Catastrophic antiphospholipid syndrome: response to repeated plasmapheresis over three years.

The catastrophic antiphospholipid syndrome (CAPS) is rare and usually fatal. In this report, we describe an unusual patient who, 31 years after experiencing an atypical preeclampsia-eclampsia presentation known today as the HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets), developed CAPS, which seemed to complicate a diagnosis of primary antiphospholipid syndrome. She responded to repeated plasmapheresis over 3 years. Anticoagulants, corticosteroids, intravenous gamma globulin, and intravenous cyclophosphamide had all failed to halt the progression of CAPS, but repeated plasmapheresis not only halted the condition, but it led to the reversal of a leukoencephalopathy. The relationship between HELLP syndrome and CAPS is discussed, and possible pathogenetic mechanisms that explain the efficacy of repeated plasmapheresis in this setting are suggested. It is postulated that perhaps plasmapheresis, through removal of cytokines or other mediators, disrupts the interaction between phospholipid-protein complexes and endothelial cells. Repeated plasmapheresis should be considered in the most refractory cases of CAPS when more conventional treatment regimens have failed.

Central nervous system systemic lupus erythematosus mimicking progressive multifocal leucoencephalopathy.

The case is reported of a patient with central nervous system systemic lupus erythematosus (SLE) with features of progressive multifocal leucoencephalopathy (PML) seen clinically and by magnetic resonance imaging. A brain biopsy sample showed microinfarcts. The use of magnetic resonance imaging and IgG synthesis rates in evaluating central nervous system lupus, the co-occurrence of SLE and PML, and the differentiation of these entities by magnetic resonance imaging and by histology are considered.

Renal infarction: CT diagnosis and correlation between CT findings and etiologies.

The CT scans and the clinical records of 12 patients who had renal infarction were reviewed. The renal infarcts were classified as either focal or global. The CT findings were correlated with the etiologies of renal infarction. Embolism was the most common cause of renal infarcts that were multifocal with involvement of both kidneys. Trauma caused a unilateral global type of infarct. A case of sickle cell anemia presented with multiple "slit-like" focal infarcts and enlarged kidneys. Forty-seven per cent of infarcts demonstrated the cortical rim sign, 11% were associated with mass effect, 21% had a subcapsular fluid collection, and 6% had an abnormally thickened renal fascia.

CT pancreatogram in carcinoma of the pancreas and chronic pancreatitis.

CT has made it possible to determine the contour of the pancreatic duct, to measure its caliber, and to detect dilatation of the duct. CT scans of 75 patients with pancreatic carcinoma and of 45 patients with chronic pancreatitis were obtained. Dilatation of the pancreatic duct was seen in 56% of patients with carcinoma, and in 70% of those with tumors confined to the pancreatic head and body. Smooth dilatation (43%) or beaded dilatation (40%) were most commonly associated with carcinoma. Ductal dilatation was present in 58% of the patients with chronic pancreatitis, and irregular dilatation was seen in 73% of the patients in this group. About half of the patients who had irregular dilatation had calculi within the ducts. The duct contour was similar to that seen in carcinoma in 27% of the cases of chronic pancreatitis. There was a significant difference in the caliber of the duct (P = .01) with larger ducts seen in patients with carcinoma. The width of the pancreatic gland (P = .005) and the ratio of duct caliber to gland width differed (P = .001) between the two diseases: the gland was wider in cases of chronic pancreatitis, and the ratio of duct to gland was larger in cases of carcinoma. Eight cases of dilatation of the duct with no detectible pancreatic mass were seen in a subgroup of 13 patients who had small carcinomas of the pancreas (tumor size of 3 cm or less). Our findings indicate that a dilated pancreatic duct with a smooth contour and a ratio of duct to total gland width of 0.50 or greater suggests carcinoma as the underlying pathology.

CT appearance of global renal infarction.

The computed tomographic (CT) appearance of global infarction of the kidney has not been previously reported. We report a clinically proven case as well as two other cases with clinical and radiographic findings consistent with renal infarction. The CT appearance of global renal infarction corresponds with its conventional intravenous urographic appearance. A thin rim of perfused cortical tissue, supplied by collateral vessels, surrounds the nonperfused central renal parenchyma.

Computed tomography of the spleen.

Computed tomography (CT) has proved to be valuable in the diagnosis of a wide variety of congenital, inflammatory, traumatic, and neoplastic lesions of the spleen, in addition to identifying certain normal variants which may present problems. A comprehensive review of the CT characteristics of splenic lesions and variants is provided, and CT is compared with other imaging modalities, principally radionuclide imaging.

Computed tomographic evaluation of the dilated pancreatic duct: the value of thin-section collimation.

Three patients with surgically proved pancreatic carcinoma and one with chronic and acute pancreatitis were evaluated by computed tomography. Scans through the pancreatic region were initially performed with standard 10-mm collimation and then repeated using thin-section (5-mm) collimation. In all cases a pathologically dilated pancreatic duct was either not seen or faint with 10-mm collimation, whereas it was clearly identified using the thin-section collimator.

The normal pancreas: acoustic characteristics and frequency of imaging.

This article reports the results of a study designed to evaluate both pancreatic echogenicity and success of visualizing recognizable pancreatic tissue. The acoustic characteristics of the normal pancreas were compared with those of the liver. Of 100 scans reviewed, 65 scans (65 percent) depicted recognizable pancreatic tissue. Two cases were discarded on the basis of hepatocellular disease, and one case was discarded because of hepatic metastases. Thirty-two cases demonstrated pancreatic echogenicity greater than that of the liver, and 30 cases demonstrated pancreatic echogenicity equivalent to that of the liver. We found no normal pancreas that displayed less echogenicity than the liver. A hypothesis is offered to explain the observed results.

Physiologic myometrial thickening: a source of confusion.

Focal myometrial thickening, thought to represent a focal transient myometrial contraction, was observed to disappear during the course of an ultrasonic examination.

Nerve rootlet avulsion. A complication of myelography using the Chynn needle.

The authors report a case of traumatic removal of nerve tissue during myelography with the Chynn aspiration cannula. If such a situation occurs, the authors suggest making a second puncture at a different level.