We report a case of epidermal nevus syndrome involving the brain in which there is chronic occlusion of the left distal internal carotid artery resulting in ipsilateral atrophy. Orbital and cerebellopontine angle cistern lipomas and a wide cortical developmental malformation are associated with the condition. We present MR imaging findings of a patient and discuss features in the context of oth...

Sturge Weber Disease is one of the important neurocutaneous syndrome characterized by abnormities of both the integument and central nervous system. We present here a classical case of Sturge Weber Disease. This 16 month male child had facial angioma and seizures. CT Scan head was consistent with the diagnosis.

A 15-month-old girl presented with developmental delay, intractable right focal motor seizures, hyperpigmented patch on the left side of the face, facial asymmetry, and right hemiparesis (figure 1). A diagnosis of linear nevus sebaceous syndrome (LNSS) was considered. Brain MRI revealed hemimegalencephaly (figure 2). EEG showed spike/wave discharges in left occipital leads. Ophthalmologic and s...

Encephalocraniocutaneous lipomatosis or Haberland syndrome is a rare, congenital neurocutaneous syndrome. It is characterized by unilateral lipomatous hamartomata of the scalp, eyelid, and outer globe of the eye and ipsilateral neurologic malformations. We describe the first case from Lebanon, an infant with classical encephalocraniocutaneous lipomatosis characterized by nevus psiloliparus, uni...

PURPOSE To report a rare case of encephalocraniocutaneous lipomatosis (ECCL) presented with characteristic multiple organ involvement. METHODS A 7-day-old white Iranian girl was referred with ocular, skin and brain abnormalities. RESULTS The findings of nevus psiloliparus, eyelid choristoma and intracranial lipoma were consistent with ECCL. CONCLUSION Since the skin and ocular manifestati...

BACKGROUND Neurocutaneous melanosis (NCM) is a rare neurocutaneous syndrome characterized by the presence of multiple congenital melanocytic nevi (CMN) and the proliferation of melanocytes in the central nervous system, usually involving the leptomeninges. Chronic partial epilepsy as a sole manifestation is rare in NCM. CASE REPORT A 32-year-old man suffering from chronic partial epilepsy pre...

Ó Abstract Sturge-Weber syndrome (SWS) also known as encephalotrigeminal angiomatosis. It is a neurocutaneous syndrome, characterized by a facial vascular birthmark and neurological abnormalities. An ipsilateral or bilateral facial cutaneous vascular malformation Port Wine Stain (PWS) usually affects the upper face. Other clinical manifestations are seizures, glaucoma, hemiparesis, mental retar...

A 58-year-old woman with a history of migraine presented with headache, aphasia, right homonymous hemianopsia, and right hemiparesis, followed by 2 seizures. On examination, she was noted to have a facial angioma (figure 1) that was present from childhood. MRI findings (figure 2, A–C) were consistent with leptomeningeal angiomatosis, making the diagnosis of Sturge-Weber syndrome. Sturge-Weber s...

Dandy-Walker Malformation (DWM) is a rare congenital malformation of the brain. It is characterized by cystic enlargement of the fourth ventricle which is communicating with an enlarged posterior fossa, cerebellar dysgenesis, high tentorial insertion and hydrocephalus. Neurocutaneous Melanosis (NCM) is a congenital neurocutaneous syndrome characterized by large or multiple melanocytic nevi and ...

Background: The neurocutaneous disorders, also known as the phakomatoses, are a group of hereditary conditions that have neurological manifestations as well as benign cutaneous lesions; both tissue types share a common embryologic origin. Neurofibromatosis: Neurofibromatosis (NF) is the most common neurocutaneous disorder. Although there are as many as 8 distinct forms of NF, the term is most c...