Aquaporin-4 autoimmune syndrome and anti-aquaporin-4 antibody-negative opticospinal multiple sclerosis in Japanese

Matsushita, Takuya; Isobe, Noriko; Matsuoka, Takeshi; Song, Nan; Kawano, Yoshiaki; Wu, Xiongfei; Yamada, Takashi; Nakao, Yosuke; Ishizu, Takaaki; Kira, J.

doi:10.1177/1352458509104595

Cited by 63 publications

(80 citation statements)

References 38 publications

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“…In a French study, Marignier and colleagues reported that there was no difference between NMOIgG sero-positive and sero-negative NMO patients according to their clinical, CSF, and imaging features 14 . However, in the Japanese, AQP4 antibody-positive NMO/OSMS patients had a greater frequency of longitudinally extensive spinal cord or brain lesions than did anti-AQP4 antibody-negative NMO/OSMS patients 9,[15][16][17] . These discrepancies may be accounted for by patient selection (NMO versus OSMS patients; different populations) and false-negative findings because of the low sensitivity of the assay.…”

Section: Discussionmentioning

confidence: 88%

“…A previous study suggested that the presence of AQP4 antibodies was 33-91% sensitive (median 63%) and 85-100% specific (median 99%) for NMO 9 .…”

Section: Discussionmentioning

confidence: 92%

“…Although more than 15 different immunoassays for the detection of AQP4 antibodies in patients with NMO have been developed, only a proportion of patients (33-91% among Western NMO cases and 41.7-91% among Japanese opticospinal MS (OSMS) are positive for AQP4 antibodies in serum 3,9,10 . In other words, the number of sero-negative NMO patients is not negligible.…”

Section: Original Articlementioning

confidence: 99%

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Serum-Positive and -Negative AQP4 Antibody NMO in Chinese Patients

Long

Liu

2012

Can. J. Neurol. Sci.

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232Neuromyelitis optica (NMO) is a severe idiopathic immunomediated inflammatory demyelinating and necrotizing disease that is characterized by transverse myelopathy and optic neuropathy. The exact etiology and mechanisms of NMO remain unknown. Serological and clinical evidence of B cell autoimmunity has been observed in a high proportion of patients, 1 suggesting a prominent role for humoral mechanisms in the pathogenesis of NMO. Neuromyelitis optica lesions show a marked deposition of immunoglobulins and complement in a characteristic perivascular rosette pattern along the outer rim of thickened vessel walls 2 . A specific IgG autoantibody, NMO-IgG, selectively targeting aquaporin 4 (AQP4), which accumulates in the central nervous system (CNS) microvessels, pia, subpia, and Virchow-Robin space, has been confirmed by indirect immunofluorescence with a composite substrate of mouse tissue 3,4 . According to this antibody, the new diagnostic criteria for NMO proposed by Wingerchuk et al facilitate its distinction from multiple sclerosis (MS) 5,6 . In subsequent studies, analysis of pathological brain lesions in NMO patients revealed a pattern of vasculocentric immune complex deposition and AQP4 loss ABSTRACT: Objective: To compare the clinical features of our sero-negative and sero-positive neuromyelitis optica (NMO) patients. Methods: Thirty-nine patients with NMO were recruited and analyzed retrospectively. Serum aquaporin 4 (AQP4) antibody status was determined by a cell-based assay. For the sero-negative patients, cerebrospinal fluid (CSF) and serum samples were re-tested using the cell-based assay and an indirect immunofluorescence assay. Results: By the cell-based assay, 30 patients (76.92%, 30/39), were positive for AQP4 antibodies in serum and 37 patients (94.9%, 37/39), had a CSF-positive antibody status. Seven NMO patients (17.9%, 7/39) were sero-negative by the cell-based assay but demonstrated positive CSF results. By indirect immunofluorescence, the remaining two patients, who had no AQP4 antibodies in serum or CSF by the cell-based assay, were positive for IgG antibodies in serum, which selectively targeted the central nervous system microvessels, pia, subpia, Virchow-Robin space, kidney, and stomach. There were no significant differences between the sero-positive and sero-negative NMO groups among their demographic and clinical data. Conclusions: Repeating the test using a different assay or CSF is helpful to clarify whether sero-negative NMO patients do in fact carry AQP4 antibodies. ORIGINAL ARTICLEidentical to that seen in NMO lesions, suggesting that AQP4 antibodies are related to NMO pathology 7,8 .Although more than 15 different immunoassays for the detection of AQP4 antibodies in patients with NMO have been developed, only a proportion of patients (33-91% among Western NMO cases and 41.7-91% among Japanese opticospinal MS (OSMS) are positive for AQP4 antibodies in serum 3,9,10 . In other words, the number of sero-negative NMO patients is not negligible.https://www.cambridge.org/core/terms. h...

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Section: Discussionmentioning

confidence: 88%

“…A previous study suggested that the presence of AQP4 antibodies was 33-91% sensitive (median 63%) and 85-100% specific (median 99%) for NMO 9 .…”

Section: Discussionmentioning

confidence: 92%

Section: Original Articlementioning

confidence: 99%

See 1 more Smart Citation

Serum-Positive and -Negative AQP4 Antibody NMO in Chinese Patients

Long

Liu

2012

Can. J. Neurol. Sci.

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“…Amongst Hong Kong Chinese, 60% seropositivity was reported. 106,108,135,139 In a mixed Asian and European Turkish population, sensitivity was 45%. 109 A lower rate of seropositivity (33%) was reported in the Caribbean area, where NMO patients are of predominantly African ancestry, 60 whereas in Spanish, Italian and French populations of Europe, and Brazilian cohorts, seropositivity more frequently corresponded to Caucasian data (41-64%).…”

Section: Brain Mrimentioning

confidence: 98%

“…4,26,61,110 Anti-AQP4 antibody-positive Japanese patients with optico-spinal MS (OSMS) might be immunologically different from antibody-negative OSMS patients. 135 The NMO-IgG ⁄ anti-AQP4 seropositivity rate widely varied according to ethnicity. The sensitivity of the original indirect immunofluorescent method detecting NMO-IgG in Caucasian populations is 42-78%, whereas specificity is 91-100%.…”

Section: Brain Mrimentioning

confidence: 99%

Pathogenesis, diagnosis and treatment of neuromyelitis optica: Changing concept of an old disease

Illés

2010

Clinical & Exp Neuroim

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The concept of neuromyelitis optica (NMO) has changed considerably during the past few years. The identification of autoantibodies in the sera generated against the water channel aquaporin 4 (AQP4) has increased the specificity of diagnosis and modified guidelines. A pathogenic role of anti‐AQP4 antibodies has recently been indicated; they evoke the pathological and clinical hallmarks of the disease on transfer and cause necrosis of astrocytes expressing AQP4. The diagnosis of NMO is based on clinical, neuroimaging and serological criteria. Early therapy preventing relapses is mandatory, because neurological impairment accumulated during relapses is the main cause of permanent disability. In a number of cases defined as NMO spectrum diseases, the clinical manifestation is spatially limited. Such events of separate myelitis or relapsing/bilateral optic neuritis challenge diagnosis and might delay proper therapy. Here, current concepts of diagnosis and treatment of NMO and NMO spectrum diseases are summarized. Diagnostic and treatment decisions in different clinical situations are shown by discussion of cases. (Clin. Exp. Neuroimmunol. doi: 10.1111/j.1759‐1961.2010.00011.x, 2010)

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