Encephalo-myelo-radiculo-ganglionitis presenting as pandysautonomia

A 68-year-old man developed pandysautonomia with severe orthostatic dysfunction, fixed heart rate, low serum levels of norepinephrine and epinephrine, absent sympathetic skin responses, and pupillary abnormalities. CSF protein was 92 mg/dl. In spite of a good recovery following corticosteroid administration, a relapse occurred, with accompanying sensory symptoms confined to both arms. Fatal sudden cardiac arrest occurred after 4 months. Autopsy revealed numerous lymphocytic infiltrates, predominantly in autonomic and sensory ganglia and, to a lesser extent, in the nerve roots, spinal cord, and brainstem without evidence for an underlying tumor. This case provides histopathologic evidence for an inflammatory etiology of panautonomic neuropathy in some patients.     

Work-related injuries in minors

Since 1938, federal child labor laws have restricted employment of persons under 18 years old, in part to protect them from hazardous occupations. Workers' compensation claims reported to the Supplementary Data System of the Bureau of Labor Statistics were examined to define the current status of occupational injuries among minors. Data tapes from 1980 to 1983 were searched to identify all current claims for injuries and illnesses occurring in 1980 in persons under age 18. Injury rates were calculated using information about employment in 1979 available from the 1980 census. In the 24 states included in this study, 23,823 claims were reported for persons less than 18 years old. Of these claims, approximately 10% were from persons under age 16. Rates of injuries in 16- and 17 year olds were 12.6 per 100 full-time male workers and 6.6 per 100 full-time female workers. Serious injuries included fractures, dislocations, and amputations, accounting for 5.8%, 0.7%, and 0.6% of cases, respectively. California, the only state that coded whether injuries resulted in fatalities, reported 12 deaths in this age group. Machines and vehicles, many of which are restricted under child labor laws, accounted for 8.3% and 5.8% of claims. These data suggest that persons under age 18 years are not adequately protected from occupational injury. Further attention and, possibly, new preventive strategies are needed.     

Monitoring left ventricular dilation in mice with PET.

Molecular imaging by small-animal PET is an important noninvasive means to phenotype transgenic mouse models in vivo. When investigating pathologies of the left ventricular (LV) myocardium, the serial assessment of LV volumes is important. By this, the presence of LV dilation as a sign of developing heart failure can be detected. Whereas PET is usually used to derive biochemical and molecular information, functional parameters such as ventricular volumes are generally measured using echocardiography or MRI. In this study, a novel method to monitor LV dilation in mice with PET is presented and evaluated using cardiac MRI. METHODS: A semiautomatic 3-dimensional algorithm was used to delineate the LV myocardial wall on static PET images depicting myocardial glucose metabolism ((18)F-FDG PET) for 20 mice: 10 wild-type and 10 genetically modified littermates designed to develop a dilative cardiomyopathy phenotype (cardiomyocyte-specific knockout of survivin). The volume enclosed by the 3-dimensional midmyocardial contour was calculated as a measure for LV volume for each mouse. Data were compared with ventricular volumes measured by MRI in the same animals. RESULTS: LV volumes obtained by PET and MRI correlated well (R = 0.89) for hearts with small and large left ventricles. In accordance with the hypothesis, the LV volumes were increased significantly for transgenic mice examined at an older age compared with those examined at a younger age (MRI: 160.5 +/- 25.7 microL vs. 114.7 +/- 15.2 microL [P = 0.012]; PET: 129.3 +/- 15.3 microL vs. 73.8 +/- 15.0 microL [P < 0.001], all values shown as mean +/- SD; for MRI, mean of end-diastolic and end-systolic volumes are given), whereas they did not for their wild-type littermates (MRI: 106.2 +/- 12.3 microL vs. 94.7 +/- 14.6 microL [P = 0.214]; PET: 82.6 +/- 20.9 microL vs. 65.0 +/- 16.9 microL [P = 0.185]). CONCLUSION: Evaluation and quantitation of LV dilation in both control and cardiomyopathic mice can be reliably and serially performed using small-animal PET and (18)F-FDG, yielding useful functional information in addition to metabolic data.     

