Stiff Neck and Torticollis as Unusual Presenting Symptoms of Pseudotumor Cerebri

Abstract

Source: Straussberg R, Harel L, Amir J. Pseudotumor cerebri manifesting as stiff neck and torticollis. Pediatr Neurol. 2002;26:225–227.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, which began 3 weeks before he complained of headache, neck pain and head tilt to the left. Funduscopic examination revealed papilledema and hemorrhages while computed tomography (CT) and magnetic resonance imaging (MRI) showed no mass effect (ie, no tumor or midline shift). Cerebrospinal fluid (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 normalized. Papilledema was reduced after 3 weeks of 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 was 280 mm water. Within one half-hour of removal of 7 ml CSF, symptoms were completely relieved and 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, and endocrine factors were absent. CT showed slit-like ventricles. CSF pressure was 480 mm water. Torticollis and neck pain resolved within 1 hour following lumbar puncture. He was asymptomatic and had normal fundi after 3 weeks of treatment with acetazolamide and prednisone.Pseudotumor cerebri should be considered in the differential diagnosis of acute onset of stiff neck or torticollis. (See also AAP Grand Rounds, October 2001;6:45). A recent review of 10 children with pseudotumor cerebri found 4 patients presenting with a stiff neck.l The classic presenting manifestations of pseudotumor cerebri are headache, vomiting, and papilledema. Torticollis is classified as congenital, traumatic, inflammatory, neurogenic, and other including Sandifer’s syndrome (hiatus hernia and gastroesophageal reflux).2 The most common form of torticollis of infancy is congenital and associated with a sternocleidomastoid fibrous tumor.3 If acquired later in childhood, apart from neurologic causes, torticollis can be associated with cervical lymphadenitis, paratonsillar and retropharyngeal abscesses, trauma, or ocular abnormalities such as paralysis of the superior oblique muscle.The more common neurologic disorders that may cause neck rigidity and torticollis are posterior fossa tumor, meningitis, subarachnoid hemorrhage, and syringomyelia. Paroxysmal, usually idiopathic and non-epileptic, torticollis is characterized by recurrent episodes of head tilt with onset in infancy and spontaneous remission in early childhood. Spasmodic torticollis may be a manifestation of dystonia, a rare cause in childhood.4Funduscopic examination for papilledema is recommended in children presenting with unexplained neck rigidity or torticollis, with or without headache. When a posterior fossa tumor is excluded by MRI scan, and the diagnosis of pseudotumor is suspected, lumbar puncture should result in the rapid relief of stiff neck and torticollis, while the papilledema should resolve after a short course of acetazolamide and steroids.These 3 cases of torticollis represent an interesting twist on the usual presentation of pseudotumor cerebri. We need to consider pseudotumor as a possibility in our school age and adolescent patients who present with torticollis without an obvious infectious cause.