More than a dozen primary hematologic disorders have been associated with ischemic stroke. Inherited deficiencies of antithrombin III, protein C, and protein S have been linked with stroke in case reports; optimal screening requires functional as well as antigenic assays. Antiphospholipid antibodies and lupus anticoagulants are the most frequently identified acquired states associated with ischemic stroke. Polycythemia vera, sickle cell anemia, sickle-C disease, and essential thrombocythemia are the major disorders of formed blood elements causing stroke. Special, step-wise screening for occult prothrombotic entities in stroke patients is recommended for young persons with stroke of uncertain cause, for those with prior venous thrombosis, for those with a family history of unusual thrombosis, and for those with no other explanation for recurrent stroke. Acquired, perhaps transient, abnormalities of platelets, coagulation inhibition, and fibrinolysis may contribute importantly to brain ischemia in synergy with other mechanisms, but at present these remain ill-defined. The contribution of prothrombotic diatheses to stroke is probably underrecognized and warrants further investigation. (Stroke 1990;21:llll-1121)

We reviewed 212 consecutive episodes of infective endocarditis in 203 patients at six hospitals between 1978 and 1986 and found that 21% were complicated by stroke. Of 133 episodes involving native mitral and/or aortic valves, brain ischemia occurred in 19%, brain hemorrhage in 7%, and non-central nervous system emboli in 11%; vegetations were identified in 56% of 113 adequate echocardiograms and did not correlate with risk of embolism. In native-valve endocarditis, most (74%) ischemic strokes had occurred by the time of presentation and an additional 13% occurred <48 hours after diagnosis; the incidence of brain ischemia was 13% on presentation, 3% during the first 48 hours of hospitalization, and 2%-5% during the remainder of the acute course. Stroke recurred at a rate of 0.5%/day, often heralding relapse/uncontrolled infection. Only 9% of ischemic infarcts were large (all in patients with Staphylococcus aureus infection), while 8% were small and subcortical. Brain hemorrhage occurred primarily at the time of presentation, particularly in intravenous drug abusers, and was associated with uncontrolled S. aureus infection with pyogenic arteritis. Ischemic and hemorrhagic stroke continue to be frequent and important in patients with infective endocarditis and are clustered during uncontrolled infection. Patients with infective endocarditis and ischemic stroke on presentation seldom had recurrent emboli after the infection was controlled; anticoagulants and surgery are not warranted to prevent recurrent stroke in these patients. Symptomatic mycotic aneurysms are quite uncommon. (Stroke 1990:21:695-700)

Neurologic syndromes often complicate the management of infective endocarditis (IE). We retrospectively reviewed 166 episodes of native valve endocarditis to assess the occurrence and implications of nonfocal encephalopathy, meningitis, salient headache, back pain, and brain abscess. Neurologic complications occurred in 35% (58/166) of patients: 41% (54/133) of mitral or aortic valve IE and 12% (4/33) of tricuspid valve IE. Of 133 cases of mitral or aortic valve IE, encephalopathy occurred in 14%, meningitis in 5%, and salient headache in 3%. All neurologic complications occurred more often with Staphylococcus aureus infection (67%) than with viridans streptococci (22%), including encephalopathy (22% versus 7%), meningitis (17% versus 0%), stroke (39% versus 16%), and death (39% versus 9%). Encephalopathy was associated with virulent organisms, increased patient age, and uncontrolled infection. Clinical, radiologic, and neuropathologic data all suggest that infective microemboli are often etiologic in IE-related encephalopathy. There were no macroscopic brain abscesses clinically identified. Meningitis occurred only with virulent organisms. While many clinical aspects of IE have changed in recent years, the frequency and gravity of neurologic complications have not.

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Observer variability in interpreting medical tests and in making diagnoses influences both clinical practice and research. Uniform classification of epileptic seizures is especially difficult. Although the ILAE classification scheme for seizures has been available for many years, the reliability of this system has not been previously assessed. Verbatim descriptions of seizure manifestations were transcribed from medical records as part of a large, population-based prevalence study of childhood epilepsy conducted in two countries in central Oklahoma. One senior neurologist and three neurology residents reviewed these descriptions independently and classified them by seizure type based on the ILAE system. Unweighted and weighted kappa statistics were used to assess the level of agreement between the study neurologist and each resident. The overall agreement between observer pairs in classifying seizure types based on all available descriptions was relatively poor (kappa = 0.24-0.38). Some improvement was evident when unclassified seizures were excluded, and comparisons were restricted to those based on descriptions with some degree of detail (kappa = 0.34-0.51). When specific types of seizures were classified, agreement was fair to excellent for most types (kappa = 0.45-0.90), with the exceptions of atypical absence (kappa = 0.11-0.28), partial seizures with secondary generalization (kappa = 0.26-0.40), and generalized motor seizures (kappa = 0.29-0.32). Sources of observer variability in addition to the classification scheme are considered. Use of specific criteria for the categorization of symptoms might improve the reliability of seizure classification.