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

Neuroimmunology is a rapidly expanding field in the medicine and there has been great advancement in the understanding of the pathogenesis of autoimmune diseases during the past decade. In neurology, immune mechanism has been implicated in the pathogenesis of more than twenty neurological diseases. The understanding of the immunological mechanism of these diseases has helped clinicians in management of these diseases. In many neurological diseases with immunological deterioration, multiple sclerosis and Guillain-Barre syndrome are relatively common diseases that share common features like demyelination. But multiple sclerosis is a disease of central nervous system and Guillain-Barre syndrome is a syndrome developed in peripheral nervous system. This review will describe the recent advances in clinical immunology with regard to general aspects of autoimmune diseases of neurological system, pathogenesis of two common autoimmune diseases, multiple sclerosis and Guillain-Barre syndrome.
Allergy and Immunology ; Autoimmune Diseases ; Central Nervous System ; Demyelinating Diseases ; Guillain-Barre Syndrome* ; Multiple Sclerosis* ; Neurology ; Peripheral Nervous System

OBJECTIVEMultiple sclerosis is a demyelinating disease frequently showing a relapsing-remitting disease course. Clinical manifestations of 25 inpatients with MS were summarized and analyzed so that the clinical features and therapeutic approaches to childhood multiple sclerosis (MS) were investigated in order to improve its diagnosis and management.

METHODSClinical features and information during following-up of 25 cases with MS from June 1993 to May 2006 were collected and analyzed.

RESULTSAmong the 25 cases, 16 were female and the F:M ratio was 1.78:1. The relapsing-remitting type was seen in 21 cases, the secondary progressive MS in 3 cases and the classification was impossible in one case. The mean age of onset was 6.7 years (2-12) with various initial symptoms including visual loss (11 cases), cortical symptoms (8 cases with seizures, consciousness disturbance, aphasia and apraxia, etc.), myeleterosis (3 cases), symptoms of brainstem (2 cases) and cerebellar ataxia (1 case). Fever was present in 10 cases at the onset. Nine cases were monosymptomatic, while the other 16 had multiple symptoms. Visual loss occurred in 19 cases during the course of MS and 22 were found to have abnormal visual evoked potential (88%). The mean course of disease was 8.5 years (1.2-17.2) and 0-4 times of recurrences (0 means no new clinical attack occurred during following-up period).

CONCLUSIONSMS is increasingly recognized as a disease affecting children though it is uncommon. Childhood MS possesses some manifestations different from those of adults. There was a female predominance. The most common finding at the onset of disease was optic neuritis. Other features include acute onset and shorter course of disease. Atypical demyelinating symptoms were often seen. White matter lesions on MRI are required for the diagnosis. CSF oligoclonal bands could be found less commonly than in adults. Neurological sequelae were less often seen than in adults MS even though optic nerve atrophy and visual loss were relatively common. Steroid and IVIG are effective in acute period treatment.

Age of Onset ; Child ; Child, Preschool ; Demyelinating Diseases ; etiology ; Disease Progression ; Female ; Humans ; Immunoglobulins, Intravenous ; immunology ; Male ; Multiple Sclerosis ; immunology ; physiopathology ; therapy ; Optic Neuritis ; etiology ; immunology ; Secondary Prevention

No abstract available.
Animal ; Central Nervous System/virology ; Central Nervous System/pathology ; Central Nervous System/immunology ; Cytokines/metabolism ; Cytokines/immunology ; Demyelinating Diseases/virology* ; Demyelinating Diseases/immunology ; Disease Models, Animal ; Human ; Inflammation/virology ; Multiple Sclerosis/virology ; Multiple Sclerosis/immunology ; T-Lymphocytes/immunology ; Theiler Murine Encephalomyelitis Virus/pathogenicity ; Theiler Murine Encephalomyelitis Virus/immunology*

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