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1.  Clinical Efficacy of Plasmapheresis in Patients with Neuromyelitis Optica Spectrum Disorder and Effects on Circulating Anti-Aquaporin-4 Antibody Levels 
Background and Purpose
Although plasmapheresis is becoming standard practice as a rescue therapy for neuromyelitis optica (NMO), evidence for the therapeutic efficacy of plasmapheresis is limited, and the effect of plasmapheresis on anti-aquaporin-4 (AQP4) levels in patients with NMO has not been reported. Here, our objective was to evaluate the clinical efficacy of therapeutic plasmapheresis and its effect on anti-AQP4 antibody levels in patients with NMO spectrum disorder (NMOSD).
Methods
We retrospectively reviewed the medical records of 15 patients with NMOSD who had 18 acute attacks and received plasmapheresis because they did not respond to high-dose intravenous methylprednisolone (IVMP) therapy. Anti-AQP4 antibodies were measured before and after plasmapheresis. The primary outcomes were functional improvements immediately and 6 months after plasmapheresis, and the secondary outcome was the change in anti-AQP4 antibody serum levels following plasmapheresis.
Results
Plasmapheresis following IVMP therapy led to significant improvement in 50% of the 18 attacks in 15 patients immediately after the procedure was completed, and in 78% (14 attacks) after 6 months. Plasmapheresis was generally well tolerated in all patients. Anti-AQP4 antibody serum levels declined significantly following plasmapheresis, to a mean of 15% of the preplasmapheresis levels. Lower scores on the visual outcome scale recorded before an attack were associated with significant immediate improvement upon the completion of plasmapheresis (p=0.03).
Conclusions
Plasmapheresis following IVMP therapy effectively removed anti-AQP4 antibodies and was accompanied by a substantial improvement in the neurological disability of patients with NMOSD. Lower levels of pre-existing neurological damage may be associated with an improved acute response to plasmapheresis.
doi:10.3988/jcn.2013.9.1.36
PMCID: PMC3543908  PMID: 23346159
plasmapheresis; neuromyelitis optica; anti-aquaporin-4 antibody
2.  New Insights into Neuromyelitis Optica 
Neuromyelitis optica (NMO) is an idiopathic inflammatory disorder of the central nervous system (CNS) that preferentially affects the optic nerves and spinal cord. In Asia, NMO has long been considered a subtype of multiple sclerosis (MS). However, recent clinical, pathological, immunological, and imaging studies have suggested that NMO is distinct from MS. This reconsideration of NMO was initially prompted by the discovery of a specific antibody for NMO (NMO-IgG) in 2004. NMO-IgG is an autoantibody that targets aquaporin-4 (AQP4), the most abundant water channel in the CNS; hence, it was named anti-AQP4 antibody. Since it demonstrated reasonable sensitivity and high specificity, anti-AQP4 antibody was incorporated into new diagnostic criteria for NMO.The spectrum of NMO is now known to be wider than was previously recognized and includes a proportion of patients with recurrent, isolated, longitudinally extensive myelitis or optic neuritis, and longitudinally extensive myelitis or optic neuritis associated with systemic autoimmune disease or with brain lesions typical of NMO. In this context, a new concept of "NMO spectrum disorders" was recently introduced. Furthermore, seropositivity for NMO-IgG predicts future relapses and is recognized as a prognostic marker for NMO spectrum disorders. Humoral immune mechanisms, including the activation of B-cells and the complement pathway, are considered to play important roles in NMO pathogenesis. This notion is supported by recent studies showing the potential pathogenic role of NMO-IgG as an initiator of NMO lesions. However, a demonstration of the involvement of NMO-IgG by the development of active immunization and passive transfer in animal models is still needed. This review focuses on the new concepts of NMO based on its pathophysiology and clinical characteristics. Potential management strategies for NMO in light of its pathomechanism are also discussed.
doi:10.3988/jcn.2011.7.3.115
PMCID: PMC3212597  PMID: 22087205
neuromyelitis optica; Devic's disease; neuromyelitis optica spectrum disorder; pathogenesis; diagnosis; management
3.  Secondary Amyloidosis Associated with Multiple Sclerosis 
Background
Multiple sclerosis (MS) is a demyelinating disease of the central nervous system. Secondary amyloidosis can occur as a complication of chronic systemic inflammatory and infectious diseases. Until now there has been no report of secondary amyloidosis associated with MS. We report herein a case of renal biopsy-proven secondary amyloidosis in a patient with MS.
Case Report
A 41-year-old woman with MS was hospitalized due to aggravated quadriparesis and edema in both lower extremities. Laboratory findings showed nephrotic-range proteinuria and hypoalbuminemia. A percutaneous renal biopsy procedure was performed, the results of which revealed secondary amyloid-A-type amyloidosis associated with MS.
Conclusions
This is the first report of secondary amyloidosis associated with MS.
doi:10.3988/jcn.2009.5.3.146
PMCID: PMC2760720  PMID: 19826566
multiple sclerosis; secondary amyloidosis; nephrotic syndrome
4.  Progressive Multifocal Leukoencephalopathy in AIDS: Proton MR Spectroscopy Patterns of Asynchronous Lesions Confirmed by Serial Diffusion-Weighted Imaging and Apparent Diffusion Coefficient Mapping 
Progressive multifocal leukoencephalopathy (PML) is a rare disease that occurs mainly in immunocompromised patients. Despite the progressive nature of the disease, the changes on MRI during the disease course - which may help in monitoring the disease process - have seldom been reported. Here we describe a patient with polymerase-chain-reaction-proven PML examined using serial diffusion-weighted imaging (DWI) and apparent-diffusion-coefficient mapping. Magnetic resonance spectroscopy (MRS) revealed that the demyelinating process was more active without significant neuronal loss at the newer and advancing edge of a lesion than in the older central part of the lesion. This case shows that MRI findings such as DWI and MRS may improve the diagnosis and the understanding of the pathophysiology of PML.
doi:10.3988/jcn.2007.3.4.200
PMCID: PMC2686949  PMID: 19513133
AIDS; Progressive multifocal leukoencephalopathy; Proton magnetic resonance spectroscopy; Diffusion-weighted imaging

Results 1-4 (4)