| dc.description.abstract |
A 7-month-old Caucasian boy was admitted for recurrent generalized seizures since the age of 1 month. Prenatal and perionatal history and developmental milestones were normal. There was nothing in the past medical history that could have accounted for the seizures. The patient was the fourth child of healthy parents, who were first cousins. He had three completely normal brothers. There was no family history of congenital nevi, vascular pathology, or seizures. Physical and neurologic examination was normal. Dermatologic examination revealed a distinct, dark-brown, elevated plaque measuring 17 x 23 cm, studded with small papules and coarse hairs, covering the lower thoracic, lumbar, and sacroiliac regions in a garment distribution. This giant nevus was associated with multiple, scattered, darkly pigmented satellite nevomelanocytic nevi. Histopathologic examination of this lesion was compatible with congenital nevomelanocytic nevus (CNN). An electroencephalogram (EEG) was normal. Magnetic resonance imaging (MRI) revealed a 0.8 x 2 cm, irregularly shaped lesion deep in the right temporal region adjacent to the suprasellar cistern, that could be a hamartoma. Cranial computed tomography (CT) with a 55 Hounsfield unit confirmed a calcified hamartomatous lesion in the right hippocampus. The parents did not consent to brain biopsy. A partial remission was achieved with phenytoin treatment during g months of follow-up. |
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