Neuromyelitis optica spectrum disorder as a paraneoplastic manifestation of lung adenocarcinoma expressing aquaporin-4

2015 ◽  
Vol 21 (6) ◽  
pp. 791-794 ◽  
Author(s):  
Raffaele Iorio ◽  
Guido Rindi ◽  
Carmen Erra ◽  
Valentina Damato ◽  
Michela Ferilli ◽  
...  
Keyword(s):  
Lung Adenocarcinoma ◽  
Tumor Cells ◽  
Neuromyelitis Optica ◽  
Late Onset ◽  
Transverse Myelitis ◽  
Aquaporin 4 ◽  
Aqp4 Expression ◽  
Spectrum Disorders ◽  
Oncological Screening ◽  
Extensive Transverse Myelitis

Background: The observations of neuromyelitis optica spectrum disorders (NMOSD) occurring in the setting of cancer suggest that aquaporin-4 (AQP4) autoimmunity may in some cases be paraneoplastic. Results: We describe a 72-year-old patient who developed a longitudinally extensive transverse myelitis associated with AQP4 autoantibodies in the setting of a lung adenocarcinoma recurrence. AQP4 expression was demonstrated in tumor cells. IgG in patient’s cerebrospinal fluid bound to tumor cells co-localizing with AQP4 immunoreactivity. Conclusions and relevance: This case expands the spectrum of paraneoplastic AQP4 autoimmunity highlighting the importance of considering an oncological screening in patients with late-onset NMOSD.

Atypical myelitis in patients with multiple sclerosis: Characterization and comparison with typical multiple sclerosis and neuromyelitis optica spectrum disorders

2020 ◽  
pp. 135245852090699
Author(s):  
K Bigaut ◽  
C Lambert ◽  
L Kremer ◽  
C Lebrun ◽  
M Cohen ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
First Episode ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Disability Status ◽  
Plane Transverse ◽  
Spectrum Disorders ◽  
Extensive Transverse Myelitis

Background: Atypical myelitis in multiple sclerosis (MS) is characterized by extensive myelitis in the longitudinal (longitudinally extensive transverse myelitis) or axial plane (transverse myelitis). Objective: To characterize a cohort of MS patients with atypical myelitis. Methods: Atypical myelitis was extracted from the French and Luxembourg MS databases and compared to two cohorts of MS patients with typical myelitis and neuromyelitis optica spectrum disorders (NMOSDs) patients with myelitis. Results: We enrolled 28 MS patients with atypical myelitis, 68 MS patients with typical myelitis and 119 NMOSD patients with a first episode of myelitis. MS patients with atypical myelitis were characterized by a mean age of 34.0 (±10.7) years and 64.3% were women. In 82.1% of the patients, atypical myelitis was the first episode of MS. Mean Expanded Disability Status Scale (EDSS) scores at nadir and 3–6 months after onset were 4.1 ± 2.1 and 3.3 ± 2, respectively. Differences between groups revealed a predominance of cervicothoracic myelitis and a higher level of disability in NMOSD patients. Disability in MS patients with atypical myelitis was more severe than in the MS patients with typical myelitis; 28% had already converted to progressive MS within our mean follow-up of 39.6 (±30.4) months. Conclusion: Atypical myelitis may be the first presentation of MS and is associated with poorer prognosis.

scholarly journals Memantine ameliorates motor impairments and pathologies in a mouse model of neuromyelitis optica spectrum disorders.

