The neurologic complications of paget's disease

Abstract

Paget's disease of bone is assoCiated with involvement of the central and peripheral nervous system. The brain, spinal cord, cauda equina, spinal roots, and cranial nerves can be a f f e c t e d in Paget's disease due to their anatomic relationship to bone. Neurologic syndromes are uncommon but include headache, dementia, brain stem and cerebellar dysfunction, cranial neuropathies, myelopathy, cauda equina syndrome, and radiculopathies. The central complications result from pagetic involvement of the skull. Expansion of diseased bone can result in compression of cranial nerves as they exit their bony foramina. Softening of the skull leads to basilar invagination with compression of the brain stem, cerebellum, and lower cranial nerves. Brain stem Compression can cause hydrocephalus. Rarely, there is direct compression of the brain from acute epidural hematoma or hypertrophy of the calvarium. Myelopathy, cauda equina syndrome, and radiculopathies most commonly result from hypertrophy of the spine with direct compression. Spinal sten& can also result from ossification of extradural structures or pathologic fractures. Ischemia from vascular compression or a steal syndrome has also been described. Neurologic complications rarely occur due to sarcomatous transformation of pagetic bone. Magnetic resonance imaging (MRI), computerized tomography (CT)-myelography, and bone X-rays are helpful to localize the lesion and direct therapy. Treatment options include surgical decompression, ventricular shunt placement, and medical management with calcitonin andor the bisphosphonates. The selection of treatment will vary depending upon the rate of progression and the severity of the neurologic deficit. (J Bone Miner Res 1999,14, suppl. 2:88-91)

Paget’s Disease of Bone with Neurological Complications in A Bangladeshi Male

“…The male-to-female ratio is approximately 1.8:1 2 . Because of the close anatomic relationship of the nervous system to the bones, compression due to expanding bone is the most common cause of neurologic features 3 . Paget's disease may be present for many years without symptoms and is asymptomatic in at least 70% of patients 4,7 .…”

“…Plain radiographs and radionuclide scans are important to localize disease activity and the former can often show pathologic fractures. Magnetic resonance imaging (MRI) scanning or CTmyelography is crucial to determine compression of neural structures and to exclude other causes 3 . Differentiating Paget's disease from metastatic bone disease is sometimes difficult.…”

“…The disease is rare in Asian countries, especially China, India, Malaysia, and in the Middle East and Africa 2 . It is associated with involvement of the central and peripheral nervous system 3 . The neurologic complications of cranial disease, excluding auditory involvement, are rare 4 .…”

Headache in patients with Paget’s Disease of Bones

“…These complications are possibly result of mechanical compression or reduced blood flow of neural tissue 8 . Head involvement of PDB may lead to various types of headache, hearing loss, or other cranial nerves deficits, hyperemia of skull and basilar invagination and its consequences.…”

Skull Paget's Disease Developing into Chiari Malformation

“…Paget's cases are mostly asymptomatic with only 5% of the patients developing the symptoms. Skull involvement, shown in half of the cases of Paget's disease, compresses vessels and nerves, resulting in neurological complications such as headache, cranial nerve palsies, spinal cord compression and loss of auditory acuity [1]. In many studies on Paget's disease, effectiveness of bisphosphonate administration has been evident although the underlying mechanism of Paget's is still poorly understood except for its viral etiology [2].…”