We report two cases of epidermal nevus syndrome (ENS) involving the spine. MR imaging of the spine demonstrated intraspinal lipomas in both cases. Abnormal, enhancing, enlarged cervical and lumbosacral nerve roots were present in one patient. Spinal imaging for patients with ENS may help in the diagnosis of subtle intracranial manifestations, as it did in both of our cases. ENS has features sim...

Corresponding Author: B. Basiri MD Tel: +98 9188121848 Email: [email protected] Abstract Objective Incontinentia Pigmenti (IP) (Bloch_Sulzberg syndrome) is a rare neurocutaneous syndrome characterized by multisystemic involvement that is prenatally lethal in the majority of affected males and shows great clinical variability when expressed in women. The diagnosis of IP is performed based o...

Corresponding Author: B. Basiri MD Tel: +98 9188121848 Email: [email protected] Abstract Objective Incontinentia Pigmenti (IP) (Bloch_Sulzberg syndrome) is a rare neurocutaneous syndrome characterized by multisystemic involvement that is prenatally lethal in the majority of affected males and shows great clinical variability when expressed in women. The diagnosis of IP is performed based o...

Sturge-Weber syndrome, encephalotrigeminal angiomatosis, is a neurocutaneous disorder characterized by a facial cutaneous vascular nevus, seizures, mental retardation, hemiplegia, homonymous hemianopia, and buphthalmos or glaucoma (1). The clinical findings are related to vascular anomalies of the face, ocular choroid, and leptomeninges. The imaging findings in Sturge-Weber syndrome may include...

Neurocutaneous melanosis (NCM) is a rare, congenital non-hereditary syndrome, characterized by multiple pigmented nevi. We report the radiologic findings of a newborn who had extensive cutaneous melanotic nevus with satellite lesions in the brain. Ultrasound showed multiple echogenic foci in the cerebral parenchyma. Subsequent MRI confirmed these lesions as characteristic deposits of melanin. T...

Neurocutaneous melanosis is a rare congenital syndrome characterized by large or multiple congenital melanocytic nevi and benign or malignant pigment cell tumors of the leptomeninges. The prognosis is extremely poor for symptomatic patients, even in the absence of malignant melanoma. We present serial MR imaging findings in the brain and spine of a child with congenital giant hairy nevi who dev...

Tuberous sclerosis (TS) is an autosomal dominant neurocutaneous syndrome characterized by multiorgan hamartomas, epilepsy and mental retardation. Prenatal genetic testing for a mutation is laborious, time consuming and not widely available because of the genetic heterogeneity, polymorphism and a high rate of denovo mutations(1,2). We hereby highlight the utility of prenatal magnetic resonance i...