A case of monofocal motor neuropathy with GM1 and GD1b antibodies improved with intravenous immunoglobulin.

Multifocal motor neuropathy is a purely motor neuropathy with a probably dysimmune pathogenesis, supported by the presence of anti-GM1 IgM antibodies in about half of the cases. Single nerve involvement allows for diagnosis of possible multifocal motor neuropathy. We present the case of a middle age man presenting with progressive weakness and hypotrophy in the left leg and difficulty in walking, in which we have diagnosed a dysimmune mononeuropathy. Treatment with IVIg was performed with substantial improvement. Although only one nerve is involved, early diagnosis of dysimmune mononeuropathy is important to start IVIg treatment that is often decisive.

The Wandering Spleen.

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Outcomes of Bleomycin-based electrochemotherapy in patients with repeated loco-regional recurrences of vulvar cancer.

Objective To evaluate the safety, local tumor efficacy and relief of symptoms of electrochemotherapy (ECT) treatment in patients affected by recurrence of vulvar cancer (VC), unsuitable for standard treatments. Methods Ten patients were recruited with histological diagnosis of recurrence of VC. Intravenous bleomycin was injected, after an accurate mapping of all lesions and ECT was performed. Response to therapy was evaluated and quality of life (QoL) was evaluated via questionnaires. Results Diagnosis stage of primary tumors, according to the FIGO system, was: four patients respectively at stage IB (40%), and at stage II (40%), one patient at stage IIIA (10%), one patient with Paget cancer (10%). Mean age was 76 years (SD ± 7) at time of enrollment. Eight patients (80%) were previously submitted to surgery and/or radio-chemotherapy. Mean treatment time was 20 (range 10-20) min. After a median follow-up of 12 (3-22) months, six patients (60%) were alive. Conclusions Objective responses (ORs) with local control of the tumor were obtained in 80%. After a mean follow-up of 12 (3-22) months six patients (60%) were alive. The favorable outcome of this study, indicates that ECT is a reliable treatment option that may improve their functioning, thus enhancing the care provided in the palliative setting.

Clinical validation of glucose pump test (GPT) compared with ultrasound dilution technology in arteriovenous graft surveillance.

BACKGROUND: Blood flow (Qa) measurements are an important step in the surveillance protocol of haemodialysis vascular access (VA). The glucose pump test (GPT) is a new test for Qa measurement based on the dilution of a constant glucose infusion. The aim of this study is to verify the clinical accuracy of GPT in a graft surveillance protocol with sequential Qa measurements. METHODS: In 30 chronic haemodialysis patients with graft, we compared monthly sequential Qa measurements performed with GPT in pre-dialysis and the ultrasound dilution technique (HD01 device Transonic Systems Inc., USA) during dialysis. The colour Doppler ultrasonography study (CDU) was our reference standard for the diagnosis of stenosis. The endpoints were the graft thrombosis or PTA treatment. RESULTS: According to the K/DOQI guidelines we could identify the thrombosis high-risk grafts when Qa was <600 ml/min or <1000 ml/min with a decrease >25% in serial Qa measurements. HD01 yielded 27 of 112 high-risk Qa measurements (21 Qa <600 ml/min; mean 406+/-145 ml/min; 6 deltaQa >25%; mean 43+/-7%). In 12 of 27 cases the CDU control did not show haemodynamically significant stenoses (false positive); 15 of 27 cases were confirmed high-risk accesses by CDU and did PTAs (HD01 specificity 86%). GPT yielded 14 of 112 high-risk Qa measurements (8 Qa <600 ml/min; mean 404+/-135 ml/min; 6 deltaQa >25%; mean 38+/-8%) and all had severe stenoses and underwent PTA treatments showing a GPT specificity of 100%. The CDU study allowed us to correctly assess the Qa negative cases. HD01 method had 10 false negative cases (treated or clotted grafts with a Qa >600 ml/min and deltaQa <25%) with a sensitivity of 60%, while GPT had 11 false negative cases with a sensitivity of 56%. The diagnostic accuracy tested with the ROC curves was similar with both tests (area under the curve was 0.762 and 0.752 with GPT and ultrasound dilution, respectively; P = 0.985). The diagnostic efficiency (percentage of grafts with agreement between test result and factual situation) was 90 and 80% (P = 0.056) for GPT and HD01, respectively. CONCLUSION: Compared with HD01, the GPT had a lower false positive rate and similar diagnostic accuracy and efficiency. The clinical implication is a smaller number of unnecessary, invasive procedures (angiographies or PTAs), without increasing the thrombosis risk. This study has shown that GPT is an accurate, quick and economic test for Qa monitoring.