Post-polio syndrome patients treated with intravenous immunoglobulin: a double-blinded randomized controlled pilot study

Post-polio syndrome (PPS) is characterized by new muscle weakness, atrophy, fatigue and pain developing several years after the acute polio. Some studies suggest an ongoing inflammation in the spinal cord in these patients. From this perspective, intravenous immunoglobulin (IvIg) could be a therapeutic option. We performed a double-blinded randomized controlled pilot study with 20 patients to investigate the possible clinical effects of IvIg in PPS. Twenty patients were randomized to either IvIg 2 g/kg body weight or placebo. Primary endpoints were changes in pain, fatigue and muscle strength 3 months after treatment. Surrogate endpoints were changes in cerebrospinal fluid (CSF) cytokine levels. Secondary endpoints were pain, fatigue and isometric muscle strength after 6 months. Patients receiving IvIg reported a significant improvement in pain during the first 3 months, but no change was noted for subjective fatigue and muscle strength. CSF levels of tumour necrosis factor- α (TNF- α ) were increased compared with patients with non-inflammatory neurological disorders. In conclusion, in this small pilot study no effect was seen with IvIg treatment on muscle strength and fatigue, however IvIg treated PPS patients reported significantly less pain 3 months after treatment. TNF- α was increased in the CSF from PPS patients. The results are promising, but not conclusive because of the low number of patients studied.     

Activity and distribution of phosphoinositidase C in rat sciatic nerve.

The hydrolysis of phosphatidylinositol-4,5-bisphosphate (PIP2) by rat sciatic nerve cytosolic phosphoinositidase C [phosphoinositide-specific phospholipase C (PIC)] was studied at neutral pH and at ionic concentrations that approximate intracellular conditions. The principal water-soluble product formed was shown to be inositol trisphosphate by anion exchange chromatography. The maximum hydrolysis rate (2.5 nmol/min/mg protein) was achieved at less than 100 nM Ca2+. Hydrolysis was markedly increased to 15 nmol/min/mg protein by inclusion of K+ in the reaction mixture. In the presence of 200 mM K+, the optimum Ca2+ was increased to approximately 600 nM. Higher Ca2+ concentrations progressively inhibited PIP2 hydrolysis. Mg2+ also inhibited the reaction, but the presence of equimolar amounts of ATP and Mg2+ had no effect. Appreciable degradation of phosphatidylinositol-4-phosphate (PIP) also occurred in the nanomolar Ca2+ range, whereas breakdown of phosphatidylinositol (PI) required millimolar Ca2+. The presence of PIP but not PI inhibited PIP2 hydrolysis. Upon subcellular fractionation of nerve, more than 50% of recovered PIC activity was in the cytosol and about 20% was located in a myelin-enriched fraction. Using PIP2 as substrate, PIC activities in nerves from normal and streptozotocin-induced diabetic animals were not different. However, the myelin-associated enzyme from diabetic animals was more labile to freezing and thawing.     

Macrophage-like cells from explant cultures of rat sciatic nerve produce apolipoprotein E

Apolipoprotein E is synthesized and secreted by degenerating peripheral nerve, but the role of resident endoneurial cells in this process is not clear. To exclude the involvement of nonresident cells, we examined the cellular source of endoneurial apolipoprotein E in explant cultures of rat sciatic nerve. The cellular outgrowth from these explant cultures released apolipoprotein E into the culture medium. The cellular outgrowth contained fibroblasts, Schwann cells, and a population of cells with many phenotypic characteristics of macrophages, including the production of apolipoprotein E. No other cell type in the cultures appeared to contribute to this production. These data suggest that apolipoprotein E is produced by resident endoneurial cells in explant cultures and that these cells are macrophages.     

Neuropathy in children and adolescents with diabetes mellitus

Nerve conduction velocities were studied in the median, posterior tibial, radial and sural nerves of 50 juvenile diabetics, average age 13 +/- 1.3 years and mean duration of diabetes 2.3 +/- 1.4 years. Motor conduction velocity (MCV) in the median nerve was reduced in 10% of the subjects, and in the posterior tibial in 32%. Sensory conduction velocity (SCV) in the radial nerve was reduced in 30% of the subjects, and in the sural in 44%. No relationship was found between the reduction in conduction velocity and the duration of diabetes; nevertheless, a correlation was observed between this reduction and the degree of glycaemic control represented by the glycosylated haemoglobin concentration. The authors emphasize the importance of good glycaemic control for the prevention of diabetic neuropathy.     

