Detection Of Serological Nmo-Igg And Clinical Analysis In Patients With Disorders Of The Spectrum Of Neuromyelytis Opica

Background:Neuromyelitis optica(NMO) is a severe demyelinating disease in the central nervous system with acute onset, it is characterized by involving the optic nerve and spinal cord preferably. The recurrent rate is greater than the MS and there may be no obvious remission between two relapses, the rapid progress could lead to rapid and unrecoverable disability. The disease was first reported by Eugene and his disciple Fernand Gault, since then on there is always the debate about whether NMO is a new entity or a severe variant of Multiple Sclerosis. The discovery of NMO-IgG which can target to the aquaporin4,the most abundant aquaporin in the brain, together with its clinical manifestation, Magnetic Resonance and pathology suggest that NMO is different from Multiple Sclerosis and support the theory that humoral immune reaction perform a critical role in NMO. And the discovery of NMO-IgG makes it possible that a group of diseases, such as longitudinally extensive transverse myelitis and recurrent optic neuritis where the NMO-IgG can also be detected, are classified as neuromyelitis optica spectrum disorder. Compared to Multiple Sclerosis, the Asian are more susceptible to NMO and the treatment and prognosis are quite different from MS. The serological positivity of NMO-IgG is of great importance for NMO and the longitudinally extensive transverse myelitis and recurrent optic neuritis may transform into NMO, so the detection of serological NMO-IgG is critical in the diagnosis and treatment of NMOSD.Purpose:To summarize the clinical features of17cases of NMOSD, detect the serological NMO-IgG status and analysis the diagnostic significance.Methods:The clinical data and sera of17NMOSD patients,1multiple sclerosis and8other neurological diseases came from Qilu hospital and affiliated hospital of Weifang Medical College between March2011June and2011December were collected, the serological test, cerebral spinal fluid test, imaging examination, evoked potential and the serological NMO-IgG detection were performed.Results:1. NMO has strong female predilection, the male to female gender ratio is1:4; the median age onset is53years old; The main clinical manifestations include vision loss, movement disorders, sensory disturbances and sphincter dysfunction. Rheumatoid series of5cases were normal;2cases underwent tumor series test and elevated serum sialic acid was found in1case; thyroid function and antibody detection of2cases were normal;2cerebrospinal fluid laboratory tests showed cells account increased slightly (10-50/ml), total protein increased slightly (0.42-0.44g/l), Immunoglobulin G increased slightly (42.7-49.8mg/l); MRI revealed the length of spinal cord involvement equaled to4-10spinal segments, the involvement of spinal cord included medulla oblongata in1case, cervical cord and thoracic cord in the other4cases; the lesions were diffuse in2cases and linear combind with diffuse distribution in3cases in sagittal view and mostly distributed centripetally in axial view, abnormal signals in the brain could also be found, abnormal siganals could be found in the white matter around the body of lateral cerebral ventricle in3cases, in area of basal gangalia in1case and in the white matter of cerebral hemisphere in1case; EMG evoked potential showed conduction block of visual pathway in cases with significant vision loss; Seropositivity rate of NMO-IgG was100%.2. There are3males and8females of the11LETM patients, the male to female gender ratio is1:2.7; the median age onset is54years old; the main clinical manifestations include movement disorders, sensory disturbances and sphincter dysfunction.2of the6rheumatoid series tests were abnormal,1rheumatoid factor and1anti-SSA antibody positive;5out of the7cases underwent tumor series tests were abnormal;1ANA abnormal and2ANCA were normal; Cerebrospinal fluid laboratory tests of7cases showed cells account was normal or increased slightly (42-48/ml), total protein and immunoglobulin G were normal or increased slightly; MRI revealed the length of spinal cord involvement spaned from3vertebral segments to the whole spinal cord,2cases with the involvement of cervical cord,3cases with the involvement of cervical and thoracic cord,3cases with the involvement of thoracic cord,2cases with the involvement of thoracic and lumbar cord, and1with he involvement of the whole spinal cord; the lesions were diffuse in4cases and linear combind with diffuse in7cases in sagittal view and mostely distributed centripetally in axial view;2of the4cases were patchy enhanced after enhancement; abnormal signals in the brain could also be found in5out of the6patients who underwent the examination of brain MRI,3cases with the abnormal siganals in both sides of the cerebral hemosphere,2cases with the abnormal siganals in cornu posterius ventriculi lateralis, and1was normal;3EMG evoked potential were all normal; Seropositivity rate of NMO-IgG was45.5%.3. There was1recurrent ON patient out of the17NMOSD patients, he was55years old and manifested as vision loss in both eye with an interval of3months; The detection of serological NMO-IgG was negative.Conclusions:1. The disease has strong female predilection, the male to female gender ratio of NMO and LETM is1:4and1:2.7respectively; the median age onset is53and54years old respectively and is higher than that of MS.2. Besides NMO-IgG, some other auto-antibodies can also be detected in some NMOSD patients, with low specificity however, these auto-antibodies cannot lead to the diagnosis of NMOSD. The data of cerebrospinal fluid were normal or increased slightly, pleocytosis cannot be a support diagnostic criteria for NMO.3. The MRI examination is characteristic for diagnosis of NMOSD, long segmental spinal lesions could be found in both NMO and LETM, and intracranial abnormal signals were common in NMOSD patients. Early examination of evoked potential is suggested and can devote to the diagnosis and treatment of NMOSD.4. The seropositive rate of the17NMOSD was58.8%, the seropositive rate of NMO and LETM were100%and45.5%respectively, which is higher than the other neurological diseases,and both the specificity is100%; the relapse rate of the seropositive LETM patients was higher than that of seronegative; the1rON patient was seronegative. NMO-IgG is prevalence in NMO and LETM with high specificity; early detection of serological NMO-IgG can facilitate the diagnosis and instruct the treatment, and series detection of NMO-IgG is of great significance in predicting the clinical relapse.