2.Seroprevalence and diagnostic value of aquaporin-4 antibody in patients with inflammatory central nervous system demyelinating diseases.
Lei WU ; Yang YANG ; De-Hui HUANG ; Wei-Ping WU
Journal of Southern Medical University 2011;31(2):350-352
OBJECTIVETo assess the seroprevalence and diagnostic value of aquaporin-4 antibody (AQP4-Ab) in patients with inflammatory central nervous system demyelinating diseases.
METHODSSeventy-two patients with neuromyelitis optica (NMO), 68 with multiple sclerosis (MS), 4 with optic neuritis (ON), and 41 with transverse myelitis (TM) were included in this study. The TM group comprised 19 patients with non-longitudinally extensive transverse myelitis (nLETM), 14 with monophasic longitudinally extensive transverse myelitis (mLETM), and 8 with recurrent longitudinally extensive transverse myelitis (rLETM). The serum levels of AQP4-Ab was detected by indirect immunofluorence assay in these patients.
RESULTSAQP4-Ab was detected in 72.2% (52/72) patients with NMO, 5.9% (4/68) patients with MS, 25.0% (1/4) patients with ON, and 17.1% (7/41) patients with TM, showing a significant difference in the positivity between NMO and MS groups (P<0.01). AQP4-Ab seropositivity rate was 5.3% (1/19) in nLETM patients, 62.5% (5/8) in rLETM patients and 7.1% (1/14) in mLETM patients, significantly higher in rLETM than in nLETM (P<0.01) and mLETM groups (P<0.05), but no statistical difference was found between rLETM and NMO groups.
CONCLUSIONSA high seroprevalence of AQP4-Ab is observed in patients with NMO and rLETM, which support the hypothesis that NMO and rLETM belong to NMO spectrum disorders. AQP4-Ab can serve as a useful index for diagnosing NMO and differential diagnosis from MS. More attention and effective immunosuppressive treatments should be given to patients positive for AQP4-Ab.
Aquaporin 4 ; immunology ; Autoantibodies ; blood ; Demyelinating Autoimmune Diseases, CNS ; diagnosis ; immunology ; Female ; Humans ; Male ; Multiple Sclerosis ; diagnosis ; immunology ; Neuromyelitis Optica ; diagnosis ; immunology ; Seroepidemiologic Studies
3.Idiopathic inflammatory demyelinating diseases of the central nervous system in patients following allogeneic hematopoietic stem cell transplantation: a retrospective analysis of incidence, risk factors and survival.
Xiao-Hui ZHANG ; Xiao-Jun HUANG ; Kai-Yan LIU ; Lan-Ping XU ; Dai-Hong LIU ; Huan CHEN ; Wei HAN ; Yu-Hong CHEN ; Feng-Rong WANG ; Jing-Zhi WANG ; Yu WANG ; Ting ZHAO ; Yao CHEN ; Hai-Xia FU ; Min WANG
Chinese Medical Journal 2013;126(6):1096-1102
BACKGROUNDAllogeneic hematopoietic stem cell transplantation (allo-HSCT) is a curative therapy for many hematological diseases, but there are many complications following allo-HSCT, among which neurological complications (NC) are one of the most commonly described ones. However, little is known about idiopathic inflammatory demyelinating diseases (IIDDs) of the central nervous system (CNS) in patients following allo-HSCT.
METHODSA nested case-control study was conducted in a large cohort of 1365 patients, who underwent allo-HSCT at the Institute of Hematology and Peking University People's Hospital, between January 2004 and December 2009, 36 patients of whom developed CNS IIDDs. Kaplan-Meier method, univariate and multivariate Cox regression were applied in our statistical analysis using SPSS 16.0.
RESULTSThe cumulative incidence of all cases of IIDDs at 6 years posttransplantation was 3.6%. Thirty-five patients (97.2%) suffered IIDDs after transplantation, 16 patients (44.4%) between day 0 to day 100 post-transplantation, 10 patients (27.8%) between day 100 to 1 year post-transplantation, and 9 patients (25.0%) 1 year post-transplantation. Multivariate regression analysis identified donor type (P = 0.031), infection (P = 0.009), and acute lymphatic leukemia (P = 0.017) as independent risk factors for posttransplantation IIDDs. The median survival time of patients with IIDDs was 514 days after transplantation (95%CI: 223 - 805). Survival at 6 years was significantly lower in patients who developed the diseases compared to those who did not (26.6% vs. 73.5%, P < 0.001). Of the 36 patients experiencing IIDDs, 58.3% (n = 21) died. The causes of death were graft-versus-host disease (GVHD) (n = 4), underlying disease relapse (n = 3), infections (n = 12), and other causes (n = 2).
CONCLUSIONSIIDDs is an uncommon but serious complication of allo-HSCT, especially in patients with a primary diagnosis of acute lymphatic leukemia, mismatched transplants, and infections. Our study results indicate that patients with IIDDs tend toward a poor prognosis following allo-HSCT.
Adolescent ; Adult ; Case-Control Studies ; Central Nervous System ; Child ; Child, Preschool ; Demyelinating Autoimmune Diseases, CNS ; etiology ; Female ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Infant ; Male ; Middle Aged ; Retrospective Studies ; Risk Factors ; Young Adult
4.Relationship between anti-myelin basic protein antibody and myelinoclasis in rat brain stem after brain trauma.
