History
Common signs and symptoms of idiopathic intracranial hypertension (IIH) in the young include headache, vomiting, blurred vision, and diplopia. [3, 4]
Headaches are intermittent, diffuse, and worse at night; they may awaken the child and are often aggravated by sudden movement. Visual disturbances include transient visual obscurations, blurred vision, double vision, and photophobia. Diplopia is almost always horizontal (side by side) and is secondary to paresis of cranial nerve (CN) VI (the abducens nerve). It has been estimated that up to 50% of children with IIH have CN VI dysfunction.
Other symptoms of increased intracranial pressure (ICP) include lethargy, irritability, and vomiting. Nonspecific associated symptoms include neck stiffness, tinnitus, dizziness, clumsiness, and paresthesias.
The possible association of pseudotumor cerebri and signs and symptoms suggestive of varicella infection has previously been mentioned in a few case reports. Ravid S et al have described 3 immunocompetent children with pseudotumor cerebri as the only manifestation of varicella zoster virus reactivation. They have suggested considering varicella zoster virus in children with pseudotumor cerebri, even in the absence of a history of recent varicella infection. [21]
Physical Examination
The general medical examination may reveal signs of otitis media or mastoiditis, which raise the possibility of venous sinus thrombosis. The presence of acne vulgaris should prompt an inquiry about the possible use of retinoic acid or tetracyclines. Physical findings of adrenal or thyroid dysfunction may also be present.
The neurologic examination typically yields normal results, with the exception of papilledema and weakness of one or both of the abducens nerves. Obeid M et al reported 10-week-old monozygotic twins with cystic fibrosis, facial palsy, and increased intracranial pressure. [22] Low levels of vitamin A are associated with facial nerve paralysis and are at least partly implicated in the development of increased intracranial pressure in infants with cystic fibrosis. Other CN palsies have been reported on rare occasions.
Ophthalmoscopic (funduscopic) examination reveals optic disk nerve swelling (papilledema). The diagnosis of IIH should not be made in the absence of papilledema unless the patient has optic atrophy. Papilledema is typically bilateral but may be asymmetrical or unilateral. [17] Initially, visual acuity is usually preserved, which helps the clinician to distinguish acute papilledema from optic neuritis.
Visual field testing is useful for both examination and monitoring. Perimetry can be used to evaluate response to therapy. [5] Common field defects include enlargement of the blind spot, loss of the inferonasal portion and generalized constriction. Other defects include a variety of scotomas and altitudinal patterns of visual loss. Visual acuity assessment is helpful. Most patients will have some measurable visual loss that is often asymptomatic, usually occurs gradually, and improves with therapy. Komur et al have described coexisting optic disc drusen and idiopathic intracranial hypertension. [23]
Serial photographs of the fundus may be taken for follow-up. A related article is Anterior Segment and Fundus Photography.
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For IIH to be diagnosed, brain scans (such as MRI) must be performed to ensure there is no underlying cause for the increased pressure around the brain
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Left optic disc with moderate chronic papilledema in patient with idiopathic intracranial hypertension (pseudotumor cerebri). Paton lines (arc-shaped retinal wrinkles concentric with disc margin) are seen along temporal side of inferior pole of disc.
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Right optic disc with postpapilledema optic atrophy in patient with idiopathic intracranial hypertension (pseudotumor cerebri). Diffuse pallor of disc and absence of small arterial vessels on surface are noted, with very little disc elevation. Disc margin at upper and lower poles and nasally is obscured by some residual edema in nerve fiber layer and gliosis that often persists even after all edema has resolved.
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Most common early visual field defect in papilledema as optic nerve develops optic atrophy is inferior nasal defect, as shown in left eye field chart (left side of figure). Shaded area indicates defective portion of field. Note sharp line of demarcation between defective lower nasal quadrant and normal upper nasal quadrant along horizontal midline. This is characteristic of early papilledema optic atrophy and is referred to as nasal step or inferonasal step.