2020 ◽  
Author(s):  
Leung-Wah Yick ◽  
Chi-Ho Tang ◽  
Oscar Ka-Fai Ma ◽  
Jason Shing-Cheong Kwan ◽  
Koon Ho CHAN
Keyword(s):  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Neurotrophic Factor ◽  
Water Channel ◽  
Transverse Myelitis ◽  
Aquaporin 4 ◽  
Glutamate Excitotoxicity ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Motor Impairments ◽  
Spectrum Disorders

Abstract Background: Neuromyelitis optica spectrum disorders (NMOSD) are central nervous system (CNS) autoimmune inflammatory demyelinating diseases characterized by recurrent episodes of acute optic neuritis and transverse myelitis. Aquaporin-4 immunoglobulin G (AQP4-IgG) autoantibodies, which target the water channel aquaporin-4 (AQP4) on astrocytic membrane, are pathogenic in NMOSD. Glutamate excitotoxicity, which is triggered by internalization of AQP4-glutamate transporter complex after AQP4-IgG binding to astrocytes, is involved in early NMOSD pathophysiologies. We studied the effects of memantine, a N-methyl-D-aspartate (NMDA) receptor antagonist, on motor impairments and spinal cord pathologies in mice which received human AQP4-IgG. Methods: Purified IgG from AQP4-IgG-seropositive NMOSD patients were passively transferred to adult C57BL/6 mice with disrupted blood-brain barrier. Memantine was administered by oral gavage. Motor impairments of the mice were assessed by beam walking test. Spinal cords of the mice were assessed by immunofluorescence and ELISA. Results: Oral administration of memantine ameliorated the motor impairments induced by AQP4-IgG, no matter the treatment was initiated before (preventive) or after (therapeutic) disease flare. Memantine profoundly reduced AQP4 and astrocyte loss, and attenuated demyelination and axonal loss in the spinal cord of mice which had received AQP4-IgG. The protective effects of memantine were associated with inhibition of apoptosis and suppression of neuroinflammation, with decrease in microglia activation and neutrophil infiltration and reduction of increase in levels of proinflammatory cytokines including interleukin-1β (IL-1β), interleukin-6 (IL-6) and tumor necrosis factor-α (TNF- α). In addition, memantine elevated growth factors including brain-derived neurotrophic factor (BDNF), glial cell line-derived neurotrophic factor (GDNF) and vascular endothelial growth factor (VEGF) in the spinal cord. Conclusions: Our findings support that glutamate excitotoxicity and neuroinflammation plays important roles in complement-independent pathophysiology during early development of NMOSD lesions, and highlight the potential of oral memantine as a therapeutic agent in NMOSD acute attacks.

scholarly journals Short segment myelitis as the initial and only manifestation of aquaporin-4 immunoglobulin G-positive neuromyelitis optica spectrum disorders

2020 ◽  
Vol 13 ◽  
pp. 175628641989859
Author(s):  
Wei Fang* ◽  
Yang Zheng* ◽  
Fan Yang ◽  
Meng-Ting Cai ◽  
Chun-Hong Shen ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Immunoglobulin G ◽  
Correct Diagnosis ◽  
Transverse Myelitis ◽  
Aquaporin 4 ◽  
Chinese Patients ◽  
Careful Study ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Short Segment ◽  
Spectrum Disorders

Background: Short segment myelitis (SSM, < 3 vertebral segments) is an under-recognized initial manifestation of neuromyelitis optica spectrum disorders (NMOSD). Though infrequent, failure to recognize SSM in patients with NMOSD would lead to incorrect diagnosis and treatment. Therefore, delineation of features of NMOSD-associated SSM is of paramount importance. Objective: Our study aimed to determine the demographic, clinical and radiological features of NMOSD-associated SSM, and compare those with NMOSD-associated longitudinally extensive transverse myelitis (LETM) and multiple sclerosis (MS)-associated SSM, respectively. Methods: Chinese patients presenting initially only with acute myelitis and diagnosed with NMOSD ( n = 46) and MS ( n = 11) were included. Clinical, serological, imaging and disability data were collected. Mann–Whitney U test or two-tailed Fisher’s exact tests were used to analyse the data. Results: Of the 46 enrolled NMOSD patients, 34 (74%) collectively had 38 LETM lesions, while 12 (26%) had 14 SSM lesions. When compared with LETM, NMOSD presenting with SSM were more likely to have a delayed diagnosis and a lower level of disability at nadir during the first attack. T1-weighted imaging hypointensity was more prominent in NMOSD-associated LETM lesions than NMOSD-associated SSM lesions. When compared with MS-associated SSM, NMOSD-associated SSM lesions were more likely to be centrally located, grey matter involving and transversally extensive on axial imaging and spanned no less than 2 vertebral segments on sagittal imaging. Conclusion: These findings suggest that SSM does not preclude the possibility of a NMOSD diagnosis. Testing for serum aquaporin-4 immunoglobulin G (AQP4-IgG) and careful study of lesions on spinal cord magnetic resonance imaging could aid in an earlier and correct diagnosis.

