Neurologic manifestations of the organoid nevus syndrome
R. R. Clancy, M. B. Kurtz, D. Baker, J. T. Sladky, P. J. Honig and D. P. Younkin
Prominent neurologic abnormalities were observed in six patients with
epidermal or linear sebaceous nevi (organoid nevi). These cases were
remarkable for unilateral facial nevi, cognitive impairment, seizures, and
focal or lateralized epileptic EEG abnormalities. Additional manifestations
included the onset of seizures in the neonatal period, unilateral
hypsarrhythmia or Lennox-Gastaut EEG pattern, hemiparesis, asymmetric
macrocephaly, and somatic growth disturbances. The full expression of this
disorder was not apparent at birth, but emerged gradually during infancy.
The neurologic abnormalities in these patients were attributed to
unilateral or asymmetric malformations of the CNS as demonstrated by
computed tomography. A lateralized disorder of neuroectodermal
proliferation, differentiation, and migration could account for both the
cutaneous and neurologic abnormalities in this disorder. The striking
clinical similarities in these patients suggest a close link between
epidermal and linear sebaceous nevi.
