Cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses – resulting in facial asymmetry – and elevation of the sphenoid wing and petrous ridge, in association with contralateral hemiplegia, seizures, mental retardation, difficulty and impairment of speech development.

Both sexes affected; present from birth.

Aetiology unknown but may be associated with

1)atrophy of ipsilateral cerebral hemisphere (in childhood)

2)cerebral infarction (most often)

3)infection

4)trauma

5)congenital hypoplasia