scholarly journals The Frequency of Anti-Aquaporin-4 Ig G Antibody in Neuromyelitis Optica and Its Spectrum Disorders at a Single Tertiary Referral Center in Malaysia

2014 ◽  
Vol 2014 ◽  
pp. 1-10 ◽  
Author(s):  
Shanthi Viswanathan ◽  
Masita Arip ◽  
Norhazlin Mustafa ◽  
Jasbir S. Dhaliwal ◽  
Norzainie Rose ◽  
...  
Keyword(s):  
High Risk ◽  
Neuromyelitis Optica ◽  
Demyelinating Disease ◽  
Transverse Myelitis ◽  
Female Gender ◽  
Aquaporin 4 ◽  
Central Gray Matter ◽  
Tertiary Referral Center ◽  
Spectrum Disorders ◽  
Ig G

Background. In the past the occurrence of neuromyelitis optica in Malaysia was thought to be uncommon and the frequency of anti-aquaporin-4 Ig G antibody was unknown.Objective. To evaluate the frequency of anti-aquaporin-4 Ig G antibody (Anti-AQP4 antibody) amongst patients with neuromyelitis optica (NMO) and its spectrum disorders (NMOSD) and the differences between the seropositive and seronegative groups.Methods. Retrospectively, 96 patients with NMO/high risk syndromes for NMOSD (HRS-NMOSD) were identified out of 266 patients with idiopathic inflammatory demyelinating disease from a single center hospital based registry. Anti-AQP4 seropositivity was found in 38/48 (79.2%) with NMO, 12/21 (57.1%) with brain involvement at high risk for NMOSD, 12/15 (80%) with transverse myelitis (i.e., 11/15 with relapsing transverse myelitis and one with monophasic transverse myelitis), and 3/7 (42.8%) with relapsing optic neuritis. Sixty-five out of 96 patients, that is, 67.7%, with NMO/HRS for NMOSD were seropositive. Seropositivity was significantly associated with female gender, a higher number of mean relapses, that is, 5.15 ± 4.42 versus 2.10 ± 1.68, longer length of spinal cord lesions, that is, 6.6 ± 4.9 versus 2.9 ± 2.5, vertebral bodies, higher EDSS, 4.5 ± 2.4 versus 2.4 ± 2.6, presence of paroxysmal tonic spasms, and blindness (unilateral/bilateral);P<0.001. Longitudinally extensive cord lesions (contiguous or linear), presence of lesions in the cervical and thoracic regions, and involvement of the central gray matter or holocord regions on axial scans, were also significantly associated with seropositivity;P<0.001.Conclusion. NMO and HRS for NMOSD are present in larger numbers than previously thought in Malaysia. More than 2/3rds are seropositive. Seropositive and seronegative NMO/NMOSD have differences that are useful in clinical practice.

scholarly journals Pulmonary Tuberculosis with Longitudinally Extensive Transverse Myelitis

2020 ◽  
Vol 11 (01) ◽  
pp. 178-182
Author(s):  
Kiran Kumar Ramineni ◽  
Ravi Kanth Jakkani ◽  
B. V. G. Swamy ◽  
Sravan Kumar M.
Keyword(s):  
Pulmonary Tuberculosis ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Good Recovery ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Active Pulmonary Tuberculosis ◽  
Rare Phenomenon ◽  
Spectrum Disorders ◽  
Extensive Transverse Myelitis

AbstractLongitudinally extensive transverse myelitis (LETM) is described in neuromyelitis optica spectrum disorders. Simultaneous active pulmonary tuberculosis in these disorders is a relatively rare phenomenon. We report a 16 year-old boy diagnosed as LETM with clinicoradiological correlation. Further evaluation revealed active pulmonary tuberculosis. He had good recovery following the combination of antituberculosis regimen with corticosteroids.

