Paraneoplastic Cerebellar Degeneration and Lambert-Eaton Myasthenic Syndrome with SOX-1 Antibodies

A 69-year-old man was admitted to our hospital for progressive muscle weakness in both lower limbs and limb ataxia (day 0). Nerve conduction studies showed low compound muscle action potential amplitudes at rest and increased amplitudes after maximum voluntary contraction. Blood testing revealed SOX-1 antibodies. He was diagnosed with paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome (PCD-LEMS). He died from aspiration pneumonia on day 9. Small-cell lung carcinoma (SCLC), which had not been obvious on computed tomography, was found during the autopsy. Patients with PCD-LEMS who test positive for SOX-1 antibodies should be carefully evaluated for SCLC.     

A case of systemic lupus erythematosus that manifested in the course of schizophrenia

A case of schizophrenia is presented in which SLE was diagnosed after 14-year duration. Antibodies to single and double-stranded DNAs, but not to histone. were detected. This case suggests that similar immunological abnormalities as SLE are associated with the pathogenesis of a group of schizophrenia and that class-switch of anti-dsDNA antibodies are important in the pathogenesis of SLE.     

Trends in age and anthropometric data at start of growth hormone treatment for girls with Turner syndrome in Japan

The purpose of this study is to evaluate the trends in age and anthropometric data for girls with Turner syndrome (TS) at start of growth hormone (GH) treatment in Japan. The data for analysis were obtained from a retrospective cohort, the Foundation for Growth Science, Japan. We analyzed trends in starting age of GH treatment for girls with TS in Japan after dividing subjects (n=1,478) into three registration periods: 1991-1994, 1995-1999 and 2000-2004. We also assessed the ratio of the subpopulation of subjects under five years of age. As results, the mean age (standard deviation (SD)) at start of GH treatment was significantly different among the three groups (10.95 (3.63), 10.15 (3.39) and 8.78 (3.61), p<0.0001). The proportion of the subjects under five years of age increased significantly over time (5.11%, 7.11% and 16.85%, p<0.0001). Mean (SD) height SD scores were also significantly different (-3.41 (0.87), -3.26 (0.81) and -3.17 (0.79), p<0.0001). However, the proportions of the karyotype of 45,X were not significantly different among the three groups (p=0.25). We concluded that age and shortness at initiation of GH treatment had been improving over time. However, these favorable trends have not fully met the conditions recommended by international clinical guidelines for TS.     

Incidence of human parvovirus B19 DNA detection in blood donors

Summary. 1000 serum samples from blood donors were tested for human parvovirus B19 (B19) DNA by a nested PCR assay: six samples were positive for B19 DNA. The frequency was 1/167 (0-6%), considerably higher than previous surveys (0-004-0-03%). Five of the six samples were also positive for anti-B19 IgM, indicating an acute phase of infection. It is recommended to screen for B19 DNA in blood products to prevent transfusion mediated viral infection for those susceptible such as immunocompromised patients and pregnant women.     

DNA strand breaks and death of thymocytes induced byN-methyl-N-nitrosourea

Summary N-Methyl-N-nitrosourea (MNU) is a potent carcinogen in various sites of experimental animals and induces thymic lymphoma in rats, which has long been hard to induce by any carcinogen. To analyze the action of MNU on thymocytes, DNA strand breaking in thymocytes from the MNU-treated rat and that in MNU-treated cultured thymocytes were assayed. Fluorometric analysis of DNA unwinding (FADU assay), first reported by Birnboim and Jevcak to detect X-ray-induced DNA damage, was modified and applied to detect DNA damage in thymocytes treated with MNU in vitro or in vivo. In the present modified method, cell lysate was admixed with 0.15M sodium hydroxide, and DNA unwinding was processed at pH 12.0 for up to 2 h at 0° C in iced water. Double-stranded DNA remaining after alkaline reaction was detected by binding ethidium bromide and measuring its fluorescence. The severity of DNA damage, both in vivo and in vitro, depended on the MNU concentration. In addition, the sequential survival rate and cell-size distribution of thymocytes treated with MNU in vitro were investigated. A close relationship between the severity of DNA damage and cell death was demonstrated in MNU-treated thymocytes, and DNA damage by a non-cell-killing dose of MNU was detected with this FADU assay. MNU-induced cell death is not programmed as in apoptosis, which is caused in thymocytes physiologically, immunologically and by X-ray irradiation or corticoids.Abbreviations used MNU N-methyl-N-nitrosourea - FADU fluorometric analysis of DNA unwinding - PBS calcium- and magnesium-free phosphate-buffered saline     

Effect of Gamma Knife Radiosurgery on a Pituitary Gonadotroph Adenoma: A Histologic,Immunohistochemical and Electron Microscopic Study

The morphologic findings in a pituitary macroadenoma removed from a 65-year old man by the transsphenoidal approach 9 months after gamma knife surgery are reported. The tumor was immunoreactive for FSH and showed ultrastractural features consistent with an oncocytic gonadotroph adenoma. Accumulation of connective tissue separating small groups of adenoma cells was evident. Several dilated vessels and numerous vascular endothelial growth factor immunopositive adenoma cell were noted. By electron microscopy the endothelial linings frequently showed discontinuities with platelet accumulation attached to the gaps. Several vessels were severely injured showing necrosis of endothelial cells. It can be concluded that gamma knife surgery caused severe alterations in pituitary adenoma microcirculation indicating that vascular injury plays a crucial role in tumor shrinkage.     

Assessment of inflamed synovial membrane in the knee joint by dynamic magnetic resonance imaging

Dynamic magnetic resonance imaging (dynamic MRI) was used to examine the synovial membrane in the knee joints of 15 patients with rheumatoid arthritis (RA) in order to investigate the relationship between pathological and MRI findings. Signal intensities in the regions of interest (ROI), identified as the synovial membrane of the suprapatellar pouch, were measured on MR images. Signal intensities at various times after the injection of contrast medium Gd–diethylenetriaminopentoacetic acid (Gd–DTPA) were normalized relative to the signal intensity at 80s, and designated as the normalized signal intensity (NSI). Pathological findings were quantified, and the types of inflamed synovial membrane were classified as either acute or chronic. A significant difference in NSI was observed between acute and chronic types (P 0.05). Dynamic MRI was capable of classifying acute and chronic RA by measuring NSI 20s after contrast medium injection. Dynamic MRI was therefore shown to be useful for assessing regional synovial inflammation.