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The authors examined the possibility of assessing cerebral damage with phosphorus-31 magnetic resonance (MR) spectroscopy in 24 patients (three infants, four children older than age 5 years, and 17 adults) who met the adult criteria for brain death. In all patients except patient 3 (an infant 8 months old), inorganic phosphate with phosphodiester was observed, and adenosine triphosphate (ATP) and phosphocreatine (PCr), which are detectable in healthy brains, were not detected. In 22 of these 23 patients, cardiac death occurred within 7 days after clinical brain death, and in one infant 14 months old, cardiac death occurred 38 days after clinical brain death. In patient 3, ATP and PCr were detected with P-31 MR spectroscopy, and now, more than 20 months after brain death, cardiac death has not yet occurred. In children younger than age 5 years, P-31 MR spectroscopic findings were predictive of the patient's clinical outcome. These findings may help establish criteria for the diagnosis of brain death in children younger than age 5 years.

A mother and daughter are described with light and electron microscopic, and biochemical abnormalities of their connective tissue characteristic of both cutis laxa and the Ehlers-Danlos syndrome. The mother was clinically normal, while her 8-year-old daughter exhibited loose, wrinkled skin and other clinical features of cutis laxa, and also fragility, bruisability and hyper-extensibility of the skin and poor healing of wounds, leaving "cigarette paper" scars, features characteristic of the Ehlers-Danlos syndrome. Light and electron microscopic studies of skin biopsy specimens and cultured skin fibroblasts from both individuals revealed reduced and distorted elastic fibres, a finding usually seen in cutis laxa. Electrophoretic studies of collagen excreted from cultured skin fibroblasts revealed in both individuals an alpha 2(I) chain with a molecular size smaller than usual. The father and elder daughter were normal by clinical, light and electron microscopic and electrophoretic studies. It was concluded from these findings that the mother and daughter represented a hitherto undescribed disease of the connective tissue with dominant inheritance and variable expressivity.