Neuromyelitis optica spectrum disorders: beyond longitudinally extensive transverse myelitis

2015 ◽  
Vol 70 (6) ◽  
pp. 630-637 ◽  
Author(s):  
M.D. Lemos ◽  
G.B.S. Carvalho ◽  
R.S. Carvalho ◽  
D.B. Bichuetti ◽  
E.M.L. de Oliveira ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Spectrum Disorders ◽  
Extensive Transverse Myelitis

scholarly journals The expanded spectrum of neuromyelitis optica: evidences for a new definition

2012 ◽  
Vol 70 (10) ◽  
pp. 807-813 ◽  
Author(s):  
Marco A Lana-Peixoto ◽  
Dagoberto Callegaro
Keyword(s):  
Optic Nerve ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Brain Mri ◽  
Transverse Myelitis ◽  
Aquaporin 4 ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Index Event ◽  
Aquaporin 4 Antibody ◽  
Extensive Transverse Myelitis

Neuromyelitis optica (NMO) has been traditionally described as the association of recurrent or bilateral optic neuritis and longitudinally extensive transverse myelitis (LETM). Identification of aquaporin-4 antibody (AQP4-IgG) has deeply changed the concept of NMO. A spectrum of NMO disorders (NMOSD) has been formulated comprising conditions which include both AQP4-IgG seropositivity and one of the index events of the disease (recurrent or bilateral optic neuritis and LETM). Most NMO patients harbor asymptomatic brain MRI lesions, some of them considered as typical of NMO. Some patients with aquaporin-4 autoimmunity present brainstem, hypothalamic or encephalopathy symptoms either preceding an index event or occurring isolatedly with no evidence of optic nerve or spinal involvement. On the opposite way, other patients have optic neuritis or LETM in association with typical lesions of NMO on brain MRI and yet are AQP4-IgG seronegative. An expanded spectrum of NMO disorders is proposed to include these cases.

scholarly journals Neuromyelitis optica: phenotypic characteristics in a Brazilian case series

2017 ◽  
Vol 75 (2) ◽  
pp. 81-86 ◽  
Author(s):  
Maria Cristina Del Negro ◽  
Patricia Beatriz Christino Marinho ◽  
Regina Maria Papais-Alvarenga
Keyword(s):  
Neuromyelitis Optica ◽  
Case Series ◽  
Transverse Myelitis ◽  
International Panel ◽  
Longitudinal Extensive Transverse Myelitis ◽  
Study Results ◽  
Fourth Decade ◽  
Spectrum Disorders ◽  
Definition Of ◽  
Extensive Transverse Myelitis

ABSTRACT The definition of neuromyelitis optica (NMO) is still evolving. In 2015, the International Panel for NMO Diagnosis was convened to develop revised diagnostic criteria. There have been few studies on NMO in the Brazilian population. Objective To describe the characteristics of 34 Brazilian NMO patients. To evaluate the contribution of the 2015 criteria to the diagnosis of NMO spectrum disorders (NMOSD) in 40 patients with longitudinal extensive transverse myelitis (LEMT). Methods This is a retrospective, descriptive and analytic study. Results Among NMO patients, there was a predominance of women, with onset in the fourth decade of life, and AQP4-IgG seropositivity in 73.5%. The diagnosis of NMOSD was established in 37.5% of LETM patients according to AQP4-IgG positivity and in 5% of LETM patients if the AQP4-IgG result was unknown. Conclusions The characteristics of this series are similar to those of other Western populations. The AQP4-IgG testing assists in the diagnosis of NMOSD.

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