Three patients with hereditary motor sensory neuropathy type I developed neurologic deficits attributable to hypertrophic nerve roots. Compression of the cervical spinal cord by enlarged nerve roots occurred in our index patient. Multilevel decompressive laminectomies relieved the myelopathy. An unrelated patient who had syncope precipitated by neck rotation had hypertrophied nerve roots that eroded into the transverse foramina in juxtaposition to the vertebral arteries. In a 3rd patient, compression of hypertrophied nerve roots within the thecal sac and neural foramina was associated with spinal claudication and radiculopathy, respectively.
Because of technical difficulties and diagnostic limitations encountered with other myelographic techniques in patients with achondroplasia, the authors employed a lateral C1-2 puncture and non-ionic, water-soluble contrast medium in 18 achondroplastic patients with spinal compression (21 procedures). This technique proved most appropriate for identifying the upper limit of degenerative osteophytes causing exacerbation of congenital spinal stenosis, which is crucial in planning decompressive surgery. A potentially important additional finding was the presence of degenerative lower cervical spine disease in the majority of patients. There were no serious complications. The authors recommend this technique as safe and effective in achondroplastic patients with severe congenital spinal stenosis.
The diagnostic usefulness, limitations, and adverse reactions associated with computed tomographic myelography using metrizamide were assessed for broad outpatient application. The initial approach was to examine inpatients (n = 38) with low-dose metrizamide (100 mgI/ml). This low dose was believed less likely to be associated with side effects. They were then treated as if they were outpatients, with the liberties this entailed. The consequences of needle puncture were minimized by using a 25-gauge disposable needle. Thirty-four (89%) patients remained free of side effects after the procedure. Subsequently, this technique was extended to 42 outpatients, 38 (90.5%) of whom remained asymptomatic. For comparison, 170 mgI/ml was used in another 25 outpatients, who evidenced more symptoms. The potential medical, economic, and therapeutic benefits of obviating hospitalization by safer outpatient CT myelography seem clear.
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