scholarly journals Enhancing Brain Lesions during Acute Optic Neuritis and/or Longitudinally Extensive Transverse Myelitis May Portend a Higher Relapse Rate in Neuromyelitis Optica Spectrum Disorders

2017 ◽  
Vol 38 (5) ◽  
pp. 949-953 ◽  
Author(s):  
G. Orman ◽  
K.Y. Wang ◽  
Y. Pekcevik ◽  
C.B. Thompson ◽  
M. Mealy ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Relapse Rate ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Brain Lesions ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Acute Optic Neuritis ◽  
Spectrum Disorders ◽  
Extensive Transverse Myelitis

scholarly journals Anti-B-cell therapy in patients with neuromyelitis optica spectrum disorders

2021 ◽  
Vol 13 (4) ◽  
pp. 18-24
Author(s):  
S. V. Kotov ◽  
E. S. Novikova ◽  
A. S. Kotov
Keyword(s):  
Optic Neuritis ◽  
Relapse Rate ◽  
Neuromyelitis Optica ◽  
Clinical Manifestations ◽  
Treatment Protocol ◽  
Transverse Myelitis ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Intravenous Infusions ◽  
Annualized Relapse Rate ◽  
Spectrum Disorders

Neuromyelitis optica spectrum disorders (NMOSDs) are a group of central nervous system autoimmune diseases characterized by similar clinical manifestations, optic neuritis, and transverse myelitis being the most frequent among them. In most cases, the pathogenesis of NMOSDs is associated with autoantibodies to aquaporin-4 (AQP4-IgG). However, AQP4-IgG is not detected in at least 10-20% of patients with NMOSDs. In this subgroup and in patients with isolated transverse myelitis or optic neuritis, IgG antibodies to myelin oligodendrocyte glycoprotein (MOG-IgG) were detected. Patients seronegative for both AQP4-IgG and MOG-IgG have also been described.Objective: to evaluate rituximab (RTX) effectiveness in preventing relapses and disability in patients with NMOSDs.Patients and methods. The study included 27 patients with NMOSDs (9 men and 18 women) aged 20-51 years who received RTX in 2019-2021. The treatment protocol included intravenous infusions of 1000 mg of RTX on the 1st and 15th days, the second and subsequent courses (maintenance therapy) - intravenous infusions of 1000 mg of RTX once every six months. Treatment effectiveness was assessed by the average annualized relapse rate, the median changes of the Expanded Disability Status Scale (EDSS), and based on the magnetic resonance imaging (MRI) changes.Results and discussion. The annualized relapse rate at baseline and 18 months after the start of treatment was: all patients (n=27) — 0.6±0.3 and 0.07±0.27(p<0.0001); AQP4-IgG+ patients (n=6) — 1.1±0.9 and 0.17±0.41 (p=0.028); MOG-IgG+ patients (n=14) — 0.4±0.3 and 0.07±0.28(p=0.001); AQP4-IgG-, MOG-IgG-patients (n=7) — 0.8±0.4 and 0.0±0.0 (p=0.018). The EDSSscore at baseline and 18months after the start of treatment was: all patients — 4.5 [3.25; 6.0] and 4.0 [3.0; 5.75] (p=0.679); AQP4-IgG+ — 3.5 [2.625; 4.75] and 3.5 [2.5; 4.5] (p=0.869); MOG-IgG+ - 5.5[3.75; 6.5] and 5.5[2.75; 6.25] (p=0.465); AQP4-IgG-, MOG-IgG- - 4.0[3.75; 5.25] and 3.5[3.0; 3.5] (p=0.043). We observed two clinical relapses during the study period: one in an AQP4-IgG+ male and another one in a MOG-IgG+ woman. There was a significant decrease in the annualized relapse rate in all groups. The disability indicator did not increase during the study period, and in AQP4-IgG and MOG-IgG seronegative patients, it slightly but significantly decreased. Brain and spinal cord MRI monitoring during the treatment period revealed new active foci only in two patients with clinical relapses.Conclusion. RTX treatment in NMOSDs is reasonably efficient and safe, but with the obligatory prior patient evaluation and monitoring of treatment results.

