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In this study, our goals were to determine whether Reye's syndrome existed before the first published report of this entity in 1963 and to describe the long-term incidence and trend of this syndrome. Medical and pathologic records and pathologic specimens for Mayo Clinic patients, including those who were residents of Olmsted County, Minnesota, were reviewed through the facilities of the Rochester Epidemiology Project. On review, 12 cases were identified for the period 1920 through 1962; 2 of these cases were in Olmsted County residents. Of the 12 cases identified before 1963, 10 were in patients younger than 5 years of age. In addition to the aforementioned two cases, five others in Olmsted County were diagnosed between 1963 and 1989. Incidence rates were computed for the Olmsted County population. The number of cases and rates per 100,000 persons younger than 18 years of age in that population were 2 cases (0.3) for 1920 through 1962, 4 cases (0.7) for 1963 through 1980, and 1 case (0.4) for 1981 through 1989. Although cases found during the earlier periods may have included disorders that mimic Reye's syndrome, the recommended criteria from the Centers for Disease Control were applied to review of medical records and supported by pathologic findings in the liver. The data showed an age distribution tending toward young patients and no seasonality of occurrence. The incidence rates in Olmsted County, Minnesota, showed an increase for 1963 through 1980 over previous decades, followed by a decrease. The small numbers preclude any conclusion about trends in the incidence rates.

Previous case reports of vertebral-basilar system infarction following chiropractic cervical manipulation have emphasized the role of predisposing factors such as cervical spondylosis, atherosclerosis, and congenital asymmetry of the posterior circulation. Ten patients without prior neurologic symptoms had vertebral-basilar system infarction promptly after chiropractic maneuvers. One patient, who was free of clinical and radiographic evidence of predisposing factors, subsequently died. Autopsy studies revealed massive nonhemorrhagic brainstem infarction due to bilateral vertebral artery thrombosis. Nine patients survived with residual neurologic deficits due to lesions in various locations of the posterior circulation. No patient received anticoagulants. Previous case reports are summarized and the kinetic anatomy of the vertebral arteries is reviewed to clarify the potential mechanisms involved in the pathogenesis of this entity. Although a causal relationship may be difficult to establish in individual cases, cervical manipulation seems to be the major identifiable factor in the pathogenesis of stroke in some patients.

Intraventricular hemorrhages were the most common neuropathologic findings in both full-term and premature infants in a retrospective study of neonatal deaths. In general, the intraventricular hemorrhages of full-term infants were less frequent, originated from veins of the choroid plexus, were small, and rarely caused death. Intraventricular hemorrhages of premature infants usually originated in the germinal matrix and were large enough to be fatal. Intraventricular hemorrhages were most frequently found when death occurred between 24 and 72 hours of age.

We conducted postmortem magnetic resonance imaging (MRI) studies of five brains from patients with a clinical and pathologic diagnosis of tuberous sclerosis. Areas of prolonged T1 and T2 relaxation could easily be identified within the white matter subjacent to the cortical tubers despite formalin fixation and storage. The signal changes were identical to those reported in living patients with the disease. The detection of cortical tubers by MRI in two patients who were 34 and 35 years of age, respectively, at the time of death suggests that the signal changes on MRI are less affected by aging than are the low-attenuation changes on computed tomography, which are rarely identified in patients older than 27 years of age. Once the changes caused by fixation of tissues are considered, postmortem MRI is a viable investigative tool in studying tuberous sclerosis and other diseases, and it seems to correlate well with the MRI findings in living patients, as well as the gross and histopathologic changes seen at autopsy.

Primary cerebrovascular amyloidosis resulting in significant cerebral parenchymal damage was encountered in 23 autopsied cases at the Mayo Clinic over the past 10 years. Patients were 60 to 97 years old and both sexes were equally represented. Large- and medium-sized leptomeningeal and cortical arteries showed the characteristic pattern of medial and intimal involvement, with luminal stenosis. The walls of smaller arteries were often diffusely infiltrated, with fibrinoid degeneration and miliary aneurysm formation. The amyloid nature of the infiltrate was confirmed by electron microscopic examination in all cases. All cases showed varying numbers of perivascular or independent senile plaques in the cerebral cortex. Alzheimer's neurofibrillary tangles were absent or were limited to the hippocampal region in all but two cases. Multiple, small cortical infarcts and hemorrhages were regularly present. Larger hemorrhage was present in nine cases. Of nine patients with terminal massive cerebral hemorrhage, only two were hypertensive. Six patients had had progressive dementia; four had had single episodes of vascular events and seven, multiple episodes; and four had had both dementia and vascular episodes. Primary cerebral amyloid angiopathy should be regarded as an important cause of mental deterioration and fatal cerebrovascular accidents in the elderly.

The diagnostic criteria, natural history, nerve conduction characteristics, pathology, laboratory features, and efficacy of corticosteroid treatment have been evaluated personally in 53 patients with chronic inflammatory polyradiculoneuropathy (CIP) who were followed up for an average of about 7.5 years. These were patients whose monophasic neurologic deficit had not crested by 6 months, patients with recurrences, and patients with a steady or stepwise progression. The typical features of CIP include absence of an associated disease, frequent history of preceding infection or receipt of foreign protein, and tendency to involve cranial, truncal, and proximal as well as distal limb structures and to have diffusely slow conduction velocity of peripheral nerves. The most marked slowing is often very proximal. The pathologic features include serous edema, mononuclear cell infiltrates (especially in perivascular areas, but without evidence of vasculitis), macrophage-induced segmental demyelination, and hypertrophic neuritis. If our patients are representative, complete recovery occurs only infrequently; about 60% of patients are able to be ambulatory and work, 25% become confined to a wheelchair or become bedridden, and approximately 10% die from their disease. Although the bulk of the pathologic changes affect spinal roots and proximal nerves, the brain and spinal cord may be involved also. Degeneration into linear rows of myelin ovoids is the predominant type of myelinated fiber degeneration of the sural nerve at the ankle.

The fat embolism syndrome is a well-defined clinical entity that can usually be recognized in patients who have long-bone fractures. Cerebral symptoms of restlessness, confusion, stupor, and coma correlate with the autopsy findings of fat in the brain, but the amount of fat or amount of petechial hemorrhage cannot be quantitated with the severity of the cerebral symptoms. There is a correlation between the clinical manifestations of dyspnea and tachypnea and the autopsy findings in the lungs, which are heavy from edema and alveolar hemorrhage; however, we cannot correlate the degree of pathologic change in the lung, including lung weights, with the severity of findings clinically.

The apparent hypogonadism in patients with the Laurence-Moon syndrome has been variably attributed to unresponsiveness of target organs to gonadal hormones, primary end-organ failure, hypothalamic dysfunction, or pituitary failure. We report the first immunocytologic study of the pituitary gland in this rare disorder. No morphologic abnormalities were noted. The numbers and immunoreactivities of adenohypophyseal cell types were normal. No microscopic abnormalities were evident in the hypothalamus and target organs. The results of our study are consistent with recent biochemical data that suggest that pituitary function is normal in patients with this syndrome.

A 79-year-old man with unremitting painful ophthalmoplegia had a necrotizing inflammatory process that involved the intracranial and intracavernous portions of the right internal carotid artery. The condition ultimately resulted in rupture of the carotid vessel and death. At autopsy, thrombophlebitis of the sphenopalatine sinus was also noted. To our knowledge, this is the second reported autopsy study of painful ophthalmoplegia and chronic nongranulomatous inflammation that involved the carotid siphon and the cavernous sinus.