Eosinophilic intracytoplasmic inclusions in Purkinje neurons of children.
Eosinophilic intracytoplasmic inclusions have been rarely described in Purkinje neurons of children with a variety of neurological conditions. Here we document these inclusions in five children from 3 to 14 years of age. One child had 7q deletion syndrome and a second had profound motor and cognitive delay ("cerebral palsy") of unknown origin, while three others were neurologically normal prior to death. These inclusions stain with the PAS method, are not strongly ubiquitinated, and are located in the lumen of endoplasmic reticulum. Their appearance in a wide range of disorders and in neurologically normal children suggests that they are a nonspecific protein trafficking anomaly, possibly aggravated under degenerative conditions.
Zherebitskiy
Viktor
V
Department of Pathology, Health Sciences Centre and University of Manitoba, Winnipeg, Canada.
Del Bigio
Marc R
MR
D000293:Adolescent; D001921:Brain / Q000648:ultrastructure; D002648:Child; D002675:Child, Preschool; D002897:Chromosomes, Human, Pair 7 / Q000235:genetics; D003072:Cognition Disorders / Q000473:pathology; D002658:Developmental Disabilities / Q000473:pathology; D004721:Endoplasmic Reticulum / Q000648:ultrastructure*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000648:ultrastructure*; D008297:Male; D008854:Microscopy, Electron; D009422:Nervous System Diseases / Q000473:pathology; D010503:Periodic Acid-Schiff Reaction; D010784:Photomicrography; D011689:Purkinje Cells / Q000648:ultrastructure*; D017384:Sequence Deletion; D025801:Ubiquitin / Q000378:metabolism
D025801:Ubiquitin