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Regional Ependymal Upregulation of Vimentin in Chiari II Malformation, Aqueductal Stenosis, and Hydromyelia

  • Original Article
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Pediatric and Developmental Pathology

Abstract

Vimentin, glial fibrillary acidic protein (GFAP) and S-100 β protein were studied by immunocytochemistry in the ependyma of patients with Chiari II malformations, congenital aqueductal stenosis, and hydromyelia. Paraffin sections of brains and spinal cords of 16 patients were examined, 14 with Chiari II malformations, most with aqueductal stenosis and/or hydromyelia as associated features, and 2 patients with congenital aqueductal stenosis without Chiari malformation. Patients ranged in age from 20-wk gestation to 48 years. The results demonstrated: 1) in the fetus and young infant with Chiari II malformations, congenital aqueductal stenosis, and hydromyelia, vimentin is focally upregulated in the ependyma only in areas of dysgenesis and not in the ependyma throughout the ventricular system; 2) GFAP and S-100β protein are not coexpressed, indicating that the selective upregulation of vimentin is not simple maturational delay; 3) vimentin upregulation also is seen in the ependymal remnants of the congenital atretic cerebral aqueduct, not associated with Chiari malformation; 4) in the older child and adult with Chiari II malformation, vimentin overexpression in the ependyma becomes more generalized in the lateral ventricles as well, hence evolves into a nonspecific upregulation. The interpretation from these findings leads to speculation that it is unlikely that ependymal vimentin is directly involved in the pathogenesis of Chiari II malformation, but may reflect a secondary upregulation due to defective expression of another gene. This gene may be one of rhombomeric segmentation that also plays a role in defective programming of the paraxial mesoderm for the basioccipital and supraoccipital bones resulting in a small posterior fossa. This interpretation supports the hypothesis of a molecular genetic defect, rather than a mechanical cause, as the etiology of the Chiari II malformation.

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Acknowledgments

I thank several pathologist colleagues who generously shared their cases with me for this study: Drs. C.E. Alvord, Jr. C.-M. Shaw, S.M. Sumi, D. Anderson, R.P. Kapur, and K. Patterson of the University of Washington Medical Center and Children’s Hospital, Seattle, WA; Dr. A. D’Agostino, of the Department of Pathology (Neuropathology), Oregon Health Sciences University, Portland, OR: and Dr. C.L. Trevenen of the University of Calgary and Alberta Children’s Hospital, Calgary, Alberta, Canada. Dr. A. Smith assisted in identifying archived cases at the University of Washington. Technical assistance with the immunocytochemical preparations was provided by M. Wilhyde, R. Small, and C. Olman at the University of Washington Medical Center. I am grateful also to Drs. L. Flores-Sarnat, of the Department of Pediatrics (Neurology), W. Yong, of the Department of Pathology (Neuropathology), and M. Danielpour, of the Department of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, CA, for their helpful suggestions in reading the manuscript.

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  1. Department of Pathology (Neuropathology), Cedars-Sinai Medical Center and UCLA School of Medicine, 4221 North Tower, 8700 Beverly Boulevard, Los Angeles, CA 90048, USA

    Harvey B. Sarnat

  2. Department of Pediatrics (Neurology), Cedars-Sinai Medical Center and UCLA School of Medicine, Los Angeles, CA, USA

    Harvey B. Sarnat

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Sarnat, H. Regional Ependymal Upregulation of Vimentin in Chiari II Malformation, Aqueductal Stenosis, and Hydromyelia. Pediatr. Dev. Pathol. 7, 48–60 (2004). https://doi.org/10.1007/s10024-003-2127-5

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