Neuroimaging features of epidermal nevus syndrome.
Epidermal nevus syndrome is a kind of neurocutaneous syndrome that is associated with epidermal nevus and a variety of congenital CNS disorders. Clinical presentations include seizures, paresis, mental retardation, and developmental delay. We report three cases with MR imaging and magnetoencephalography findings; one patient underwent ictal and interictal single photon emission CT. Both structural and functional imaging studies indicated that the frontal lobes had lesser involvement or were intact. One patient underwent hemispherectomy because of the medically intractable seizure. He remained seizure free with topiramate monotherapy.
Zhang
Wenbo
W
Department of Neurosurgery, Vivian L. Smith Center for Neurologic Research, Texas Comprehensive Epilepsy Program, The University of Texas Health Science Center at Houston, Houston, TX 77030, USA.
Simos
Panagiotis G
PG
Ishibashi
Hideaki
H
Wheless
James W
JW
Castillo
Eduardo M
EM
Breier
Joshua I
JI
Baumgartner
James E
JE
Fitzgerald
Michele E
ME
Papanicolaou
Andrew C
AC
D000015:Abnormalities, Multiple / Q000175:diagnosis*; D002490:Central Nervous System / Q000002:abnormalities* / Q000000981:diagnostic imaging; D002675:Child, Preschool; D004817:Epidermis / Q000002:abnormalities* / Q000000981:diagnostic imaging; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D015225:Magnetoencephalography; D008297:Male; D020752:Neurocutaneous Syndromes / Q000151:congenital / Q000175:diagnosis*; D011859:Radiography; D012640:Seizures / Q000151:congenital / Q000175:diagnosis; D015899:Tomography, Emission-Computed, Single-Photon