Isolated Neck Extensor Myopathy Associated With Cervical Spondylosis: A Case Report and Brief Review

Dropped head syndrome (DHS) is manifested as the inability to maintain the head in an upright posture. It has been associated with a wide spectrum of myopathies and neurological conditions. Isolated neck extensor myopathy (INEM) is one of many potential causes of DHS. This is a case report of a 72-year-old man who presented with degenerative cervical spondylosis and DHS for 2 years. He had previously failed to achieve a positive outcome to medication, cervical collar and exercise rehabilitation. However, he was able to regain his voluntary head control after a 4-month chiropractic program. It is believed that INEM is caused by isolated myopathic changes from chronic injury and overloading of the cervical muscles. Cervical spondylosis has been attributed as the cause of DHS secondary to denervation of the cervical extensors. While INEM associated with degenerative spondylosis is not medically treatable, manipulative therapies may be adopted before considering surgical intervention.

SURGICAL CORRECTION OF SEVERE AND FIXED CHIN-ON-CHEST DEFORMITY

ABSTRACT Objective: To describe a successful surgical treatment for the challenging severe and fixed chin-on-chest deformity due to isolated neck extensor myopathy (INEM). Background data: INEM is an idiopathic cause of dropped head syndrome (DHS) that results in severe cervicothoracic kyphosis, defined as chin-on-chest deformity. The existing literature on surgical management is limited, with outcomes ranging from poor to excellent. INEM may present to the spinal surgeon for consideration of surgical management. Methods: The authors present a technique that uses a staged posterior and anterior approach combined with osteotomies and corpectomy to correct the severe and fixed deformity. A state of the art anterior and posterior instrumentation system was used. Results: At the three-months follow-up, there was good deformity correction and the patient's satisfaction was high, with no neurological deterioration occurring. Conclusions: The technique illustrated in this study represents a successful option to treat this debilitating deformity. More evidence is needed to set up a definitive algorithm for the management of this condition. Level of evidence IV, Case Report.

Cervical myelopathy caused by dropped head syndrome

✓The authors present a rare case of cervical myelopathy caused by dropped head syndrome. This 68-year-old woman presented with her head hanging forward. After 1 month, she was admitted to the medical service because of head drop progression. Examination of biopsy specimens from her cervical paraspinal muscles showed nonspecific myopathic features without inflammation, and isolated neck extensor myopathy was diagnosed. The patient’s condition did not respond to the administration of corticosteroids. During follow up as an outpatient, the patient’s head drop continued to gradually progress. At 1 year after onset, she developed bilateral weakness of the upper and lower extremities, clumsiness of the hands, and gait disturbance. A radiograph of the cervical spine obtained in a standing position showed a pronounced kyphotic deformity and instability at the level of C4–5. Magnetic resonance imaging demonstrated spinal cord compression at C-3 and C-4. The patient underwent a C3–4 laminectomy and occipitocervicothoracic fixation. Gait and hand coordination gradually improved, and she was able to walk with no support 1 month postoperatively. Surgical fixation was beneficial in this patient with dropped head syndrome, myelopathy, and cervical instability.