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The neuropathology of glycine encephalopathy

A report of five cases with immunohistochemical and ultrastructural observations

Departments of Pathology, (Drs. Agamanolis and Potter), Children's Hospital Medical Center, Akron OH, Santa Clara Valley Medical Center (Dr. Herrick), San Jose, CA, and the Center for Brain Research (Dr. Sternberger), University of Rochester Medical Center, Rochester, NY.

We studied the spongy myelinopathy of glycine encephalopathy in five patients by using specific antisera. The walls of the vacuoles were stained with the myelin basic protein but not with the myelin associated glycoprotein or the glial fibrillary acidic protein immunostains. The pattern suggested that the vacuoles originated in compact myelin and not from the adaxonal portion of the sheath or from glial processes. Ultrastructural study revealed myelin vacuoles resulting from intraperiod splitting, and there were unusual intranuclear and cytoplasmic inclusions in skeletal muscle in two cases. In addition to the action of glycine as an inhibitory neurotransmitter, structural alterations of myelin may be important in the pathogenesis of the neurologic disorder of glycine encephalopathy.

Address correspondence and reprint requests to Dr. Agamanolis, Department of Pathology, Children's Hospital Medical Center, 281 Locust Street, Akron, OH 44308.

Accepted for publication February 19, 1982.

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