Acute-onset severe occipital neuralgia as a manifestation of neuromyelitis optica spectrum disorder relapse: A case series study

Objective:To describe leptomeningeal blood-barrier impairment reflected by MRI gadolinium-enhanced lesions in patients with aquaporin-4 immunoglobulin G (AQP4-IgG)–positive neuromyelitis optica spectrum disorder (NMOSD).Methods:A retrospective case series of 11 AQP4-IgG–positive NMOSD patients with leptomeningeal enhancement (LME) were collected from 5 centers. External neuroradiologists, blinded to the clinical details, evaluated MRIs.Results:LME was demonstrated on postcontrast T1-weighted and fluid-attenuated inversion recovery images as a sign of leptomeningeal blood-barrier disruption and transient leakage of contrast agent into the subarachnoid space in 11 patients, 6 in the brain and 6 in the spinal cord. The patterns of LME were linear or extensive and were accompanied by periependymal enhancement in 5 cases and intraparenchymal enhancement in all cases. The location of LME in the spinal cord was adjacent to intraparenchymal contrast enhancement with involvement of a median number of 12 (range 5–17) vertebral segments. At the time of LME on MRI, all patients had a clinical attack such as encephalopathy (36%) and/or myelopathy (70%) with median interval between symptom onset and LME of 12 days (range 2–30). LME occurred in association with an initial area postrema attack (44%), signs of systemic infection (33%), or AQP4-IgG in CSF (22%) followed by clinical progression. LME was found at initial clinical presentation in 5 cases and at clinical relapses leading to a diagnosis of NMOSD in 6 cases.Conclusion:This study suggests that altered leptomeningeal blood barrier may be accompanied by intraparenchymal blood-brain barrier breakdown in patients with AQP4-IgG–positive NMOSD during relapses.

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Pediatric Neuromyelitis Optica Spectrum Disorder: Case Series and Literature Review

Life ◽

10.3390/life12010019 ◽

2021 ◽

Vol 12 (1) ◽

pp. 19

Author(s):

Michela Ada Noris Ferilli ◽

Roberto Paparella ◽

Ilaria Morandini ◽

Laura Papetti ◽

Lorenzo Figà Talamanca ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Pediatric Patients ◽

Demyelinating Disease ◽

Area Postrema ◽

Case Series ◽

Myelin Oligodendrocyte Glycoprotein ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder ◽

Periaqueductal Gray Matter ◽

Aquaporin 4 Antibody

Neuromyelitis Optica Spectrum Disorder (NMOSD) is a central nervous system (CNS) inflammatory demyelinating disease characterized by recurrent inflammatory events that primarily involve optic nerves and the spinal cord, but also affect other regions of the CNS, including hypothalamus, area postrema and periaqueductal gray matter. The aquaporin-4 antibody (AQP4-IgG) is specific for NMOSD. Recently, myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) have been found in a group of AQP4-IgG negative patients. NMOSD is rare among children and adolescents, but early diagnosis is important to start adequate therapy. In this report, we present cases of seven pediatric patients with NMOSD and we review the clinical and neuroimaging characteristics, diagnosis, and treatment of NMOSD in children.

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Stroke Chameleons Manifesting as Distinct Radial Neuropathies: Expertise Can Hasten the Diagnosis

Case Reports in Neurology ◽

10.1159/000480229 ◽

2017 ◽

Vol 9 (3) ◽

pp. 277-283

Author(s):

Fabrício Diniz de Lima ◽

Gustavo José Luvizutto ◽

Arthur Oscar Schelp ◽

Gabriel Pereira Braga ◽

Rodrigo Bazan

Keyword(s):

Case Series ◽

Acute Onset ◽

Brain Scans ◽

Case Series Study ◽

Central Origin ◽

Central Gyrus ◽

Wrist Drop ◽

Series Study ◽

Ct And Mri

Objective: Stroke chameleons encompass an atypical group of syndromes that do not initially appear to be cerebrovascular accidents. The objective of this study was to report patients with different lesions of central origin clinically presenting as wrist drop and with a semiology similar to that produced by peripheral lesions of the radial nerve at different topographical levels. Methods: This is a case series study of patients presenting with wrist drop during the acute phase of stroke who were assessed by clinical examination and CT and MRI brain scans. Results: Three cases presenting as monoparesis were evaluated. In all patients, the MRI revealed restricted diffusion in the pre- and post-central gyrus. Electromyography showed that the functionality of the radial, median, and ulnar nerves were intact in all three cases. The monoparesis resolved completely within 1 month of rehabilitation therapy, and no evidence of recurrent or new events was reported during the 6-month follow-up after stenting. Conclusion: The central message of this study is that when acute onset symptoms are present in a relatively old patient with vascular risk factors, stroke should be considered as the possible aetiology until proven otherwise, and the appropriate steps should be taken to avoid a delay in the treatment and to improve outcomes.

