclerosis, Parsonage–Turner syndrome, rotator cuff tear and Hirayama disease being the principle differential diagnoses. Conservative management of CSA includes cervical traction, neck immobilization and physical therapy, with vitamin B12 or E administration being useful in some patients. Surgical treatment, including anterior decompression and fusion or laminoplasty, with or without foraminotomy, is indicated after conservative treatment failure. Factors associated with a poor outcome include the distal-type CSA, long symptom duration, older age and greater preoperative muscle weakness.
Conclusion
Although the disease process of CSA is self-limited, treatment remains challenging, leaving scope for future studies.
Graphical abstract
These slides can be retrieved under Electronic Supplementary Material.
