Cervical spondylotic amyotrophy presenting as dropped head syndrome.
ABSTRACT We report a case of acute-onset dropped head syndrome in a 65-year-old patient in whom the diagnosis of amyotrophic lateral sclerosis (ALS) was initially proposed based on electromyographic signs of neck and shoulder muscle denervation. There were no signs of pyramidal involvement and the clinical and electromyographic signs of motor denervation never evolved beyond the neck and shoulder girdle muscles after a 6-year follow-up period, which argued against ALS. Other causes of dropped head syndrome were carefully ruled out based on clinical findings, electrodiagnostic studies and blood investigations. The restriction of muscle denervation to a few cervical myotomes, the presence of C3-C4 spondylotic changes without associated root or spinal cord compression, and the absence of an alternative explanation to the patient's symptoms strongly supported the diagnosis of cervical spondylotic amyotrophy (CSA). CSA is thought to result from spinal cord microcirculatory disturbances and secondary anterior horn cell degeneration due to ischemia. Our case enlarges the clinical spectrum of focal cervical anterior horn disease, which classically results in more distal monomelic atrophy affecting one or both upper limbs.
Article: Cervical spondylotic amyotrophy.[show abstract] [hide abstract]
ABSTRACT: Cervical spondylotic amyotrophy is characterized with weakness and wasting of upper limb muscles without sensory or lower limb involvement. Two different mechanisms have been proposed in the pathophysiology of cervical spondylotic amyotrophy. One is selective damage to the ventral root or the anterior horn, and the other is vascular insufficiency to the anterior horn cell. Cervical spondylotic amyotrophy is classified according to the most predominantly affected muscle groups as either proximal-type (scapular, deltoid, and biceps) or distal-type (triceps, forearm, and hand). Although cervical spondylotic amyotrophy always follows a self-limited course, it remains a great challenge for spine surgeons. Treatment of cervical spondylotic amyotrophy includes conservative and operative management. The methods of operative management for cervical spondylotic amyotrophy are still controversial. Anterior decompression and fusion or laminoplasty with or without foraminotomy is undertaken. Surgical outcomes of distal-type patients are inferior to those of proximal-type patients.European Spine Journal 03/2011; 20(3):351-7. · 1.97 Impact Factor