<unk> <unk> , sometimes referred to as encephalotrigeminal angiomatosis, is an extremely rare congenital neurological and skin disorder. It is one of the phakomatoses, and is often associated with port-wine stains of the face, which is a capillary malformation, glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma.  It is caused by an arteriovenous malformation that occurs in the cerebrum of the brain on the same side as the physical signs described above.  Normally, only one side of the head is affected.
It is an embryonal developmental anomaly resulting from errors in mesodermal and ectodermal development. Unlike other neurocutaneous disorders (phakomatoses),<unk><unk>  does not have a hereditary tendency but occurs sporadically.
The diagnosis of<unk>  is established with neuroimaging techniques such as MRI with contrast, which shows the presence of leptomeningeal malformations in the brain.
Treatment of this is symptomatic such as seizures are treated with anti-epileptic medications, ocular symptoms are managed with surgical modalities, and skin manifestations are treated with the laser.
Prognosis of<unk>  is dependent on the age of onset of seizures, the extent of the brain, and dermatological involvement.
Sturge-Weber syndrome
Landau-Kleffner syndrome
Braun-Bayer syndrome
Turcot syndrome
Saethre-Chotzen syndrome
Cruveilhier-Baumgarten syndrome
Treacher Collins syndrome
Prader-Willi syndrome