Syringomyelia in mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome): imaging findings following bone marrow transplantation.
We present the imaging findings in a patient with mucopolysaccharidosis (MPS) type VI (Maroteaux-Lamy syndrome) who developed holocord syringomyelia. This represents the only reported case of syrinx formation in a child with MPS VI. Clinical, neurologic and spinal magnetic resonance imaging findings are presented. The patient has maintained a stable clinical and neurologic course over the period following allogeneic bone marrow transplant.
Hite
S H
SH
Department of Radiology, Box 292, University of Minnesota Hospital and Clinic, 420 Delaware Street SE, Minneapolis, MN 55455, USA.
Krivit
W
W
Haines
S J
SJ
Whitley
C B
CB
D016026:Bone Marrow Transplantation; D002803:Chondro-4-Sulfatase / Q000378:metabolism; D006025:Glycosaminoglycans / Q000652:urine; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009083:Mucopolysaccharidoses / Q000150:complications* / Q000378:metabolism; D013595:Syringomyelia / Q000175:diagnosis* / Q000209:etiology*
D006025:Glycosaminoglycans; D002803:Chondro-4-Sulfatase