Syringomyelia in Paget's disease.
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The case of a 30-year-old woman with Chiari I malformation and a cervicothoracic syrinx is presented. The patient was followed clinically over a 2 1/2-year period. Spontaneous and complete resolution of the syrinx, as documented by serial magnetic resonance studies, was accompanied by only a minimal change in objective symptomatology.
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A 16-year-old boy presented at the authors' emergency department with a sudden deterioration of respiration. He had been paraparetic for 3 years and had become quadriplegic 2 days previously. Magnetic resonance images revealed a Chiari I malformation and a hydromyelic cavity extending from C-1 to T-11. Rostrally, a small cylindrically shaped lesion extended from the cervicomedullary junction to the left semioval center. The patient made a dramatic neurological recovery following suboccipital craniectomy and upper cervical laminectomies with augmentation duraplasties followed by placement of a syringoperitoneal shunt.
The authors describe the presence of a syrinx in a patient with spontaneous intracranial hypotension. The likely pathophysiological basis of this hitherto unreported association is also presented. It is believed that chronic obstruction at the foramen magnum may be the most important factor for the development of syringes.
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The authors present the case of progressive thoracic myelopathy caused by the extensive ossification of the arachnoid membrane and associated intramedullary syrinx. Based on their findings and results of the literature search, they describe a pathological basis for this rare condition, discuss its incidence and symptomatology, and suggest a simple classification for various types of the arachnoid ossification. They also discuss the magnetic resonance imaging features of arachnoid ossification and associated spinal cord changes. The particular value of plain computerized tomography, which is highly sensitive in revealing intraspinal calcifications and ossifications, in the diagnostic evaluation of patients with a clinical picture of progressive myelopathy is emphasized.
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