The Association Between the Rotator Cuff Status and the Severity and Recovery of Weakness in the Shoulder Abductor Strength in a Case of Proximal Type Cervical Spondylotic Amyotrophy

Study Design: Case series study. Objective: We aimed to clarify the prevalence of rotator cuff tear (RCT), and the association between RCT and the severity and prognosis in patients with proximal type cervical spondylotic amyotrophy (CSA). Methods: We retrospectively analyzed 35 proximal type CSA patients who were treated conservatively. The following data was collected: age, rotator cuff status on MRI, manual muscle test (MMT) score of shoulder abductor and biceps brachii muscles both at the first visit and final follow-up. We investigated the prevalence of RCT and the association between the rotator cuff status, and the severity and recovery of upper extremity weakness in patients with proximal type CSA. Results: Of the 35 patients, 21 had an RCT on MRI, indicating that the prevalence of RCT in patients with proximal type CSA was 60%. An age-adjusted analysis showed that the presence of RCT was significantly associated with the MMT score of the shoulder abductor muscles both at the first visit and at the final follow-up. The presence of RCT was significantly associated with the recovery of the shoulder abductor muscles. The size of the RCT was negatively correlated with the MMT score of the shoulder abductor muscles at the final follow-up. The size of the RCT was independently correlated with the change of the MMT score of the shoulder abductor muscles. Conclusion: RCT was detected in >50% in patients with proximal type CSA, and the presence and severity of RCT can be used as prognostic factors for proximal type CSA patients who are treated conservatively.

A Case of Cervical Spondylotic Amyotrophy Mimicking Amyotrophic Lateral Sclerosis

Cervical spondylotic amyotrophy (CSA) is a rare clinical condition characterized by weakness and atrophy of the upper limb with minimal to no associated sensory deficits. The detection of the disease is based on clinical features at presentation, neurological examination, electrophysiological studies, and imaging. The proposed pathophysiological mechanisms include selective damage to the ventral root or anterior horn cells of the spinal cord. Depending on the muscle groups that are involved, CSA is broadly classified into a proximal type and a distal type. The clinical profiles of patients with CSA and ALS have a very close resemblance to each other, especially at the early stages of the disease. Cervical spine magnetic resonance imaging (MRI) may show T2 hyperintensity in both proximal and distal types. Electromyogram demonstrates denervation potentials and reduced motor unit potentials in the affected muscles. The conservative management is often the first-line modality, and those who fail to respond to conservative treatment have severe muscular atrophy and weakness, and distal-type CSA are considered potential candidates for surgery. We present the case of a 57-year-old female who presented with a 1-year history of left-hand weakness and wasting with no sensory deficits. She denied any involvement of her other hand or bilateral lower limbs, and she was referred to our clinic with the potential diagnosis of amyotrophic lateral sclerosis (ALS). An elaborate history, physical examination, electrophysiological studies, and imaging assisted us in reaching the diagnosis of CSA, 1 year after the onset of symptoms.