Neurological dysfunction in Paget's disease of the vertebral column

Abstract

✓ The authors report four cases of Paget's disease of the vertebral column causing neurological deficit, one with sarcomatous degeneration and one without compression of the spinal cord, and review 86 reported cases. Most cases responded well to decompressive laminectomy. The paradox of neurological deficit without myelographic block is discussed and the relatively common incidence of sarcomatous degeneration noted.

Intravenous disodium etidronate therapy in spinal cord injury patients with heterotopic ossification

“…The experiments in animals con firm these clinical observations, that only three-to five-fold higher doses per body weight were associated with bone frac tures.24 It seems that the fractures are more common in elderly subjects, and that younger patients better tolerate higher eti dronate doses. 26 Gastrointestinal symptoms Paraplegia 31 (1993) [660][661][662][663][664][665][666] (nausea and abdominal discomfort) were present in the majority of our patients but these symptoms did not require the discon tinuation of therapy. Renal tests showed no abnormalities after intravenous administra tion of etidronate; it is likely that serious complications occur only in patients with preexisting kidney disease.…”

Principles of management of osteometabolic disorders affecting the aging spine

“…In our series, patients who demonstrated either partial or temporary improvement after laminectomy and were treated with further antipagetic medical treatment exhibited marked improvement of their symptomatology with sustained relief [50]. From our experience and from other reports, spinal surgery for pagetic spinal stenosis may fail to reverse the neurological deficit completely [15], and may be associated with serious complications such as a mortality rate of 11% [117] and dangerously profuse, if not torrential, bleeding [116]. To avoid such catastrophes, we recommend the preoperative assessment of bone vascularity by means of radionuclide bone blood flow in the affected spinal region.…”

“…In these circumstances, delaying decompression may result in irreversible myelopathy or radiculopathy [80]. On the other hand, the results of surgery have shown variable improvement in 85% of patients [117], with frequent relapses or failures, which may improve with subsequent medical antipagetic therapy [1,16,107]. In our series, patients who demonstrated either partial or temporary improvement after laminectomy and were treated with further antipagetic medical treatment exhibited marked improvement of their symptomatology with sustained relief [50].…”

“…The mechanism of neural ischemia is, however, still hypothetical, and supported only by circumstantial evidence. For example, patients with spinal cord symptomatology respond to calcitonin treatment better than patients with spinal nerve root lesions [28]; some patients experience progressive deterioration of neural function without evidence of myelographic block, which is not easily explained by mechanical effect alone [117]; neurologic signs do not always correlate with the site of skeletal involvement; and rapid clinical improvement occurs in some patients with medical antipagetic treatment alone. These observations suggest that neural dysfunction in PD may also result from mechanisms other than simple bone encroach-S123 Fig.…”

Paget's Disease of the spine and its management

“…The mechanism of neural ischemia is, however, still theoretical and supported only by circumstantial evidence. For example, patients with spinal cord symptomatology respond to calcitonin treatment better than patients with spinal nerve root lesions [32]; some patients experience progressive deterioration of neural function without evidence of myelographic block, which is not easily explained by mechanical effect alone [142]; neurologic signs do not always correlate with the site of skeletal involvement; and rapid clinical improvement occurs in some patients with medical antipagetic treatment alone. These observations suggest that neural dysfunction in PD may also result from mechanisms other than simple bone encroachment on the neural element [40,68,76,83,121,162,169], such as deprivation of blood supply to the neural elements by the rapidly remodeling hypervascular pagetic bone producing "arterial steal phenomenon."…”