Two infants with linear nevus sebaceum syndrome and infantile spasms are reported from Safra Childrens Hospital, Sheba Medical Center, Tel Hashomer, Israel; and Hospital for Sick Children, Toronto, Canada. Case 1 presented at age 4 months with focal motor and generalized convulsive seizures with low-grade fever. Family history was positive for febrile seizures in the mother. A 3-cm gray-yellow scaly patch was noted on the frontal-central scalp area that enlarged and turned red and thickened after discharge. Brain MRI showed bilateral polymicrogyria and subependymal heterotopia. At age 8 months, she developed infantile spasms and modified hypsarrhythmia on EEG. Seizures were controlled with vigabatrin. At age 2.5 years, she is seizure-free on topiramate and clobazam. Case 2 presented at age 3 months with generalized seizures and two 2.5 cm hyperpigmented nevi in the right parietaltemporal area. Eye exam revealed a right esotropia and coloboma. MRI showed right hemimegalencephaly. EEG recorded right hemisphere slowing and interictal spikes and slow waves. Seizures were controlled with phenobarbital. He was readmitted at age 11 months with infantile spasms and hypsarrhythmia, resistant to vigabatrin and controlled by ACTH. At age 3 years he presented with frequent generalized tonic-clonic and myoclonic seizures and developmental delay. The pigmented nevi had each enlarged to 5 cm in diameter. EEG showed a generalized slow-spike and wave pattern, consistent with Lennox-Gastaut syndrome. The literature on linear nevus sebaceous syndrome is reviewed. [1]

COMMENT. The neurocutaneous linear nevus sebaceum syndrome is characterized by a triad of epidermal nevi, seizures, and mental retardation. A review of 60 cases by Solomon et al, in 1975, described the dermatologic lesions as epidermal nevi associated with neurologic, ophthalmic, skeletal, cardiovascular, and urological abnormalities. Children with a suspected linear nevus sebaceum syndrome should have EEG, MRI, and ophthalmology exams. Seizures occur in up to 75% cases, frequently infantile spasms, West syndrome and evolving into Lennox-Gastaut syndrome. The term linear nevus sebaceum syndrome is usually reserved for cases with midline nevi, while “epidermal nevus syndrome” is more inclusive for all varieties of epidermal nevi.