Neurological involvement in the epidermal naevus syndrome.

The group of epidermal nevus syndromes

“…11 Affected children have a keratinocytic nevus of the orthokeratotic, nonepidermolytic category showing a bizarre, hystrix-like white or brownish hyperkeratosis (Fig 4), which is why this epidermal nevus (EN) has sometimes been described as ' 14 or of ''the ENS.'' 15,16 The angiodysplasia involves the large vessels with arteriovenous shunts and may cause death during the neonatal period. 12 Further clinical research may reveal whether NEVADA syndrome represents a distinct entity within the group of ENSs.…”

Epidermal nevus syndrome

“…Seizures, of any type, typically commence by the end of the first year of life. Other neurologic manifestations include hypotonia, 13 hyperkinesia, 14 hemiplegia, 10 hemiparesis, 15 cranial nerve palsies, 16 ventricular abnormalities, 11,17 cortical lesions, 18 and intracerebral calcification 19…”

Epidermal nevus syndrome

“…A child with patent ductus arteriosus had chronic occlusion of the left distal internal carotid artery that resulted in ipsilateral cerebral atrophy (Canyigit and Oguz, 2006). Another case of occlusion of the right internal carotid artery in the neck was discovered in a teenage girl with an extensive verrucous nevus affecting the right side of her body since birth (from the forehead to the foot) after a sudden, severe, right-sided headache, expressive dysphasia, and left-sided paresthesiae (McAuley et al, 1978). Fusiform dilated aneurysm of the left intracavernous carotid artery is associated with hypervascularity (Baker et al, 1987).…”