Three prepubertal children diagnosed with pseudotumor cerebri and presenting with stiff neck and torticollis are reported from Schneider Children’s Medical Center, Sackler School of Medicine, Tel Aviv, Israel. Patient 1. a 7-year-old male admitted with stiff neck, had been evaluated at 2 years of age for short stature and treated first with thyroxine and later, with growth hormone injections, starting 3 weeks before complaining of headache, neck pain and head tilt to the left. Funduscopic examination revealed papilledema and hemorrhages. CT and MRI showed no mass effect. CSF opening pressure was 340 mm water, with normal glucose and protein. Following withdrawal of 6 ml CSF, the rigidity and neck pains resolved and neck movements were normal. Papilledema was reduced after 3 weeks treatment with acetazolamide and dexamethasone. Patient 2. a 9-year-old previously healthy female had a 10 day history of neck pain followed by headache, neck stiffness, and papilledema. CT was normal and CSF pressure 280 mm. Within one half hour of removal of 7 ml CSF, symptoms were completely relieved. Symptoms did not recur during subsequent treatment with acetazolamide and dexamethasone. Patient 3. an 8-year-old male was admitted with a 6 week history of headaches, torticollis for 4 weeks, right sided neck pain and papilledema. Known causes of pseudotumor, including trauma, infection, vitamins, and endocrine factors, were absent. CT showed slit-like ventricles. CSF pressure was 480 mm. Torticollis and neck pain resolved within 1 hour after lumbar puncture. He was asymptomatic and had normal fundi at 3 week follow-up, following therapy with acetazolamide and prednisone. [1]

COMMENT. Pseudotumor cerebri should be considered in the differential diagnosis of acute onset of stiff neck or torticollis. A recent review of 10 children with pseudotumor cerebri, cited by the authors, found 4 patients presenting with stiff neck [2]. The mechanism of the stiff neck and torticollis and the association with prepubertal cases of pseudotumor are undetermined. The more classical presenting manifestations are headache, vomiting, and papilledema. Other more common neurologic disorders that underly neck rigidity and pain and/or torticollis are cervical trauma or inflammation, meningitis, subarachnoid hemorrhage, posterior fossa tumor, spinal cord syrinx, and cervical radiculitis. Funduscopic examination for papilledema is recommended in children presenting with unexplained neck rigidity or torticollis, with or without headache. The rapid relief of symptoms following lumbar puncture and the resolution of papilledema following a short course of acetazolamide and steroids are noteworthy.