Most patients had extremely extensive cerebral abnormalities with only minimal symptomatology. Ophthalmologic fundus examination may reveal papilledema or optic nerve pallor. Biopsy specimens taken from the alopecic area of the scalp reveal focal dermal fibrosis associated with increased amounts of subcutaneous fat extending into the upper reticular dermis. The cutaneous and ocular findings are limited to 1 hemicranium and are ipsilateral to the developmental defects of the brain. The histologic examination of the brain performed in a case reported by Haberland and Perou revealed a defective lamination of the cerebrum, polymicrogyria, and calcification in the outer cerebral cortex overlying the porencephalic cyst.
Antiepileptic treatment is administered in patients with clinical epileptic fits.
Antiarrhythmic treatment may be required in patients with cardiac rhythm abnormalities. Selected patients with drug-resistant epilepsy may benefit from neurosurgical intervention, as reported by Roszkowski et al. Consult neurosurgery, neurology, ophthalmology, and cardiology as determined by history and physical examination. No special activity limitations are required for most patients. No further inpatient care is needed except as indicated by associated abnormalities.