[Severe polyneuropathy in primary Sjögren's syndrome : Sjögren's syndrome should be considered in patients with motor neuropathy]. / Schwere Polyneuropathie bei primärem Sjögren-Syndrom : Bei Patienten mit motorischen Neuropathien auch an Sjögren-Syndrom denken!

A 64-year-old male patient developed over a period of 20 years a peripheral neuropathy symmetrically affecting the upper and lower limbs. The histological examination of a sural nerve biopsy revealed a severe axonal neuropathy. Despite extensive laboratory investigations including immunological and metabolic tests the origin could not be identified. Finally, a Schirmer test revealed xerophthalmia. A subsequent salivary gland biopsy from the lower lip revealed a grade III lymphocytic inflammation according to Chisholm and Mason and confirmed the diagnosis of Sjögren's syndrome.

Serological indication of chronic inflammatory demyelinating polyneuropathy as an extrahepatic manifestation of hepatitis E virus infection.

Guillain-Barré syndrome and neuralgic amyotrophy have been associated with hepatitis E virus (HEV) genotype 3 infections, while myasthenia gravis (MG) has been associated with HEV genotype 4 infections. However, whether chronic inflammatory demyelinating polyneuropathy (CIDP) is associated with HEV infections has not been conclusively clarified yet. 102 CIDP patients, 102 age- and sex-matched blood donors, 61 peripheral neuropathy patients (non-CIDP patients), and 26 MG patients were tested for HEV and anti-HEV IgM and IgG. Sixty-five of the 102 (64%) CIDP patients tested positive for anti-HEV IgG and one (1%) for anti-HEV IgM. No other patient tested positive for ati-HEV IgM. In the subgroup of CIDP patients with initial diagnosis (without previous IVIG treatment), 30/54 (56%) tested positive for anti-HEV IgG. Anti-HEV rates were significantly lower in blood donors (28%), non-CIDP peripheral neuropathy patients (20%), and MG patients (12%). No subject tested positive for HEV viremia. CSF tested negative for in 61 CIDP patients (54 patients with primary diagnosis). The development of CIDP but not non-CIDP polyneuropathy may be triggered by HEV exposure in an HEV genotype 3 endemic region. The increased anti-HEV seroprevalence in CIDP patients is not a consequence of IVIG therapy.

Selenium deficiency is associated with polyneuropathy in primary Sjögren's syndrome.

BACKGROUND: Selenium plays an important role in the regulation of the immune system. Selenium deficiency is associated with Hashimoto's thyroiditis, a common comorbidity in primary Sjögren's syndrome (pSS). Therefore we aimed to identify whether or not selenium deficiency is also associated with pSS. METHODS: 107 consecutive female patients with pSS and, as a control, 59 female patients with axial spondyloarthritis were recruited. Later, 11 male pSS patients, 5 of these suffering from polyneuropathy, and 15 male axSpA patients were additionally recruited in order to confirm the results from the female patients. All patients were consulted about their diet and food intolerances and their plasma selenium concentrations were analyzed. Current and previous extraglandular manifestations of pSS were recorded. Patients complaining of misperceptions and tingling paraesthesia underwent measurement of nerve conduction velocity. The proportion of patients and controls with a selenium deficiency was compared using Fisher's exact test. RESULTS: The proportion of female pSS patients with a low selenium concentration <0.63 µmol/L (22.4%) was significantly higher than of the controls (1.7%) (p < 0.001). Within the group of female patients with pSS, selenium deficiency was significantly associated with the presence of polyneuropathy (45.8% with vs 14.5% without polyneuropathy, p = 0.003) and particularly polyneuropathy with motor nerve involvement measured by nerve conduction velocity (41.7% with vs 10.8% without motor neuropathy, p = 0.001). The mean selenium concentrations of the 5 male pSS patients with polyneuropathy were significantly lower compared to the 6 pSS patients without polyneuropathy and to the 15 male axSpA controls. CONCLUSIONS: PSS and in particular its complication polyneuropathy is associated with selenium deficiency. Measurement of the selenium concentration in blood is advisable in patients with pSS and in particular in the subset of patients with polyneuropathy. Substitution of selenium may be a possible therapy of polyneuropathy associated with pSS.

[Congenital aortic valve stenosis with very high pressure gradient in a pregnant patient]. / Kongenitale Aortenklappenstenose mit sehr hohem Druckgradienten bei einer schwangeren Patientin.

HISTORY AND CLINICAL FINDINGS: A 34-year-old woman was in her 24th week of her second pregnancy when she suffered a recurrence of her congenital aortic valve stenosis (CAS), previously treated by surgical commissurotomy. She was dyspneic on only mild exertion. The first pregnancy had been uneventful. INVESTIGATIONS: RR was 110/70 mmHg. The electrocardiogram showed left ventricular hypertrophy and myocardial damage. Echocardiography revealed a maximal systolic gradient of 155 mm Hg (mean gradient 104 mm Hg). The blood picture showed a pregnancy-related mild anemia and haemodilution. TREATMENT AND COURSE: The patient was hospitalized as a precaution from the 31st week of pregnancy onwards. An elective section was performed under intubation aneasthesia in the 36th week of pregnancy. The aortic valve was electively replaced 13 weeks after delivery. CONCLUSION: Patients with a CAS who had been treated palliatively with a commissurotomy should be carefully and regularly monitored to avoid recurrent stenosis. Close collaboration with the delivery team is essential.

Immunocytochemical localization of the storage protein phaseolin in the embryo-suspensor of Phaseolus coccineus.

Antibodies against the bean storage protein, phaseolin, were induced in rabbits. Sections of bean embryos of various age were incubated with the antibodies, and exposed to fluorescein isocyanate (FITC) conjugated goat anti-rabbit immunoglobulin. Distinct positive signals were found in vacuoles of the suspensor giant cells. Immunofluorescent staining of protein bodies in cotyledon cells, histochemical staining reactions of the sections, as well as gel electrophoretic analysis of the proteins, isoelectric focusing and Western blotting confirmed the specificity and reliability of the immunochemical staining of the suspensor cells. The results give evidence for synthesis of storage proteins in the embryo-suspensor and indicate a function so far not detected of this embryonal nutritive organ.