Mycobacterium chelonei keratopathy with visual rehabilitation by a triple procedure.

We report a chronic keratouveitis following an eye injury, the etiology of which remained obscure despite repeated corneal scrapings and cultures. Eventually, the diagnosis was established by culturing Mycobacterium chelonei from a corneal biopsy specimen. Topical amikacin resolved active inflammation, but dense corneal scarring and a cataract remained. Visual rehabilitation was achieved through a combined penetrating keratoplasty, extracapsular cataract extraction, and intraocular lens implantation. This case illustrates that there should be a high index of suspicion of atypical mycobacteria when faced with any unusual keratitis, particularly following penetrating injuries and corneal grafts.

Travel, time zones, and sudden cardiac death. Emporiatric pathology.

In an 11-year retrospective study of coroner's autopsies on the Hawaiian island of Kauai, it was found that an unusual and apparently unique subset of travelers died suddenly. Death was caused by coronary arteriosclerosis, was characterized by acute coronary changes, and occurred in a group younger than the usual age for sudden cardiac death. Visitors with two-vessel disease had an incidence of sudden death nearly four times that of Kauaians (p less than 0.02), with a ninefold increase of coronary thrombosis (p less than 0.001), a sixfold prevalence of old myocardial infarcts (p less than 0.001), and three times the prevalence of cardiomegaly (p less than 0.001). The circadian pattern of these deaths was different from that previously described in acute myocardial infarction or sudden death, with the number of incidents rising steadily through the day in contrast to the morning peak described in previously published series. The syndrome was expressed in decedents with two-vessel coronary disease either with acute coronary artery lesions--thrombosis, hemorrhage in a plaque, or ruptured coronary artery--or no lesions, presumably spasm. Underlying chronic processes included enlarged heart or old myocardial infarct, which both occurred with a significantly greater incidence in travelers than in local residents, and in a totally different pattern from that occurring in subjects with three-vessel disease. It is postulated that the syndrome is caused by stress related to travel and time zone changes.

Sudden cardiac death following adriamycin therapy.

An 18-year-old man had had an osteogenic sarcoma of the distal tibia at age 16. Below-knee amputation was carried out and followed by adjuvant chemotherapy with Adriamycin, vincristine, methotrexate, Cytoxan, and melphalan. One month after termination of chemotherapy, he died suddenly while playing tennis. Documented ventricular fibrillation was unresponsive to cardiopulmonary resuscitation. Myocardial fibrosis ("cardiomyopathy") was the only significant anatomic finding at autopsy. The occurrence of sudden death without antecedent cardiac failure may have been related to strenuous physical activity in this patient who had received combined adjuvant chemotherapy.

Spongy degeneration of the CNS: an instance of the rare juvenile form.

To our knowledge, the juvenile form of spongy degeneration of the CNS (SD-CNS); van Bogaert-Bertrand disease) has been described previously only three times. We report the case of 21 1/4-year-old Japanese woman who was first seen at the age of 11 with growth retardation, ptosis, and ophthalmoplegia. Her progressive neurodegenerative disease included retinitis pigmentosa, blindness, partial deafness, cerebellar dysfunction, hyporeflexia, and muscle wasting. Simultaneous endocrine defects were diabetes mellitus and probable hyperaldosteronism. Heart block developed later. She died of bronchopneumonia. Autopsy showed CNS stigmas typical of spongy degeneration. Additional findings included peripheral nerve demyelination, neurogenic muscle atrophy, pituitary and pancreatic atrophy, right adrenal agenesis, and a left adrenal coritcal lipid-cell adenoma. To our knowledge, our patient was the oldest survivor, the first patient of Japanese ancestry, and had a unique concurrence of certain oculoendocrine defects.