A 38-year-old woman got a diagnosis of dropped head syndrome secondary to myopathy when she presented with complaints of unable to hold her head and that she had to hold her head with her hands.
A 38-year-old woman presented with complaints of severe chronic cervical pain along with a recent history of cervical deformity and gait problems. The patient described a gradually worsening intense tightness around the neck coupled with muscular contractions. She had a progressive neck over the past 4 years. However, it had worsened during the recent months before the presentation. She had trouble moving as it was difficult for her to hold her head up. However, there were no motor or sensory abnormalities in any other part of the body, including the extremities.
On physical examination, she had significant cervical kyphosis with hypertrophic neck extensors. The examiner was successful in reducible the deformity completely. The patient exhibited a 50% reduction in the active cervical flexion and extension with pain at the extremes.
All extremities demonstrated normal muscle bulk, tone, and power. However, mild global hyperreflexia was appreciated with positive Hoffmann reflexes bilaterally and downward Babinski reflexes bilaterally. Sensations were intact.
Cervical spine X-ray indicated marked cervical kyphosis centred on the mid-cervical spine. Moreover, it demonstrated evidence of spondylosis. Magnetic resonance imaging (MRI) showed multilevel cervical spondylosis with cerebrospinal fluid effacement at the mid-cervical levels. However, MRI did not show any change in cord signal. The physicians made a working diagnosis of cervical dystonia causing dropped head syndrome with subtle signs of myelopathy.
A trapezius muscle biopsy ruled out evidence of inflammatory myopathy. It showed nonspecific atrophic changes with fibrosis. Initial electrodiagnostic findings were also nonspecific.
During the next 2 years, the patient became progressively disabled with a functional decline to the extent that she could only ambulate short distances that too while holding her head up with both hands. Clinical examination revealed an increase in the left arm’s hyperreflexia. Repeated MRI showed hyperintensity of the cord at C3–C4 and atrophy with fat infiltration of the paraspinal muscles in the lower cervical spine.
The repeated electrodiagnostic assessment, which was initially unspecific, showed fibrillation and small amplitude polyphasic potentials in the paraspinal muscles. The results suggested that myopathy was the cause of the dropped head syndrome.
