Magnetic Resonance Findings of Central Nervous System Abnormalities in Incontinentia Pigmenti: A Case Report

Incontinentia pigmenti, or Bloch-Sulzberger syndrome is a rare neurocutaneous disease. The clinical manifestations begin at or shortly after birth. The central nervous system manifestations include seizures, spastic paralysis, mental retardation, microcephaly and hydrocephalus. The other clinical features include congenital skin lesions, dental and skeletal dysplasia. The occular manifestation of retinal arteriolar occlusion with subsequent retinal hemorrhage and infarction is quite frequent. Magnetic resonance imaging and Magnetic resonance (MR) angiography are valuable in detecting abnormalities of the brain parenchyma and cerebral circulation. More than 50% of the patients have central nervous system (CNS) involvement with MR abnormality. We present the cerebral magnetic resonance imaging (MRI) findings in a female neonate.