An unusual clinical presentation of bilateral schizencephaly

Summary We present a case with a characteristic magnetic resonance image (MRI) of bilateral open-lipped schizencephaly and atypical clinical presentation. The patient is still alive and in good health in her forties, she has never presented seizures, and although the motor dysfunction is well correlated with cerebral lobe involvement, neurobehavioral dysfunction is not proportional to the MR image of the cerebral malformation.

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Un cas inhabituel de schizencéphalie bilatérale

Résumé Nous présentons un cas de schizencéphalie bilatérale ouverte caractérisé par une présentation clinique atypique et une imagerie par résonance magnétique nucléaire caractéristique. La patiente est encore vivante, en bonne santé, à plus de 40 ans, elle n'a jamais présenté de crise comitiale et, bien que les troubles moteurs soient bien corrélés aux altérations cérébrales, les troubles neuro-comportementaux ne sont pas proportionnels aux images IRM de cette malformation cérébrale.

     

Rochalimaea henselae sp. nov., a cause of septicemia, bacillary angiomatosis, and parenchymal bacillary peliosis.

Nine strains of Rochalimaea spp. that were isolated from patients over a period of 4.5 years were characterized for their enzyme activities, cellular fatty acid compositions, and DNA interrelatedness among Rochalimaea spp., Bartonella bacilliformis, and Afipia felis (cat scratch disease bacillus). All except one isolate, which was Rochalimaea quintana, were determined to belong to a newly proposed species, Rochalimaea henselae sp. nov. After recovery from clinical material, colonies required 5 to 15 days of incubation to become apparent. Cells were small, gram-negative, curved bacilli and displayed twitching motility. Enzyme specificities for amino acid and carbohydrate substrates showed that R. henselae could be distinguished from Rochalimaea vinsonii by L-arginyl-L-arginine and L-lysyl-L-alanine peptidases, but not all strains could be distinguished from R. quintana on the basis of peptidases or carbohydrate utilization. R. henselae also closely resembled R. quintana in cellular fatty acid composition, with both consisting mainly of C18:1, C18:0, and C16:0 fatty acids. However, the strains of R. henselae all contained C18:0 in amounts averaging greater than or equal to 22%, in contrast to R. quintana, which contained this cellular fatty acid in amounts averaging 16 and 18%. DNA hybridization confirmed the identification of one clinical isolate as R. quintana and showed a close interrelatedness (92 to 100%) among the other strains. Under optimal conditions for DNA reassociation, R. henselae showed approximately 70% relatedness to R. quintana and approximately 60% relatedness to R. vinsonii. Relatedness with DNA from B. baciliformis was 43%. R. henselae was unrelated to A. felis. R. henselae is the proposed species of a newly recognized member of the family Rickettsiaceae, which is a pathogen that may be encountered in immunocompromised or immunocompetent patients. Prolonged fever with bacteremia or vascular proliferative lesions are clinical manifestations of the agent.     

Genetic diversity in the Helicobacter pylori cag pathogenicity island and effect on expression of anti-CagA serum antibody in UK patients with dyspepsia

AIMS: To investigate variation within the cag pathogenicity island (PAI) of Helicobacter pylori isolated from patients with dyspepsia in mid-Essex, and to evaluate the effect on expression of anti-CagA antibody. METHODS: Sixty two isolates of H pylori cultured from gastric biopsies were screened by specific PCR assays for the presence of cagA and other gene markers (cagD and cagE, and virD4) in the cag PAI. An enzyme linked immunosorbent assay (ELISA) kit (Viva Diagnostica helicobacter p120) was used to test for anti-CagA IgG antibody in matching sera. Isolates were also genotyped by vacuolating cytotoxin polymerase chain reaction (PCR) analysis, and tested for absence of the complete cag PAI (empty site PCR assay). RESULTS: Forty one of the H pylori isolates had a cag PAI containing cagA. One strain had no cagA but other cag PAI loci were present, whereas the remaining 20 strains had no detectable cag PAI markers. Anti-CagA IgG antibody was detected in 34 sera by the ELISA assay, and when compared with the cag PAI genotype of the infecting strain, accuracy, sensitivity, and specificity were 92%, 87%, and 100%, respectively. The seven discrepant or borderline strains in the ELISA were all vacA s1 but differed in other genotypic markers. CONCLUSIONS: The cag PAI was widely distributed in H pylori from patients with dyspepsia in mid-Essex who had different gastric pathologies. Infection with a strain having an uninterrupted cag PAI was associated with the presence of anti-CagA antibody in most patients. Discrepant ELISA results, mostly for elderly patients with duodenal ulcers, were attributed to cagA associated variation, particularly to the presence of mixed cagA+/cagA- cell variants in the infecting strain population. Tests for anti-CagA serum antibody were unreliable for predicting severity of clinical disease associated with H pylori infection in this series of patients.