CD3+ B-1a Cells as a Mediator of Disease Progression in Autoimmune-Prone Mice.

B-1a cells are distinguishable from conventional B cells, which are designated B-2 cells, on the basis of their developmental origin, surface marker expression, and functions. In addition to the unique expression of the CD5 antigen, B-1a cells are characterized by the expression level of CD23. Although B-1a cells are considered to be independent of T cells and produce natural autoantibodies that induce the clinical manifestations of autoimmune diseases, there is much debate on the role of B-1a cells in the development of autoimmune diseases. We examined the involvement of B-1a cells in autoimmune-prone mice with the lpr gene. MRL/lpr and B6/lpr mice exhibited lupus and lymphoproliferative syndromes because of the massive accumulation of CD3+CD4-CD8-B220+ T cells. Interestingly, the B220+CD23-CD5+ (B-1a) cell population in the peripheral blood and peritoneal cavity increased with age and disease progression. Ninety percent of B-1a cells were CD3 positive (CD3+ B-1a cells) and did not produce tumor necrosis factor alpha, interferon gamma, or interleukin-10. To test the possible involvement of CD3+ B-1a cells in autoimmune disease, we tried to eliminate the peripheral cells by hypotonic shock through repeated intraperitoneal injections of distilled water. The fraction of peritoneal CD3+ B-1a cells decreased, and symptoms of the autoimmune disease were much milder in the distilled water-treated MRL/lpr mice. These results suggest that CD3+ B-1a cells could be mediators of disease progression in autoimmune-prone mice.

Correct localization of epileptogenic focus with I-123 iomazenil cerebral benzodiazepine receptor imaging: a case report of temporal lobe epilepsy with discordant ictal cerebral blood flow SPECT.

A 26-year-old female with intractable epileptic seizures was studied with I-123 iomazenil cerebral benzodiazepine receptor, I-123 IMP inter-ictal and Tc-99m ECD ictal cerebral blood flow SPECT. The ictal cerebral blood flow SPECT indicated the location of the seizures to be in the left temporal lobe, where increased regional cerebral blood flow was noted in marked contrast to the inter-ictal SPECT. Ictal electroencephalograms (EEGs) recorded with scalp and sphenoidal electrodes also suggested the left temporal lobe as the location of the seizures. On I-123 iomazenil SPECT, however, decreased benzodiazepine receptor density was demonstrated in the right temporal lobe. MRI showed mild atrophy and abnormal signal intensity in the right temporal lobe. Ictal EEGs recorded with intracranial electrodes revealed that abnormal electrical activity of the brain always emerged from the right temporal lobe and then propagated to the contralateral side. Based on the findings of intracranial EEGs, partial resection of the right anterior temporal lobe including hippocampus was performed. After the surgery, no seizure occurred. Pathological examination of the surgical specimens revealed hippocampal sclerosis. This case suggested that cerebral benzodiazepine receptor imaging with I-123 iomazenil can be helpful for correct localization of epileptogenic foci.

Low-field-strength MR imaging of failed hip arthroplasty: association of femoral periprosthetic signal intensity with radiographic, surgical, and pathologic findings.

PURPOSE: To investigate the association between periprosthetic signal intensity at low-field-strength magnetic resonance (MR) imaging after failed hip arthroplasty and radiographic, surgical, and pathologic findings. MATERIALS AND METHODS: The study group comprised 22 consecutive women who underwent hip arthroplasty (mean age, 62 years; age range, 35-74 years). All patients underwent MR imaging prior to revision surgery. Coronal fast short inversion time inversion-recovery (STIR) images and spin-echo T1-weighted images were obtained with a 0.5-T MR imaging unit before and after administration of contrast material. The periprosthetic region was divided into the seven femoral Gruen zones. Two observers retrospectively analyzed signal intensity patterns. Association of signal intensity patterns with radiographic, surgical, and pathologic findings was determined with chi2 analysis and generalized estimating equations. RESULTS: Diagnostic-quality images were obtained for 150 zones. Periprosthetic signal intensity was greater than that of bone marrow in the distal femur on the fast STIR images, and no contrast enhancement was seen on the T1-weighted images (type I signal intensity pattern) in 11 zones. Signal intensity was greater than that of bone marrow on the fast STIR images, and contrast enhancement was seen on the T1-weighted images (type II signal intensity pattern) in 45 zones. Signal intensity was less than or equal to that of bone marrow on the fast STIR images, and no contrast enhancement was seen on the T1-weighted images (type III signal intensity pattern) in 94 zones. Type I and II patterns were associated with focal or nonfocal lucency, an unstable stem, and fibrosis or granuloma. A type III pattern was associated with a normal radiographic appearance, a stable stem, and normal bone tissue. Significant association was demonstrated between periprosthetic signal intensity and radiographic (P <.001, chi2 test and generalized estimating equations), surgical (P <.05, Mantel-Haenszel chi2 test and generalized estimating equations), and pathologic findings (P <.05, chi2 test). CONCLUSION: Low-field-strength MR imaging depicted periprosthetic tissue signal intensity that was significantly associated with radiographic, surgical, and pathologic findings.