Scintigraphic evaluation of hepatic blood flow after intrahepatic portosystemic shunt (TIPS)

In patients with liver cirrhosis a transjugularly placed intrahepatic portocaval shunt (TIPS) is a non-surgical portosystemic device which aims to reduce portal venons pressure. In comparison with Doppler sonography, we evaluated in 28 patients the diagnostic impact of liver perfusion scintigraphy (with technetium-99m diethylene triamine penta-acetic acid) in the assessment of changes in the hepatic blood flow after TIPS shunting. The arterial and portal contributions to hepatic flow were calculated from the areas under the biphasic timeactivity curve. In the course of TIPS shunting, patency is threatened by reocclusion. Angiography is the gold standard for TIPS shunt reassessment. However, there is a need for a less invasive diagnostic procedure, such as scintigraphy or Doppler sonography, for the early detection of shunt insufficiency. Scintigraphy demonstrated that prior to TIPS shunting the portal venons contribution to hepatic perfusion was reduced to 29.2%, this reduction being due to portal hypertension. After TIPS placement a significant increase in portal venous perfusion was observed (38.2%;P<0.02). TIPS shunt occlusion was identified in patients by a significant reduction in the scintigraphically measured portal venons contribution to hepatic blood flow. Hepatic perfusion scintigraphy appears to be a valuable method to determine the immediate effect of TIPS on hepatic blood flow. Post-TIPS follow-up studies of hepatic haemodynamics by liver perfusion scintigraphy appear able to contribute to the detection of TIPS shunt occlusion before the clinical consequences of this complication have become apparent.     

Preselection Thymocytes Are More Sensitive to T Cell Receptor Stimulation Than Mature T Cells

During T cell development, thymocytes which are tolerant to self-peptides but reactive to foreign peptides are selected. The current model for thymocyte selection proposes that self-peptide–major histocompatibility complex (MHC) complexes that bind the T cell receptor with low affinity will promote positive selection while those with high affinity will result in negative selection. Upon thymocyte maturation, such low affinity self-peptide–MHC ligands no longer provoke a response, but foreign peptides can incidentally be high affinity ligands and can therefore stimulate T cells. For this model to work, thymocytes must be more sensitive to ligand than mature T cells. Contrary to this expectation, several groups have shown that thymocytes are less responsive than mature T cells to anti-T cell receptor for antigen (TCR)/CD3 mAb stimulation. Additionally, the lower TCR levels on thymocytes, compared with T cells, would potentially correlate with decreased thymocyte sensitivity. Here we compared preselection thymocytes and mature T cells for early activation events in response to peptide–MHC ligands. Remarkably, the preselection thymocytes were more responsive than mature T cells when stimulated with low affinity peptide variants, while both populations responded equally well to the antigenic peptide. This directly demonstrates the increased sensitivity of thymocytes compared with T cells for TCR engagement by peptide–MHC complexes.     

Determination of equine serum inhibitors for poliovirus by the gel-adsorption technique

Summary Inhibitors found in certain equine sera active against poliovirus type 1 have been determined by the gel-adsorption method using aluminiumhydroxide-gel in the absence of cells. The inhibitor belongs to the 19S-class of macroglobulins (IgM), as revealed by gel-filtration with Sephadex G 200, by DEAE-cellulose-chromatography, and by sucrose density centrifugation. It is bound to the viral surface in-vitro in the absence of tissue cells. The specific complex may be precipitated with anti-equine globulin from rabbits. The inhibitor is destroyed by papain and by 2-mercapto-ethanol. The residual infectivity (10–25%) has been found bound to the inhibitor in-vitro. Its behavior in the gel-adsorption system is not altered if the virus-inhibitor complexes have previously been diluted. — Nonsensitive mutants and double mutants have been selected. There must exist at least three different combining sites for equine inhibitors on the surface of poliovirus type 1, strain Mahoney. Equine sera may be grouped according to the specificity of their inhibitory activity. The specific binding capacity is lost if the virus is heated at 50° C for 30 min.Supported by the Deutsche Forschungsgemeinschaft, Unit Medizinische Virologie; partly presented at the 5th Viruscolloquium of the Deutsche Forschungsgemeinschaft, Marburg Sept. 1965.The authors wish to acknowledge the excellent technical assistance of MissSigrid Bonk.     

Significant association of strictures and internal fistula formation in Crohn’s disease

Intestinal inflammation in Crohn’s disease (CD) may be complicated by the occurrence of strictures and fistulae. The pathogenesis of fistula formation is unknown. We therefore wanted to determine whether mechanical factors might contribute to the development of fistulae. Furthermore, we tried to define the path of internal fistulae through the muscular layer. For this purpose, surgical resection specimens from 42 consecutive patients with CD were prospectively studied. In gross examination the whole bowel was cut into circumferential cross sections 0.3 cm thick. Abnormal areas were histologically examined. Strictures were found in 38 patients (90.5%), and fistulae were observed in 27 (64.3%) patients. In 11 (40.7%) specimens fistulae were found within a stricture, in 15 (55.6%) at the proximal end, and in 1 (3.7%) no stricture was found. In 7 (25.9%) cases with fistulae, herniated mucosa was found within the muscularis propria or the subserosa. In 7 (25.9%) cases a blood vessel was identified near a fistula traversing the muscularis propria. From these findings we conclude that that mechanical factors may contribute to fistula formation. This is further supported by the fact that fistulae appear to traverse the muscular layer along piercing vessels. Received: 21 January 2000 / Accepted: 7 March 2000