Biomedical subjects
Publications and source records attributed to G L Rea.
Subependymomas are extremely rare tumors in the pediatric population and, when they occur, they are usually of a mixed type with elements of subependymoma and ependymoma. This report is of a 2 1/2-year-old male infant with a pure subependymoma of the fourth ventricle.
Serum levels of creatine kinase (CK) and its myocardial isoenzyme (CK-MB) were measured and serial ECG recorded in 24 male and 6 female patients with severe head trauma. All patients were comatose, but no patient sustained a spinal or chest injury. Total CK activity was elevated in at least one sample in each patient. Elevated CK-MB activity was found in 28 patients. The serial CK-MB data did not follow the same pattern as that of patients suffering from myocardial infarctions. The mean CK-MB remained elevated for at least 3 days after injury, although individual patterns were variable. ECG abnormalities included prolonged corrected QT interval (QTc) in 90% and a variety of nonspecific ST segment and T wave changes in 53%. These ECG findings are consistent with other clinical studies of severe neurological disorders, particularly cerebrovascular accidents. The elevated CK-MB activity indicates that ongoing myocardial damage occurs in patients with severe head injury. Although the underlying mechanism is not entirely clear, an excessive release of catecholamines is the most likely mechanism accounting for diffuse myocardial damage, prolonged elevated CK-MB values and the observed ECG abnormalities.
Ectrodactyly (lobster claw deformity), ectodermal dysplasia and clefting of the lip or palate, the EEC syndrome, is a rare genetic disorder involving both mesodermal and ectodermal derivatives. Cerebral arterial dolichoectasia, a pathological dilatation and elongation of intracranial vessels, was found in a patient with the EEC syndrome. Her hypertensive cardiovascular disease, mild dementia and trigeminal neuralgia were all related to her dolichoectasia. Although the association of dolichoectasia has not been previously observed with the EEC syndrome, it may be more frequent than currently recognized. Cerebral arterial dolichoectasia and its attendant sequelae could account for some of the unexplained abnormalities described in other case reports of patients with the EEC syndrome.