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Robert R. Clancy, MD; Michael B. Kurtz, MD; David Baker, MD; John T. Sladky, MD; Paul J. Honig, MD; Donald P. Younkin, MD

Arch Neurol. 1985;42(3):236-240.

Abstract
• 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.

Author Affiliations
From the Divisions of Neurology (Drs Clancy, Sladky, and Younkin) and Dermatology (Dr Honig), The Children's Hospital of Philadelphia; the Departments of Neurology (Drs Clancy, Sladky, and Younkin) and Pediatrics (Drs Clancy, Sladky, Honig, and Younkin), University of Pennsylvania School of Medicine, Philadelphia; the Department of Pediatrics, Hahneman University, Philadelphia (Dr Baker); and the Elwyn (Pa) Institute (Dr Kurtz).

Footnotes
Accepted for publication March 10, 1984.

Reprint requests to Division of Neurology, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104 (Dr Clancy).

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