ABSTRACT: Anti-N-methyl-D-aspartate receptor encephalitis is an increasingly common autoimmune disorder mediated by antibodies to certain subunit of the N-methyl-D-aspartate receptor. Recent literatures have described anti-thyroid and infectious serology in this encephalitis but without follow-up.
A 17-year-old Chinese female patient presented with psychiatric symptoms, memory deficits, behavioral problems and seizures. She then progressed through unresponsiveness, dyskinesias, autonomic instability and central hypoventilation during treatment. Her conventional blood work on admission showed high titers of IgG antibodies to thyroglobulin, thyroid peroxidase and IgM antibodies to Epstein-Barr virus viral capsid antigen. An immature ovarian teratoma was found and removal of the tumor resulted in a full recovery. The final diagnosis of anti-N-methyl-D-aspartate receptor encephalitis was made by the identification of anti-N-methyl-D-aspartate receptor antibodies in her cerebral spinal fluid. Pathology studies of the teratoma revealed N-methyl-D-aspartate receptor subunit 1 positive ectopic immature nervous tissue and Epstein-Barr virus latent infection. She was discharged with symptoms free, but titers of anti-thyroid peroxidase and anti-thyroglobulin antibodies remained elevated. One year after discharge, her serum remained positive for anti-thyroid peroxidase and anti-N-methyl-D-aspartate receptor antibodies, but negative for anti-thyroglobulin antibodies and IgM against Epstein-Barr virus viral capsid antigen.
Persistent high titers of anti-thyroid peroxidase antibodies from admission to discharge and until one year later in this patient may suggest a propensity to autoimmunity in anti- N-methyl-D-aspartate receptor encephalitis and support the idea that neuronal and thyroid autoimmunities represent a pathogenic spectrum. Enduring anti-N-methyl-D-aspartate receptor antibodies from admission to one year follow-up but seroreversion of Epstein-Barr virus viral capsid antigen IgM may raise the important issue of elucidating the triggers and boosters of anti- N-methyl-D-aspartate receptor encephalitis.
BMC Neurology 11/2011; 11:149. · 2.17 Impact Factor
ABSTRACT: To study the expression of tau-related protein in spinal cord of Chinese patients with Alzheimer's disease.
Gallays-Braak stain and immunohistochemical study for tau protein (AT8) were carried out in the spinal cord tissue (T2, T8, T10, L2 and S2 segments) of 3 Chinese patients with Alzheimer's disease. Seven age-matched cases without evidence of dementia or neurologic disease were used as controls.
Neurofibrillary tangles were identified in the neurons of anterior horn in 2 Alzheimer's disease cases but none was observed in the controls. Tau-positive axons and astroglia were detected in all Alzheimer's disease cases. Tau immunoreactivity in spinal cord of the patients correlated with that in brain tissue.
The expression of tau-related protein is demonstrated in the spinal cord of Alzheimer's disease patients suggesting that axonal transport defect may play a role in the pathogenesis of Alzheimer's disease.
Zhonghua bing li xue za zhi Chinese journal of pathology 03/2011; 40(3):161-4.
ABSTRACT: We evaluated the features of neuropathology, abnormal prion protein (PrP) molecules, and clinical data of a Chinese woman diagnosed with familiar Creutzfeldt-Jakob disease (CJD), having 7 octa-repeats inserted with codon 129 methionine homozygote in the PRNP gene.
Neuropathologic characteristics of the brain were analyzed by hemotoxylin-eosin stain and electronic microscopy. The presence of abnormal PrP in brains was detected by proteinase K and PrP molecules were evaluated by deglycosylation assay.
Spongiform degeneration, with diffuse neuronal loss and mild astrocytic gliosis, as well as with profound degeneration of neurons and astrocytes was observed. Proteinase K-resistant PrP was deposited widely in various regions of the brain. Calculation of the glycosylation ratios of proteinase K-resistant PrP molecules identified that the monoglycosyl isomer was predominant. PrP deglycosylation tests allowed for the identification of a predominant 19-kDa PrP signal that represents a partially proteolytic C-terminal segment, a 27-kDa band that represents the full-length wild-type PrP molecule, and a 30-kDa band that probably corresponds to the full-length mutant PrP molecule.
: Sporadic CJD-like neuropathologic changes and deposits of proteinase K-resistant PrP have been identified in this familiar CJD case with a 168 base pair nucleotide insertion. The clinical features differ from previously reported cases that had 7 octa-repeat insertion, but bear similarities to sporadic CJD.
The American Journal of the Medical Sciences 01/2009; 336(6):519-23. · 1.39 Impact Factor
ABSTRACT: To construct a fusion protein toxin DT389-hIL-13 which comprises the N-terminal 389 amino acids of diphtheria toxin (DT389) and human interlukin 13 (hIL-13), and to explore its cytotoxicity on U251 glioma cells.
The cDNA of hIL-13 gene was amplified by PCR and linked with the 3'-terminus of the gene encoding the N-terminal 389 amino acids, which correspond to the enzyme domain and transmembrane domain of diphtheria toxin. The tandem constructed gene was then inserted into an E. coli expression vector pET30a. The resulted expression vector was transformed into E. coli BL21 and induced by IPTG. The expressed protein was analyzed by SDS-PAGE and Western blot analysis. U(251) glioma cells were cultured DT389-hIl-13 was added into the culture. The cytotoxicity was determined using colorimetric MTS proliferation assay.
The expression plasmid pET30a/DT389-hIL13 was constructed with correct sequence. The recombinant protein was successfully expressed in E. coli in manner of inclusion body and with a relative molecular weight of about 55 000, which reacted well with both anti-diphtheria toxin and anti-hIL-13 polyclonal antibody in Western blot assay. The purified recombinant chimeric toxin was found to effectively inhibit the prolifieration of glioblastoma multiforme cells bearing high affinity hIL-13 receptors, and resulted in dose-dependent relationship with 50% inhibition concentration (IC(50)) of 5 x 10(-)11mol/L.
Prokaryotic expression system can be recruited to produce recombinant chimeric toxin DT389-hIL-13. The results may lay a foundation for preparing specific the agent targets for tumors overexpressing IL-13 receptor.
Zhonghua yi xue za zhi 07/2004; 84(12):1024-8.