A case of mucopolysaccharidosis IV with lower leg paresis due to thoraco-lumbar kyphoscoliosis.
We treated a patient of type IV mucopolysaccharidosis (Morquio's disease) with lower leg paresis due to kyphoscoliosis. A 65-year-old woman presented with Morquio's disease. A lateral radiograph demonstrated the classic bullet-shaped vertebrae and a 65 degrees thoraco-lumbar kyphosis. After the age of 60, she suffered from numbness in both lower legs and walking disturbance. Bilateral patellae-tendon reflexes were exaggerated. MRI showed compression of the spinal cord around T12 to L2 with a highlighted area of change inside the spinal cord. Myelography and computed tomography after the myelography showed narrowing of the sub-arachnoidal space and deformation of the spinal cord around the T12 to L2 levels. Severe vertebral osteoporosis made it necessary to first perform posterior correction of the kyphosis and fusion. The curve was stabilised with the Luque method from T7 to L4. Her neurological condition markedly recovered, but 1 year after surgery her neurological condition again began to deteriorate, resulting in walking disturbance. For this reason, anterior decompression and fusion through a lateral thoracotomy was undertaken. Decompression of the spinal cord and a bone graft from the iliac crest were attained. The patient's neurological condition again improved, but not as much as immediately after the first operation.
Ebara
S
S
Department of Orthopaedic Surgery, Shinshu University School of Medicine, Asahi, Nagano, Matsumoto-City, Japan. soebara@hsp.md.shinshu-u.ac.jp
Kinoshita
T
T
Yuzawa
Y
Y
Takahashi
J
J
Nakamura
I
I
Hirabayashi
H
H
Uozumi
R
R
Kimura
M
M
Takaoka
K
K
D000368:Aged; D005260:Female; D006801:Humans; D008159:Lumbar Vertebrae / Q000000981:diagnostic imaging / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D009085:Mucopolysaccharidosis IV / Q000150:complications* / Q000235:genetics; D018482:Muscle, Skeletal / Q000473:pathology; D019635:Neurosurgical Procedures; D010024:Osteoporosis / Q000473:pathology; D010291:Paresis / Q000209:etiology*; D011859:Radiography; D012600:Scoliosis / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D013117:Spinal Cord Compression / Q000209:etiology / Q000473:pathology / Q000601:surgery; D013131:Spine / Q000000981:diagnostic imaging / Q000473:pathology; D013904:Thoracic Vertebrae / Q000000981:diagnostic imaging / Q000473:pathology*; D016896:Treatment Outcome