Ophthalmological exam, including perimetry if the child is able to perform it. CSF cytology and other testing may be warranted on the diagnostic lumbar puncture. The cerebrospinal fluid contents (glucose, protein, cell count) should be normal. Unless the child develops a post-spinal headache, the headache and other symptoms often improve (at least transiently) after the lumbar puncture. Pressures greater than 280 mm CSF are considered abnormally elevated and suspicious for pseudotumor cerebri. Lumbar puncture with opening pressure measurement following neuroimaging: There is a wide variation of normal opening pressure in children. What laboratory studies should you request to help confirm the diagnosis? How should you interpret the results? No known genetic abnormality although familial cases have been reported Medications: Vitamin A, tetracycline/minocycline/doxycycline, human growth hormone, corticosteroid withdrawal, thyroxine replacement, levonorgestrel implants, desmopressin nasal spray Girls are more frequently affected after puberty Prior to puberty, boys and girls are equally affected Incidence 1/100,000 in the general population What caused this disease to develop at this time? Post traumatic increased intracranial pressure Meningitis (infectious, inflammatory, malignant) Impaired cerebral venous outflow: cerebral venous sinus thrombosis, jugular vein thrombosis Medication-induced pseudotumor cerebri (see medications below) What other disease/condition shares some of these symptoms? There are nerve fiber layer hemorrhages, and edema in the papillomacular bundle producing a star formation. The optic disc margins are not apparent and major vessels are obscured by the edematous nerve fiber layer as they cross the disc margin. Severe (Frisen grade 5) papilledema in an adolescent with minocycline-induced pseudotumor cerebri. Magnified view of the exudate in the papillomacular bundle in the patient with severe papilledema. There is increased vascularity of the optic nerve, rendering a “hyperemic” appearance. Note that the optic nerve seems to bulge forward with a dark peripapillary halo. There is a dark peripapillary halo with engorgement of the retinal veins. The vessels crossing the disc margins are visible in their entirety. Next most common: Stiff neck, cranial nerve palsies (III, IV, VII, XII), torticollis, ataxia, enlarged and poorly reactive pupils Some children are asymptomatic with papilledema discovered on a routine eye examinationįigure 2, Figure 3, Figure 4), visual acuity loss, visual field loss, esotropia, abducens palsy Next most common: shimmering lights, visual loss, pulsatile tinnitus, neck pain, back pain, torticollis, ataxia
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