Ontology highlight
ABSTRACT:
SUBMITTER: Manara R
PROVIDER: S-EPMC3897861 | biostudies-literature | 2014
REPOSITORIES: biostudies-literature
Manara Renzo R Concolino Daniela D Rampazzo Angelica A Zanetti Alessandra A Tomanin Rossella R Faggin Roberto R Scarpa Maurizio M
JIMD reports 20130702
Compressive cervical myelopathy is a well-known life-threatening complication in mucopolysaccharidosis (MPS) patients. Glycosaminoglycan accumulation in the growing cartilage results in dens dysplasia, atlanto-axial instability, and subsequent periodontoid fibrocartilaginous tissue deposition with upper cervical stenosis.Chiari malformation type 1 (CM1) is a congenital downward cerebellar tonsil ectopia determined by clivus and posterior cranial fossa underdevelopment, possibly leading to progre ...[more]