The authors report on female homozygous twins with idiopathic intracranial hypertension. At the age of 12 years, both twins simultaneously developed visual disturbances with photophobia. At the age of 19 years, an ophthalmological examination disclosed papilloedema in both their eyes. At the age of 22 years, a lumbar puncture showed raised CSF pressure over (200 mm H2O) in both twins. Their neurological and radiological examinations were extremely similar; both of them had severely impaired visual acuity and impaired visual field, bilateral optic nerve atrophy, intracranial hypertension, an enlarged and partial empty sella turcica, digital markings of the calvalium, and an enlarged frontal subarachnoid space. This is the first case report describing idiopathic intracranial hypertension occurring in homozygous twins.
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