Investigators from the University of Texas Southwestern Medical Center, Dallas, and centers in Philadelphia and Salt Lake City, propose updated criteria for the diagnosis of pseudotumor cerebri (PTCS) and its variations. Idiopathic intracranial hypertension (IIH) is an appropriate term for a subset of patients with primary IH of unclear etiology, but not for those precipitated by an identifiable secondary cause. The syndrome is best described using the umbrella term PTCS. Required criteria for the diagnosis of PTCS are as follows: a) Papilledema, b) Normal neurologic examination (except cranial nerves), c) Normal MRI (or CT), d) Normal CSF composition, and e) Elevated lumbar puncture opening pressure (>250 mm for adults; >280 mm for children [250 if not sedated and not obese]).

With no papilledema, diagnosis requires (b) to (e) satisfied plus abducens nerve palsy.

Uncommon manifestations include a facial nerve palsy, hemifacial spasm, or radicular pain. CSF rhinorrhea or otorrhea and confirmation of a CSF leak are highly suggestive of PTCS diagnosis. Papilledema may be absent in recurrent PTCS cases because of gliosis in the nerve or optic atrophy.

Neuroimaging abnormalities highly suggestive of PTCS are: 1) Empty sella. 2) Flattening of posterior aspect of the globe, 3) Distension of the perioptic subarachnoid space, and 4) Transverse venous sinus stenosis. Tonsillar ectopia is more frequent in cases of PTCS but is not specific; it may be a sign of low CSF pressure, and may indicate an increased risk for herniation with LP. [1]

COMMENT. Present diagnostic criteria and guidelines for the management of pediatric PTC do not consider pediatric aspects, according to a study involving all pediatric hospitals in Germany [2]. The annual incidence was 0.5 per 100,000 children. A wide range of vision problems included papilledema, visual loss, diplopia, visual field defect, disturbed color and stereo vision. Papilledema was absent in 10 (16.4%) of a total of 61 patients treated Jan to Dec 2008. The importance of the ophthalmological exam was emphasized.

In a study of 42 patients with average age at onset of 10.8 years (range, 12 months to 17 years), obesity was found in 11 (26%). Headache occurred in 76%. Various etiologic factors were associated, including trauma in 2, hypervitaminosis A, corticosteroid withdrawal, oral contraceptives, Guillain-Barre syndrome, urinary tract infection, varicella-zoster virus infection, and dural venous sinus thrombosis associated with otitis media. Prompt diagnosis and medical treatment are important to avoid visual loss. [3]

An unusual case of a 13-year-old boy presented with acute paresis of the left abducens, facial and vagus nerves. Brain MRI and angiography were normal. LP revealed an intracranial pressure of 575mmH2O. Treatment with acetazolamide resulted in improvement with no sequelae. [4]