MR in a patient with Zellweger syndrome presenting without cortical or myelination abnormalities.

We read with interest the recent paper by Barkovich and Peck describing MR features of Zellweger syndrome (ZS) (1). All six patients in their series had impaired myelination, diffusely abnormal cortical gyri, and germinolytic cysts on MR imaging. Specifically, these abnormalities consisted of diminished myelination, microgyria (particularly in the frontal and perisylvian regions), pachygyria (primarily perirolandic and occipital regions), and germinolytic cysts in the caudothalamic grooves. A marked variation in the extent and appearance of these abnormalities was observed in all the six patients. Indeed, we believe that this spectrum of abnormalities may even include patients in whom only one of the above-mentioned abnormalities is present. We recently encountered a patient in whom no cortical abnormalities or impaired myelination were present. Germinolytic cysts, however, were evident. She had a prenatal sonogram at 33 weeks of gestation with questionable hydrocephalus, but was the product of an uncomplicated vaginal delivery. At birth, she was floppy and unresponsive with low Apgar scores. Distinct facial features included an elongated forehead with hyp-