Thirty-seven patients who were clinically suspected of having vertebral osteomyelitis were prospectively evaluated with magnetic resonance (MR), radiography, and radionuclide studies. These findings were correlated with the final clinical, microbiologic, or histologic diagnoses. Based on the results of these latter studies, 23 patients were believed to have osteomyelitis. MR examinations consisted of at least a sagittal image (TE = 30 msec, TR = 0.5 sec) and an image obtained at TE = 120 msec, TR = 2-3 sec. All patients underwent radiographic and MR examinations, 36 underwent technetium 99m-HDP bone scanning, and 20 patients underwent gallium 67 scanning. Nineteen patients underwent both bone and gallium scanning. The imaging studies were reviewed independently by investigators blinded to the final diagnoses. MR had a sensitivity of 96%, specificity of 92%, and accuracy of 94%. Combined gallium and bone scan studies (19 cases) had a sensitivity of 90%, specificity of 100%, and accuracy of 94%. Bone scans alone had a sensitivity of 90%, specificity of 78%, and accuracy of 86%. Plain radiographs had a sensitivity of 82%, specificity of 57%, and accuracy of 73%. The MR appearance of vertebral osteomyelitis in this study was characteristic, and MR was as accurate and sensitive as radionuclide scanning in the detection of osteomyelitis.

Aberrant protein processing with tissue deposition is associated with many common neurodegenerative disorders; however, the complex interplay of genetic and environmental factors has made it difficult to decipher the sequence of events linking protein aggregation with clinical disease. Substantial progress has been made toward understanding the pathophysiology of prototypical conformational diseases and protein polymerization in the superfamily of serine proteinase inhibitors (serpins). Here we describe a new disease, familial encephalopathy with neuroserpin inclusion bodies, characterized clinically as an autosomal dominantly inherited dementia, histologically by unique neuronal inclusion bodies and biochemically by polymers of the neuron-specific serpin, neuroserpin. We report the cosegregation of point mutations in the neuroserpin gene (PI12) with the disease in two families. The significance of one mutation, S49P, is evident from its homology to a previously described serpin mutations, whereas that of the other, S52R, is predicted by modelling of the serpin template. Our findings provide a molecular mechanism for a familial dementia and imply that inhibitors of protein polymerization may be effective therapies for this disorder and perhaps for other more common neurodegenerative diseases.

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We report on a new familial neurodegenerative disease with associated dementia that has presented clinically in the fifth decade, in both genders, and in each of several generations of a large family from New York State-a pattern of inheritance consistent with an autosomal dominant mode of transmission. A key pathological finding is the presence of neuronal inclusion bodies distributed throughout the gray matter of the cerebral cortex and in certain subcortical nuclei. These inclusions are distinct from any described previously and henceforth are identified as Collins bodies. The Collins bodies can be isolated by simple biochemical procedures and have a surprisingly simple composition; neuroserpin (a serine protease inhibitor) is their predominant component. An affinity-purified antibody against neuroserpin specifically labels the Collins bodies, confirming their chemical composition. Therefore, we propose a new disease entity-familial encephalopathy with neuroserpin inclusion bodies (FENIB). The conclusion that FENIB is a previously unrecognized neurodegenerative disease is supported by finding Collins bodies in a small kindred from Oregon with familial dementia who are unrelated to the New York family. The autosomal dominant inheritance strongly suggests that FENIB is caused by mutations in the neuroserpin gene, resulting in intracellular accumulation of the mutant protein.

No abstract

For high-noise simulated SPECT data, HOTV-PAPA outperforms TV-PAPA, GPF-EM, and TV-OSL in terms of hot lesion detectability, noise suppression, MSE, and computational efficiency. Unlike TV-PAPA and TV-OSL, HOTV-PAPA does not create sizable staircase artifacts. Moreover, HOTV-PAPA effectively suppresses noise, with only limited loss of local spatial resolution. Of the four methods, HOTV-PAPA shows the best lesion detectability, thanks to its superior noise suppression. HOTV-PAPA shows promise for clinically useful reconstructions of low-dose SPECT data.

A maximum-likelihood (ML) expectation-maximization (EM) algorithm (called EM-IntraSPECT) is presented for simultaneously estimating single photon emission computed tomography (SPECT) emission and attenuation parameters from emission data alone. The algorithm uses the activity within the patient as transmission tomography sources, with which attenuation coefficients can be estimated. For this initial study, EM-IntraSPECT was tested on computer-simulated attenuation and emission maps representing a simplified human thorax as well as on SPECT data obtained from a physical phantom. Two evaluations were performed. First, to corroborate the idea of reconstructing attenuation parameters from emission data, attenuation parameters (mu) were estimated with the emission intensities (lambda) fixed at their true values. Accurate reconstructions of attenuation parameters were obtained. Second, emission parameters lambda and attenuation parameters mu were simultaneously estimated from the emission data alone. In this case there was crosstalk between estimates of lambda and mu and final estimates of lambda and mu depended on initial values. Estimates degraded significantly as the support extended out farther from the body, and an explanation for this is proposed. In the EM-IntraSPECT reconstructed attenuation images, the lungs, spine, and soft tissue were readily distinguished and had approximately correct shapes and sizes. As compared with standard EM reconstruction assuming a fix uniform attenuation map, EM-IntraSPECT provided more uniform estimates of cardiac activity in the physical phantom study and in the simulation study with tight support, but less uniform estimates with a broad support. The new EM algorithm derived here has additional applications, including reconstructing emission and transmission projection data under a unified statistical model.

SUMMARY Right ventricular (RV) performance during supine bicycle exercise was evaluated by gated equilibrium nuclear angiography in 19 clinically well children with d-transposition of the great arteries (d-TGA), 6.4 ± 2.7 years after Mustard's operation. Comparisons were made between rest and peak exercise. The mean resting ejection fraction was 44 ± 12% (range 30-75%) and was unchanged at peak exercise. Eight children had a normal ejection fraction response, whereas 11 children had either no increase or a decrease in ejection fraction. Relative end-diastolic volumes decreased from resting values in all patients who had an abnormal ejection fraction response. Among patients whose ejection fraction increased, the end-diastolic volume increased in three, decreased in four and was unchanged in one at peak exercise. Heart rate increased 84% (range 52-135%) and systolic blood pressure increased 16% (range 0-28%) at peak exercise. There was no correlation between exercise response and age at surgery or interval since surgery. These data indicate that clinically well children after Mustard's procedure may have abnormal right ventricular function under stress, raising concerns about the ability of the right ventricle to function as the systemic ventricle.