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 Atypical presentations of neuromyelitis optica

2011 ◽  
Vol 69 (5) ◽  
pp. 824-828 ◽  
Author(s):  
Douglas Sato ◽  
Kazuo Fujihara
Keyword(s):  
Multiple Sclerosis ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Brain Lesions ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Atypical Presentations ◽  
Different Characteristics ◽  
Extensive Transverse Myelitis ◽  
Intractable Hiccups

Neuromyelitis optica (NMO) is an inflammatory disease of central nervous system classically characterized by acute, severe episodes of optic neuritis and longitudinally extensive transverse myelitis, usually with a relapsing course. The identification of an autoantibody exclusively detected in NMO patients against aquaporin-4 (AQP-4) has allowed identification of cases beyond the classical phenotype. Brain lesions, once thought as infrequent, can be observed in NMO patients, but lesions have different characteristics from the ones seen in multiple sclerosis. Additionally, some AQP-4 antibody positive patients may present with a variety of symptoms not being restricted to optic neuritis and acute myelitis during the first attack or in a relapse. Examples are not limited to, but may include patients only with brain and/or brainstem lesions, narcolepsy with hypothalamic lesions or patients with intractable hiccups, nausea and vomiting. The prompt identification of NMO patients with atypical presentations may benefit these patients with institution of early treatment to reduce disability and prevent further attacks.

scholarly journals Neuropathic pruritus: an early indicator of neuromyelitis optica spectrum disorders

2018 ◽  
Vol 6 (8) ◽  
pp. 2867
Author(s):  
Pradeep Kumar Bansal ◽  
C. L. Nawal ◽  
Aradhana Singh ◽  
Radheyshyam Chejara ◽  
Sebastian Marker ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Neuromyelitis Optica Spectrum Disorders ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Chief Complaints ◽  
Neuropathic Pruritus ◽  
Spectrum Disorders ◽  
Tonic Spasms ◽  
Extensive Transverse Myelitis

Neuromyelitis optica is a relapsing, inflammatory astrocytopathic disorder, affecting predominantly the optic nerves and spinal cord. It is associated with antiaquaporin-4 immunoglobulin G (AQP4-IgG) in up to 70% of patients. Spinal cord involvement typically presents as a longitudinally-extensive transverse myelitis, with associated sensorimotor and sphincter dysfunction. Sensory symptoms such as numbness, dysaesthesia, pain and tonic spasms are common. Here, we present a case of a 25years old female who came to the medicine OPD, with the chief complaints of intense itching over face and forehead, which was later on progressed to quadriparesis after 3 weeks. This case highlights neuropathic pruritus as an under-recognised early feature of neuromyelitis optica.

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 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.

An early case of neuromyelitis optica: on a forgotten report by Jacob Lockhart Clarke, FRS

2011 ◽  
Vol 17 (11) ◽  
pp. 1384-1386 ◽  
Author(s):  
S Jarius ◽  
B Wildemann
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
English Language ◽  
Medical Literature ◽  
Transverse Myelitis ◽  
Longitudinally Extensive Transverse Myelitis ◽  
Devic’S Syndrome ◽  
Early Case ◽  
Extensive Transverse Myelitis

We discuss a forgotten report by the famous British neuroanatomist, neuropathologist and neurologist Jacob Augustus Lockhart Clarke (1817–1880) about a 17-year-old girl with bilateral optic neuritis and longitudinally extensive transverse myelitis. This report, which appeared in 1865, i.e. 15 years prior to Wilhelm Erb’s much-cited paper on the coincidence of optic neuritis and acute myelitis, represents the first known account of a case of Devic’s syndrome or neuromyelitis optica in the English-language medical literature.

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