Abstract

A 10-year-old African-Canadian girl experienced a sudden onset of neck stiffness. There was no history of preceding trauma, but she had reported a sore throat and hoarse voice three weeks earlier. Over the next year, she developed progressively worsening torticollis with associated neck pain, daily headaches and intermittent diplopia. She was taking ibuprofen two to three times daily for head and neck pain with partial improvement. She presented to the emergency department 13 months after onset because her head was now locked in position. There was no history of fevers, weight loss, skin rash, joint pain, muscle weakness, sensory deficits or gait abnormalities. On physical examination, the patient’s head was fixed in position with her chin rotated toward the right and her head tilted toward the left. There was minimal tenderness over her left sternocleidomastoid muscle and no palpable neck mass. Her neurological examination, including cranial nerve testing, was normal. She had no active or effused joints, enthesitis or tenosynovitis. An ophthalmological assessment, including visual acuity, was also unremarkable. Laboratory investigations revealed an increased erythrocyte sedimentation rate of 35 mm/h, but her C-reactive protein, complete blood count, creatine kinase and transaminase levels were normal. Plain radiographs showed a loss of cervical lordosis and abnormal alignment of C1–C2. Further imaging revealed the underlying diagnosis.

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