A Woman Who Had to Hold Her Head With Her Hands

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hold her head
Initial T2-weighted image with evidence of spondylosis; No evidence of cord signal change.

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.

Mid-sagittal T2-weighted image showed progression of spondylosis with distinct T2 hyperintensity of the cord at C3–C4. Evidence also of fat infiltration of the paraspinal muscles in the lower cervical spine region.

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.

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Dr. Arsia Parekh
Dr. Arsia Hanif has been a meritorious Healthcare professional with a proven track record throughout her academic life securing first position in her MCAT examination and then, in 2017, she successfully completed her Bachelors of Medicine and Surgery from Dow University of Health Sciences. She has had the opportunity to apply her theoretical knowledge to the real-life scenarios, as a House Officer (HO) serving at Civil Hospital. Whilst working at the Civil Hospital, she discovered that nothing satisfies her more than helping other humans in need and since then has made a commitment to implement her expertise in the field of medicine to cure the sick and regain the state of health and well-being. Being a Doctor is exactly what you’d think it’s like. She is the colleague at work that everyone wants to know but nobody wants to be. If you want to get something done, you approach her – everyone knows that! She is currently studying with Medical Council of Canada and aspires to be a leading Neurologist someday. Alongside, she has taken up medical writing to exercise her skills of delivering comprehensible version of the otherwise difficult medical literature. Her breaks comprise either of swimming, volunteering services at a Medical Camp or spending time with family.

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