Zellwegers Disease or Cerebrohepatorenal Syndrome

Neonates present with craniofacial dysmorphism, pigmentary retinopathy, cataract, sensorineuronal hearing impairment, hypotonia, failure to thrive, seizures, patellar calcification, and hepatomegaly. Death usually occurs within 5 to 6 months of onset.

The cerebral pathology is distinguished by developmental abnormalities, such as disorders of neuronal migration: pachygyria and microgyria along the Sylvian fissures, heterotopias of the Purkinje cells, and anomalous dentate and olivary nuclei. Other changes are variable neuronal losses, diminution and/or breakdown of myelin, lipid deposits within macrophages, and degeneration of the optic nerves. Common visceral changes include liver fibrosis, often progressing to cirrhosis; kidney cysts; and lipid-containing striated cortical cells in the adrenals.

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