A infant girl had red stellate skin lesions on the cheeks and neck, and mildly short palpebral fissures. Her skin abnormality was typical of microphthalmia with linear skin defects (MLS), a newly recognized syndrome consisting of congenital linear skin defects and ocular abnormalities in females monosomic for Xp22. She died suddenly and unexpectedly at age 4 months; the cause of death was ascribed to oncocytic cardiomyopathy. Oncocytic cardiomyopathy occurs only in young children, who present with refractory arrhythmias leading to cardiac arrest. The coexistence of two rare conditions, one of which is mapped to the X chromosome, and an excess of affected females with oncocytic cardiomyopathy, make it likely that oncocytic cardiomyopathy is also X-linked, with Xp22 being a candidate region. Overlapping manifestations in the two conditions (ocular abnormalities in cases of oncocytic cardiomyopathy and arrhythmias in MLS) offer additional support for this hypothesis.

Acute aortic dissections are catastrophic vascular events that have a high rate of mortality. Aortic dissections have been associated with a variety of factors, particularly hypertension. We reviewed 84 medical examiner autopsies on individuals dying from acute aortic dissections with particular emphasis on the role of drugs. Previous case reports have associated aortic dissections with both cocaine and methamphetamine intoxication. We found that seven of the 35 cases tested for drugs of abuse were positive for methamphetamine. Our study had no cases of solely cocaine-related dissection, although one of the cases was positive for both methamphetamine and the cocaine metabolite benzoylecgonine. No significant association was found with any other drugs. As with other studies, we found the most common risk factor to be hypertension. Surprisingly, methamphetamine use was the second most common risk factor. The association between methamphetamine use and aortic dissection is most likely due to its hypertensive effect. Although methamphetamine appears to pose a greater risk than cocaine, both drugs should be considered as possible factors in all aortic dissections.

Sudden, unexpected death can occur following rupture of an artery weakened by aneurysmal dilatation or by medial dissection. In both of these diseases the arterial abnormality is exacerbated by hypertension. This arterial weakness could also be aggravated by the use of drugs with a hypertensive effect. We report seven cases of sudden death in patients abusing methamphetamine—four cases of ruptured berry aneurysms and three cases of aortic dissection with cardiac tamponade. The autopsy findings are reviewed, and various mechanisms are considered by which methamphetamine may contribute to death in such cases.

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