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Query: UMLS:C0027960 (mole)
 21,279 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Neurologic manifestations of the organoid nevus syndrome. 397 53

We report what we believe to be the first known case of linear nevus sebaceus syndrome with infantile spasms followed by Lennox-Gastaut syndrome. The EEG revealed hemihypsarrhythmia and then asymmetrical slow spike-wave complexes. A pneumoencephalogram showed dilation and distortion of the lateral ventricle ipsilateral to the facial nevus on the left side. A computerized tomographic scan disclosed low density in the left parietotemporal region, a feature not reported previously. The findings indicated extensive lateralized cerebral lesions.
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PMID:Linear nevus sebaceus syndrome. Report of a case with Lennox-Gastaut syndrome following infantile spasms. 697 8