Epidermal nevus syndrome and intraspinal hemorrhage.
The epidermal nevus syndrome (ENS) is a rare neurocutaneous disease characterized by extensive epidermal nevi and a wide variety of abnormalities involving brain, eyes, and skeleton. Neurological symptoms in ENS include seizures, paresis, and mental retardation and are usually ascribed to hemimegalencephaly and various migration disorders. It was suggested that in some patients neurological symptoms might be secondary to vascular abnormalities. We report a case of a patient with diagnosed ENS without any primary CNS lesions, who developed paraplegia resulting from spinal cord hemorrhage. The patient presented many vascular and skeletal anomalies.
Kotulska
Katarzyna
K
Department of Neurology, The Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-730 Warsawa, Poland. k.kotulska@czd.pl
Jurkiewcz
Elzbieta
E
Jóźwiak
Sergiusz
S
Kuczyński
Dariusz
D
D002648:Child; D005260:Female; D046748:Hematoma, Epidural, Spinal / Q000150:complications* / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D018330:Nevus, Intradermal / Q000150:complications* / Q000473:pathology