Molecular analysis of congenital central hypoventilation syndrome.

Congenital central hypoventilation syndrome (CCHS or Ondine's curse; OMIM 209880) is a disorder characterized by an idiopathic failure of the automatic control of breathing. CCHS is frequently complicated with neurocristopathies such as Hirschsprung's disease (HSCR). The genes involved in the RET-GDNF signaling and/or EDN3-EDNRB signaling pathways have been analyzed as candidates for CCHS; however, only a few patients have mutations of the RET, EDN3, and GDNF genes. Recently, mutations of the PHOX2B gene, especially polyalanine expansions, have been detected in two thirds of patients. We studied the RET, GDNF, GFRA1, PHOX2A, PHOX2B, HASH-1, EDN1, EDN3, EDNRB, and BDNF genes in seven patients with isolated CCHS and three patients with HSCR. We detected polyalanine expansions and a novel frameshift mutation of the PHOX2B gene in four patients and one patient, respectively. We also found several mutations of the RET, GFRA1, PHOX2A, and HASH-1 genes in patients with or without mutations of the PHOX2B gene. Our study confirmed the prominent role of mutations in the PHOX2B gene in the pathogenesis of CCHS. Mutations of the RET, GFRA1, PHOX2A, and HASH-1 genes may also be involved in the pathogenesis of CCHS. To make clear the pathogenesis of CCHS, the analysis of more cases and further candidates concerned with the development of the autonomic nervous system is required.

[Evaluation of ultra-low-dose thoracic multi-detector-row CT using different reconstruction kernels and new reconstruction algorithms].

CT has great advantages in detecting early-stage small lung cancer and is becoming common in lung cancer screening. Multi-detector-row CT (MDCT) can provide thin-slice images with low radiation exposure. In this study, ultra-low-dose (5 mAs: 10 mAs, 0.5 sec/rot) thoracic MDCT images were evaluated. We describe the differences in image quality and quantity between the different reconstruction kernels. We also propose a new reconstruction algorithm (ultra-low-dose reconstruction algorithm: ULR) for ultra-low-dose thoracic CT, to reduce noise and streak artifacts. We are convinced of the usefulness and possibility of ultra-low-dose thoracic MDCT with ULR algorithms for lung cancer screening.

Moderately T2-weighted images obtained with the single-shot fast spin-echo technique: differentiating between malignant and benign urinary obstructions.

The purpose of this study was to determine whether a distinction could be made between benign and malignant urinary obstructions in moderately T(2)-weighted images obtained with the single-shot fast spin-echo technique. Forty-four lesions in 39 patients with urinary obstruction were evaluated with the single-shot fast spin-echo (SSFSE) technique with an effective TE of 90-100 ms and without fat saturation. Benign and malignant lesions were compared for the presence of ureteral wall thickening and a signal intensity relative to the proximal ureteral wall. Statistically significant differences were found between benign and malignant lesions in both morphologic change (P<0.0001) and signal intensity of the lesions at the obstruction position (P<0.0001). The combination of wall thickening and increased signal intensity as a predictor of malignant disease yielded a sensitivity of 88% and a specificity of 100%. Neither increased signal intensity nor wall thickening as a predictor of benign disease yielded a sensitivity of 89% and a specificity of 88%. The moderately T(2)-weighted SSFSE technique without fat saturation can accurately distinguish between benign and malignant urinary obstructions.