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Spinal Stenosis with Paraparesis in Albright Hereditary Osteodystrophy

PEDIATRIC NEUROSURGERY(2008)

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Abstract
We describe thoracic spinal stenosis with progressive myelopathy in association with Albright hereditary osteodystrophy (AHO) in a 12-year-old child with delayed diagnosis and review the relevant literature in order to identify the pathophysiological mechanism. The child was successfully treated by decompressive upper thoracic laminoplasty with full neurological recovery. The pathological changes of the skin also dissolved. Ten more cases of myelopathy and paraparesis in association with AHO, of whom two were children, could be found in the literature. Basically, two different causes for the spinal canal stenosis could be identified: abnormal ossifications of ligaments and congenital narrow spinal canal due to short vertebral pedicles. Awareness of structural spinal column changes in AHO is essential in order to appreciate the neurological symptoms of a beginning myelopathy before irreversible damage to the myelum occurs. Copyright (C) 2008 S. Karger AG, Basel.
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Key words
Albright hereditary osteodystrophy,spinal stenosis,pseudohypoparathyroidism,paraparesis,myelopathy,pseudopseudohypoparathyroidism
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