Wei LI ; Shan-Cheng CHEN ; Zhi-Gang WANG ; Xiu-Bao SONG ; Yu-Ping WANG ; Mei ZHANG
Journal of Southern Medical University 2008;28(6):1028-1030
OBJECTIVETo investigate the relations between anti-myelin basic protein antibody (anti-MBP) variation and myelinoclasis in the brain stem following brain trauma.
METHODSIn rat models of brain trauma, MBP content and anti-MBP titer in the blood were measured using enzyme-linked immunosorbent assay (ELISA) at different time points after brain trauma, and the degree of myelinoclasis in the brain stem slices was assessed with osmic acid staining.
RESULTSEarly after brain trauma, MBP content in the blood increased followed by significant reduction 10 days later. Four days after the trauma, anti-MBP titer was markedly increased, accompanied by obvious exacerbation of myelinoclasis in the brain stem, both reaching the highest levels on day 10, at the point of which anti-MBP titer increased by 4 folds and the number of myelinoclasis by 10 folds compared with the control group. Anti-MBP titer and brain stem myelinolysis both lowered 30 days later. Correlation analysis showed an intimate positive correlation between anti-MBP titer and the degree of myelinoclasis.
CONCLUSIONAfter brain trauma, MBP is released as a specific antigen into the blood to stimulate the immune system for anti-MBP production, and the antibody is intimately related to the brain stem myelinoclasis.
Animals ; Antibodies ; metabolism ; Brain Injuries ; complications ; Brain Stem ; immunology ; pathology ; Demyelinating Autoimmune Diseases, CNS ; etiology ; immunology ; Female ; Male ; Myelin Basic Protein ; Nerve Tissue Proteins ; blood ; immunology ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Transcription Factors ; blood ; immunology
5.Poliodystrophia Cerebri Progressiva(Diffuse Degeneration of Grey Matter).
Yonsei Medical Journal 1960;1(1):45-51
No abstract available.
Diffuse Cerebral Sclerosis of Schilder*
6.A Pathology-proven Case of Schilder's Disease.
Byung Su KIM ; Byoung Joon KIM ; Kwang Ho LEE ; Gyeong Moon KIM
Journal of the Korean Neurological Association 2008;26(1):50-54
Schilder's disease or myelinoclastic diffuse sclerosis (MDS) is a rare variant of multiple sclerosis. We report a 66-year-old woman with progressive motor weakness and diffuse white matter degeneration on MRI, which satisfies the Poser's restrictive criteria of MDS. As previously reported cases of probable Schilder's disease did not meet the criteria correctly, we consider our patient is the first pathology-proven case of MDS in Korea.
Aged
;
Demyelinating Diseases
;
Diffuse Cerebral Sclerosis of Schilder
;
Female
;
Humans
;
Multiple Sclerosis
8.Balo's Concentric Sclerosis in a Patient with Previous Recurrent Optic Neuritis.
Jae Hwan KIM ; Jae Hee YOON ; Kyu Sun YUM ; Bora YOON ; Kee Ook LEE ; Yong Duk KIM ; Sang Jun NA
Journal of the Korean Neurological Association 2015;33(4):310-314
Balo's concentric sclerosis is regarded as a rare variant of multiple sclerosis. Traditionally, Balo's concentric sclerosis was a post-mortem diagnosis, but the recent introduction of brain magnetic resonance imaging (MRI) scans may allow noninvasive access without biopsy. Brain MRI findings of Balo's concentric sclerosis is characteristic concentric configuration of alternating bands of white matter of different pathology, with relatively preserved myelination alternating with regions of demyelination in the cerebral white matter. We report a case of Balo's concentric sclerosis with recurrent optic neuritis.
Biopsy
;
Brain
;
Demyelinating Diseases
;
Diagnosis
;
Diffuse Cerebral Sclerosis of Schilder*
;
Humans
;
Magnetic Resonance Imaging
;
Multiple Sclerosis
;
Myelin Sheath
;
Optic Neuritis*
;
Pathology
10.Alpers-Huttenlocher Syndrome First Presented with Hepatic Failure: Can Liver Transplantation Be Considered as Treatment Option?.
Sowon PARK ; Hoon Chul KANG ; Jin Sung LEE ; Young Nyun PARK ; Seung KIM ; Hong KOH
Pediatric Gastroenterology, Hepatology & Nutrition 2017;20(4):259-262
Mitochondria play essential role in eukaryotic cells including in the oxidative phosphorylation and generation of adenosine triphosphate via the electron-transport chain. Therefore, defects in mitochondrial DNA (mtDNA) can result in mitochondrial dysfunction which leads to various mitochondrial disorders that may present with various neurologic and non-neurologic manifestations. Mutations in the nuclear gene polymerase gamma (POLG) are associated with mtDNA depletions, and Alpers-Huttenlocher syndrome is one of the most severe manifestations of POLG mutation characterized by the clinical triad of intractable seizures, psychomotor regression, and liver failure. The hepatic manifestation usually occurs late in the disease's course, but in some references, hepatitis was reportedly the first manifestation. Liver transplantation was considered contraindicated in Alpers-Huttenlocher syndrome due to its poor prognosis. We acknowledged a patient with the first manifestation of the disease being hepatic failure who eventually underwent liver transplantation, and whose neurological outcome improved after cocktail therapy.
Adenosine Triphosphate
;
Diffuse Cerebral Sclerosis of Schilder*
;
DNA, Mitochondrial
;
Eukaryotic Cells
;
Hepatitis
;
Humans
;
Liver Failure*
;
Liver Transplantation*
;
Liver*
;
Mitochondria
;
Mitochondrial Diseases
;
Oxidative Phosphorylation
;
Prognosis
;
Seizures