Biomedical subjects
Publications and source records attributed to A R Seay.
The rigid spine syndrome is an unusual disorder characterized by nonprogressive mild axial and proximal muscle weakness, flexion contractures of the neck and spine, scoliosis, and joint contractures. The biopsy specimen from the patient described in this report shows type I fiber hypotrophy and predominance. This contrasts with previous reports that describe connective tissue proliferation as the major histologic abnormality.
We review the records of 153 infants weighing 2,500 gm or less admitted consecutivtly to the University of Utah Medical Center Newborn Intensive Care Unit. Thirty-one (20.2%) infants had seizures and 28 (90%) of these died. In comparison, only 17% (21 of 122) of patients without seizures died. The diagnosis of intraventricular hemorrhage (IVH) was made in 27 (87%) of the seizure patients. These findings differ significantly from those studies in which no distinction was made between the birth weight of neonates with seizures. Anticonvulsant medication had little effect on clinical seizure activity and did not significantly alter the outcome.
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A retrospective chart review was conducted to examine risk factors associated with the occurrence of choreoathetosis, a rare but significant complication of open-heart surgery in children. Ten children were identified as having developed choreoathetosis after cardiac surgery. Their charts were reviewed and compared with 33 age- and diagnosis-matched controls who underwent open-heart surgery during the same time period. Children with choreoathetosis reached lower rectal and esophageal temperatures (p = 0.0018 for both) and spent a greater portion of total bypass time at lower rectal and esophageal temperatures (p < 0.001 for both). Duration of cooling below 20 degrees C esophageal temperature and PaCO2 at the end of the cooling period were significant predictors of choreoathetosis (p = 0.023 and p = 0.0497, respectively) in a logistic regression model, and a greater fraction of choreoathetosis patients had prior developmental delays (p = 0.017). No difference was found in the age at surgery, duration of bypass, aortic cross-clamp time, arterial pH, PaCO2 or mean arterial pressure. The combination of extended exposure to profound hypothermia and alpha-stat pH management strategy and preexisting developmental delay are associated with the development of choreoathetosis following open-heart surgery 61 in children.
Neurologic signs and symptoms of brachial plexopathy may be subtle or confusing, making clinical localization of disease along the length of the brachial plexus difficult. To determine the most direct radiographic approach to diagnosing and anatomically delineating the cause of brachial plexopathy, we reviewed the clinical and radiographic records of 43 patients presenting with signs and symptoms referable to the brachial plexus who received CT and/or myelography as part of their radiographic evaluation. The study population was divided into two groups, those with and those without trauma. Significant deficiencies were detected in the radiographic evaluation of the nontraumatic group, with 35% of these patients having an incomplete or inappropriate CT examination that failed to visualize the full extent of the brachial plexus. In four patients, this led to a significant (greater than 6 months) delay in diagnosis. It was concluded that trauma patients presenting with brachial plexus symptoms should have cervical myelography first, rather than CT. Patients without a history of trauma should be classified on the basis of clinical findings as having central (cord, epidural space, neural foramen) or peripheral (retroclavicular space, axillary apex) disease. If the abnormality is central, myelography should be the first technique used; if peripheral disease is present, CT should be the first study. If the disease extends beyond the confines of the anatomic compartment suggested clinically, the other technique should be used for further evaluation. CT scan protocols for brachial plexus evaluation should employ bolus/drip contrast enhancement to distinguish vascular structures from masses.(ABSTRACT TRUNCATED AT 250 WORDS)