New approach to management of intraaortic balloon pumps in patients with peripheral vascular disease: Case reports of four patients requiring urgent IABP insertion

Four selected cases of emergent IABP insertion in PV patients are presented. After angiographic documentation of critical iliac stenosis, conservative peripheral angioplasty was performed prior to IABP insertion. No patient experienced a peripheral ischemic event associated with IABP use. © 1992 Wiley-Liss, Inc.     

The use of a dedicated rectosigmoidoscope for ultrasound staging of tumours of the upper and middle third of the rectum

OBJECTIVE: Tumours of the upper rectum, and many in the middle third, are not accessible to endorectal ultrasound staging because of the difficulty in reaching all sites of the rectum with a rigid probe. The aim of this prospective study was to assess whether using a dedicated rectosigmoidoscope, endorectal ultrasonography (ERUS) can accurately stage any rectal lesion irrespective of its distance from the anal verge. METHOD: A total of 173 consecutive patients with a primary rectal tumour were included. A rotating, high multifrequency (5.0-10 MHz) endoprobe was introduced through a dedicated rectosigmoidoscope and advanced above the lesion. A computer allowed for three-dimensional (3D) reconstruction of 2D images. Treatment was selected on the basis of 3D-ERUS findings. ERUS staging was correlated with pathological staging. RESULTS: The depth of invasion was correctly determined by 3D-ERUS in 78.2% of tumours of the lower rectum, 76.4% of tumours extending between the lower and middle third of the rectum, 80.9% of tumours of the middle third of the rectum, 78.5% of tumours extending between the middle and upper third of the rectum and 78.9% of tumours of the upper rectum. The accuracy for the absence of lymph node metastases was 81.2% for tumours of the lower rectum, 78.5% for tumours extending between the lower and middle third of the rectum, 85.7% for tumours of the middle third of the rectum, 83.3% for tumours extending between the middle and upper third of the rectum and 78.5% for tumours of the upper rectum. Analysis showed that there was no difference between the various tumour sites. CONCLUSION: Our findings indicate that using a dedicated proctosigmoidoscope, tumours of the upper and middle third of the rectum are equally accessible to ultrasonographic evaluation. The distance of the tumour from the anal verge does not influence the accuracy of examinations considered adequate by the operator.     

Fatal encephalitis in a patient with refractory celiac disease presenting with myorhythmia and carpal spasm.

We report the case of a woman with refractory celiac disease who developed abnormal spontaneous movements of the extremities and face consistent with myorhythmia. Investigation led to a diagnosis of encephalitis, confirmed by postmortem examination. The movements were likely caused by nonparaneoplastic encephalitis associated with refractory celiac disease. Etiologic and diagnostic considerations and treatment options are discussed.     

A case of congenital supratentorial tumor: Atypical teratoid/rhabdoid tumor or primitive neuroectodermal tumor?

Two embryonal CNS tumors, atypical teratoid/rabdoid tumor (AT/RT) and primitive neuroectodermal tumor (PNET), may be confused with each other and misdiagnosed. Here we report an infant with a congenital supratentorial tumor, which was detected by fetal MRI at 37 weeks gestation. On routine histological examination, the tumor was composed mainly of small undifferentiated cells, among which many rhabdoid cells and occasional sickle‐shaped embracing cells were observed. No mesenchymal or epithelial areas were evident. Our impression was that the tumor was an atypical example of AT/RT. Immunohistochemically, almost all the tumor cells were strongly positive for vimentin. However, epithelial membrane antigen was notably negative, and most of the tumor cell nuclei were clearly positive for INI1. In addition, many tumor cells were positive for neurofilament protein. There were also occasional small areas containing many tumor cells positive for glial fibrillary acidic protein. Finally, a diagnosis of PNET, with a rhabdoid phenotype and expression of neuronal and glial markers, was made. In the present case, application of INI1 immunostaining was very helpful for distinguishing PNET from AT/RT.