Synopsis
Dialysis-related
amyloidosis that occurs secondarily to the deposition of amyloid fibrils containing
beta-2-microglobulin, is a type of amyloidosis affecting patients undergoing
long-term hemodialysis. It involves the osteoarticular system predominantly. Destructive
spondyloarthropathy, which frequently involves the
cervical spine, have
been reported only sporadically. CT is the best modality for detecting osseous
erosion or small
areas of osteolysis in
cortical bone. MRI shows the extent and distribution of
osseous, articular, spinal cord and soft-tissue involvement and indicates amyloid deposits in the
intervertebral disk,
synovium of apophyseal joints, and ligaments. The gold standart of the diagnosis is the histological identification of beta-2-microglobulin.Introduction
Dialysis-related amyloidosis that occurs secondarily
to the deposition of amyloid
fibrils containing beta-2-microglobulin,
is a type of amyloidosis affecting patients undergoing long-term hemodialysis. It
involves the osteoarticular system predominantly. The most common manifestations are carpal tunnel
syndrome and arthropathy of
the shoulders, knees, hips, and axial skeleton (1). Dialysis-related spondyloarthropathy, has been divided into
three types: destructive spondyloarthropathy (DSA), amyloid deposition in spinal
ligaments, and pseudotumor of the craniocervical junction (amiloidoma) (2). DSA, which frequently
involves the cervical spine, have
been reported only sporadically. We describe a case of a
DSA, in a long-term hemodialysis patient, presenting with myelopathy with
particular interest to cervical MRI findings.
Case Report
A 43-year-old man presented with loss of strength in the legs
and disturbances while walking, who had chronic renal failure secondary to
unknown etiology. He had been receiving hemodialysis for 29 years. Cervical
vertebral computed tomography (CT) scans showed narrowing in the cervical 2
(C2) - cervical 3 (C3) intervertebral space, osteolytic areas in the cervical 1
(C1) vertebral body, and osteolytic areas with peripheral sclerosis in the
laminae and pedicules of the various vertebrae (Figure 1). Magnetic resonance imaging (MRI) showed thickening and
decreased signal intensity on both T1and T2-weighted images, in the posterior longitudinal ligament and ligamentum flavum. Scattered
increased signal intensiy of the spinal cord was seen secondary to compression
of thickened ligaments and narrowing of spinal canal (Figure 2). The patient
was operated. Histopathological examination revealed amyloid
deposits and the presence of intense beta-2-microglobulin
fibrils (Figure 3).
Discussion
DSA is characterized
by erosions of the anterosuperior and/or anteroinferior aspects of the vertebral body, severe
narrowing of the
intervertebral disk space and erosions and cysts of adjacent vertebral
plates, with absence of
significant osteophyte formation, radiographically. In advanced stages of the
disease, vertebral body collapse, subluxation,
or listhesis may occur (3). CT is the best modality for detecting osseous
erosion or small
areas of osteolysis in
cortical bone. CT can demonstrate the distribution and extent of the destructive
changes (3).
In the case presented here, areas of osteolysis with peripheral sclerosis in the cervical vertebral bodies, laminae and pedicles were demostrated
by CT (Figure 1).
MRI
shows the extent and distribution of osseous, articular, spinal cord and
soft-tissue involvement, adding to the information obtained from radiographic
and CT images. MRI indicates
amyloid deposits in the intervertebral disk, in the synovium of apophyseal
joints, and in the ligaments
(4). Although , bone lesions
show decreased signal intensity on T1-weighted images in most patients,
T2-weighted images, show various signal intensity patterns that range from
hypointense to hyperintense. The variability in signal intensity is probably
caused by the combination of amyloid deposits and fluid collection within the
subchondral lesions. As in the case presented here, identification of an
intraosseous lesion with low signal intensity on both T1- and T2-weighted
images is helpful in the diagnosis of amyloidosis (Figure 2). After the
gadolinium-based contrast material injection, the bone lesions usually show
moderate enhancement (5).
Compression of the spinal cord and myelopathy
caused by extradural deposition and thickening of ligaments may occur (6). MRI is well suited, as in the case presented here, for assessing the
compression of the spinal cord and myelopathy, caused by the thickening
of ligamentum flavum and posterior longitudinal
ligament (Figure
2).
In the
differential diagnosis, it may be difficult to differentiate changes
secondary to
dialysis-related amyloidosis from spondylodiscitis. The other diseases of the differential diagnosis usually
includes metastatic malignancy, multiple myeloma, and secondary
hyperparathyroidism (renal osteodystrophy).
The gold standart of the diagnosis is the histological identification of beta-2-microglobulin, a major
constituent of amyloid fibrils, in the material which is obtained by surgery (11, 12) (Figure 3).
In the treatment, medical therapy is
limited to symptomatic approaches to reduce pain and inflammation. In the
patients suffering from cervical pain may be referred for surgical evaluation. The
best treatment of hemodialysis-related amyloidosis is renal transplantation.
Renal transplantation can provide a very rapid
symptomatic relief and prevents the progression of the disease. However, the effect of transplantation on
existent amyloid depositions, is contreversial (11).
Conclusion
In the long-term dialysis patients, precise
imaging diagnosis is essential for the assessment of dialysis-related
amyloidosis before serious complications arise. The changes of the vertebral
body, ligaments, facet joints and intervertebral spaces in dialysis-related
amyloidosis and the complications due to these changes, can recognize and
identify by CT and MRI:
Acknowledgements
No acknowledgement found.References
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