Other etiologies that cause focal structural changes are poorly suited to medical treatment, such as neck radiation therapy causing extensor myopathy and anterior scar contracture. In particular, Parkinson disease, inflammatory myositis, and secondary myopathies due to metabolic or endocrine dysfunction are likely to show substantial improvement when treated with appropriate medications. Many of the underlying conditions are medically treatable and in some cases the extent of the deformity may be decreased to an acceptable level without invasive intervention. Optimal management of a patient with DHS requires a comprehensive multidisciplinary workup to establish the primary cause before surgical decision making. Imaging at follow-up shows the correction and stabilization of the deformity ( Fig 4).ĭropped head syndrome is a complex and challenging condition. She regained full mobility, and at 8 months follow-up she reported increased energy and a significantly improved ability to carry out daily activities. She was managed for 8 weeks postoperatively in a cervico-thoracic orthosis. Computed tomography confirmed good positioning of all hardware, and anatomical alignment with increased space in the spinal canal. Postoperatively she recovered well with no neurological deficits. Intraoperative electromyographic recordings showed transient-spiking activity during the procedure but this resolved fast. The surgical procedure was uncomplicated, with motor and sensory-evoked potentials maintained throughout. Good reduction of the cervical deformity with neutral head alignment was achieved via direct visualization and image intensification. She underwent a C2 to T1 decompressive laminectomy, and occiput to T6 posterior instrumented fixation and fusion. The patient consented to surgical intervention to achieve the goals of decompression of neural structures, and correction of the cervical deformity with stabilization. Evidence also of fat infiltration of the paraspinal muscles in the lower cervical spine region. Subtle progression of spondylosis, now with distinct T2 hyperintensity of the cord at C3–C4. Magnetic resonance imaging (mid-sagittal T2-weighted image) from 2010. Repeated electrodiagnostic assessment showed fibrillation and small amplitude polyphasic potentials in the paraspinal muscles suggesting myopathy as the cause of DHS. 3) showed distinct T2 hyperintensity of the cord at C3–C4 and atrophy with fat infiltration of the paraspinal muscles in the lower cervical spine. Clinical examination showed an increase in hyperreflexia of the left arm but was otherwise unchanged. Over 2 years she became progressively disabled with decline in her functional state such that she could only ambulate short distances while holding her head up with both hands. A muscle biopsy from the trapezius showed no evidence of inflammatory myopathy and nonspecific atrophic changes with fibrosis which were considered nondiagnostic. Initial electrodiagnostic findings were nonspecific. Evidence of spondylosis with cerebrospinal fluid effacement at the mid-cervical levels, although no evidence of cord signal change. Magnetic resonance imaging (mid-sagittal T2-weighted image) from 2007, when the patient initially presented. A preliminary diagnosis of cervical dystonia causing DHS with subtle signs of myelopathy was considered, and further investigations to determine the primary pathology were organized. 2) showed multilevel cervical spondylosis with cerebrospinal fluid effacement at the mid-cervical levels but no evidence of cord signal change. 1) indicated marked cervical kyphosis centered on the mid-cervical spine, and evidence of spondylosis. She had mild global hyperreflexia and positive Hoffmann reflexes bilaterally, but downward Babinski reflexes bilaterally. Muscle bulk, tone, and power were normal in all four limbs. Active cervical flexion and extension were reduced to 50% of normal and painful at extremes. The deformity was completely reducible by the examiner. On examination she was found to have pronounced cervical kyphosis with hypertrophic neck extensors. However, she testified no motor or sensory dysfunction in any extremity. She also described trouble ambulating, primarily due to difficulty holding up her head. Her neck deformity had been slowly progressive over the last 3 to 4 years but had worsened in recent months. She gave a decade long history of gradually worsening intense tightness around the neck and muscular contractions. A 38-year-old woman presented to the senior author's spinal cord clinic with a history of severe chronic cervical pain and a recent history of cervical deformity and gait abnormalities.
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