Atlantoaxial instability in a case of Maroteaux-Lamy syndrome

Abhidha Shah; Neha Jadhav; Saswat Dandpat; Atul Goel

doi:10.4103/jcvjs.JCVJS_5_21

Atlantoaxial instability in a case of Maroteaux-Lamy syndrome

J Craniovertebr Junction Spine. 2021 Jan-Mar;12(1):91-94. doi: 10.4103/jcvjs.JCVJS_5_21. Epub 2021 Mar 4.

Authors

Abhidha Shah¹, Neha Jadhav¹, Saswat Dandpat¹, Atul Goel^{1

2}

Affiliations

¹ Department of Neurosurgery, K.E.M. Hospital and Seth G.S. Medical College, Mumbai, Maharashtra, India.
² Lilavati Hospital and Research Centre, Mumbai, Maharashtra, India.

Abstract

A relatively rare report of an 8-year-old girl with Maroteaux-Lamy syndrome that is Type VI mucopolysaccharidosis who presented with symptoms of spastic quadriparesis related to atlantoaxial instability is presented. Atlantoaxial stabilization resulted in rapid and sustained neurological recovery.

Keywords: Atlantoaxial instability; Maroteaux–Lamy syndrome; mucopolysaccharidosis.

Publication types

Case Reports