Clancy R R, Kurtz M B, Baker D, Sladky J T, Honig P J, Younkin D P
Arch Neurol. 1985 Mar;42(3):236-40. doi: 10.1001/archneur.1985.04060030050009.
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.
在6例患有表皮或线状皮脂腺痣（器官样痣）的患者中观察到明显的神经学异常。这些病例的显著特征为单侧面部痣、认知障碍、癫痫发作以及局灶性或偏侧性癫痫脑电图异常。其他表现包括新生儿期癫痫发作的起病、单侧高度节律失调或 Lennox-Gastaut 脑电图模式、偏瘫、不对称性巨头畸形以及躯体生长障碍。这种疾病的全部表现出生时并不明显，而是在婴儿期逐渐显现。计算机断层扫描显示，这些患者的神经学异常归因于中枢神经系统的单侧或不对称畸形。神经外胚层增殖、分化和迁移的偏侧性紊乱可解释该疾病的皮肤和神经学异常。这些患者显著的临床相似性表明表皮痣和线状皮脂腺痣之间存在密切联系。
