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A Primer on Perinatal Telencephalic Leukoencephalopathy

Posted Feb 13 2013 5:07pm
In my last post, it was correctly pointed out by two astute readers that the Netter figure demonstrating brain malformations included an entity which is NOT a brain malformation: perinatal telencephalic leukoencephalopathy (PTL). PTL results from a perinatal hypoxic insult and is not a malformation per se, as the term "malformation" should really be reserved for anatomic abnormalities resulting from developmental defects. The cerebral white matter in the fetus and neonate is particularly susceptible to injury as it is metabolically highly active. Ischemia of the germinal matrix, subependymal region, and periventricular white matter can lead to periventricular leukomalacia that may even calcify and result in periventricular mineralization (1). At its most extreme, PTL may take the form of hemispheric multicystic leukomalacia in which the cerebral hemispheric white matter is replaced by multiloculated cysts separated by strands of fibroglial tissue.(2)

References1. Fuller, Gregory N. and Goodman, J. Clay. Practical Review of Neuropathology. Lippincott 2001. Page 315.
2. Haberland, Catherine. Clinical Neuropathology: Text and Atlas. Demos Publishing 2007. Page 303.
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