A case of acute febrile neck pain

Abstract

An 80-year-old woman presented with a 2-month history of worsening pain of her neck, associated with a 3-week history of hypomobility, and a progressive hyposthenia of the upper and the lower limbs. For this reason, she was admitted to our department. The past history was positive for osteoarthritis and hypertension. The patient was febrile (up to 39°C), and she complained about sharp severe neck pain causing rigidity and preventing neck movement. The physical examination revealed plegia of both upper limbs, hyperreflexia, and paresis of lower limbs and bilateral positive Babinski signs. Blood laboratory tests showed highly elevated acute phase proteins. Computed tomography (CT) of the neck showed atlanto-axial diffused and crown-like calcifications around the dens, which appeared largely eroded, but were kept in place by calcifications. No signs of atlanto-occipital and atlanto-axial subluxation were present (Fig. 1a, b). Numerous nodular-shaped calcium depositions were also present along the ligamenta flava, around the glenohumeral joints, the sternum, the pelvis, and within the small articulations of her hands (not shown). A diagnosis of severe systemic chondrocalcinosis with crowned dens syndrome (CDS) was made. Because of the clinical suggestion of medullar compression, she underwent magnetic resonance of the cervical tract, which showed anterior compression of the spinal cord by the pannus determined by the calcium deposition associated with hypersignal intensity on T2-weighted images at C1-C2 level (Fig. 1c). Bolus corticosteroids (methylprednisolone 500 mg/daily) and oral colchicine (1 mg/daily) were administered for 1 week without any clinical improvement.