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Case Reports
. 2021 Jan-Mar;12(1):91-94.
doi: 10.4103/jcvjs.JCVJS_5_21. Epub 2021 Mar 4.

Atlantoaxial instability in a case of Maroteaux-Lamy syndrome

Abhidha Shah  1 , Neha Jadhav  1 , Saswat Dandpat  1 , Atul Goel  1   2
Affiliations
Case Reports

Atlantoaxial instability in a case of Maroteaux-Lamy syndrome

Abhidha Shah et al. J Craniovertebr Junction Spine. 2021 Jan-Mar.

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.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
(a) T1-weighted magnetic resonance imaging showing hypoplastic odntoid process with ill-defined periodontoid tissue. (b) T2-weighted magnetic resonance imaging showing compression of neural structures at the craniocervical junction. (c) Computed tomography scan with the head in flexed position showing the hypoplastic odontoid process and atlantoaxial instability. (d) Computed tomography scan with the head in extended position showing “vertical reduction” of atlantoaxial instability. (e) Computed tomography scan with the cut passing through the facets showing Type 1 atlantoaxial facetal instability. (f) Postoperative computed tomography scan showing atlantoaxial fixation in aligned position. (g) Postoperative computed tomography scan showing the implant. (h) Follow up profile image of the patient (with permission)
See this image and copyright information in PMC

References

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