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Interleukin-6 in neuromyelitis optica spectrum disorder pathophysiology

Neurology Neuroimmunology & Neuroinflammation ◽

10.1212/nxi.0000000000000841 ◽

2020 ◽

Vol 7 (5) ◽

pp. e841 ◽

Cited By ~ 2

Author(s):

Kazuo Fujihara ◽

Jeffrey L. Bennett ◽

Jerome de Seze ◽

Masayuki Haramura ◽

Ingo Kleiter ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Interleukin 6 ◽

Case Series ◽

Autoimmune Disorder ◽

Multiple Roles ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder ◽

Neurologic Disability ◽

Recent Phase

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder that preferentially affects the spinal cord and optic nerve. Most patients with NMOSD experience severe relapses that lead to permanent neurologic disability; therefore, limiting frequency and severity of these attacks is the primary goal of disease management. Currently, patients are treated with immunosuppressants. Interleukin-6 (IL-6) is a pleiotropic cytokine that is significantly elevated in the serum and the CSF of patients with NMOSD. IL-6 may have multiple roles in NMOSD pathophysiology by promoting plasmablast survival, stimulating the production of antibodies against aquaporin-4, disrupting blood-brain barrier integrity and functionality, and enhancing proinflammatory T-lymphocyte differentiation and activation. Case series have shown decreased relapse rates following IL-6 receptor (IL-6R) blockade in patients with NMOSD, and 2 recent phase 3 randomized controlled trials confirmed that IL-6R inhibition reduces the risk of relapses in NMOSD. As such, inhibition of IL-6 activity represents a promising emerging therapy for the management of NMOSD manifestations. In this review, we summarize the role of IL-6 in the context of NMOSD.

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P-OT003. A case series of familial neuromyelitis optica spectrum disorder (NMOSD)

Clinical Neurophysiology ◽

10.1016/j.clinph.2021.02.304 ◽

2021 ◽

Vol 132 (8) ◽

pp. e123

Author(s):

Chai Liang Ang ◽

Jie Ping Schee ◽

Eng Kian Ng ◽

Yuen Kang Chia

Keyword(s):

Neuromyelitis Optica ◽

Case Series ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder

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Adverse events of rituximab in neuromyelitis optica spectrum disorder: a systematic review and meta-analysis

Therapeutic Advances in Neurological Disorders ◽

10.1177/17562864211056710 ◽

2021 ◽

Vol 14 ◽

pp. 175628642110567

Author(s):

Hao Wang ◽

Juanping Zhou ◽

Yi Li ◽

Lili Wei ◽

Xintong Xu ◽

...

Keyword(s):

Adverse Events ◽

Neuromyelitis Optica ◽

Meta Analysis ◽

Case Series ◽

Spectrum Disorder ◽

Cochrane Library ◽

Neuromyelitis Optica Spectrum Disorder ◽

Duration Of Illness ◽

Evidence Quality ◽

Study Designs

Background: The adverse events (AEs) of rituximab (RTX) for neuromyelitis optica spectrum disorder (NMOSD) are incompletely understood. Aim: To collate information on the reported the AEs of RTX in NMOSD and assess the quality of evidence. Methods: PubMed, EMBASE, Web of Science, Cochrane Library, Wanfang Data, CBM, CNKI, VIP, clinicaltrials.gov, and so on were searched for studies with control groups as well as for case series that had assessed the RTX-associated AEs. The incidence of AEs and the comparison of AE risks among different therapies were pooled. The GRADE was developed for evidence quality. Results: A total of 3566 records were identified. Finally, 36 studies (4 RCTs, 6 crochet studies, 2 NRCTs, and 24 case series), including 1542 patients (1299 females and 139 males), were included for final analyses. Rates of patients with any AEs, any serious AEs (SAEs), infusion-related AEs, any infection, respiratory infection, urinary infection, and death were 28.57%, 5.66%, 27.01%, 17.36%, 4.76%, 4.76%, and 0.17%, respectively. The results from subgroup analysis showed that AE rates were most likely not associated with covariates such as duration of illness and study designs. Very low-quality evidence suggested that the risk ratios (RR) of any AEs (0.84, 95% CI = 00.42–1.69, p = 0.62) and any infections (1.24 95% CI = 0.18–8.61) of RTX were similar to that of azathioprine, and the RR of any AEs of RXT was akin to that of mycophenolate mofetil (0.66, 95% CI = 0.32–1.35 p = 0.26). Evidence of low to high quality showed the lower RR of RTX in other AEs, but not in infusion-related AEs. Strategies to handle AEs focused on symptomatic treatments. Conclusions: RTX is mostly safer than other immunosuppressants in NMOSD: the incidence of RTX-associated AEs was not high, and when present, the AEs were usually mild or moderate and could be well controlled. Given its efficacy and safety, RTX could be recommended as a first-line treatment for NMOSD.

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Seropositive neuromyelitis optica spectrum disorder in Emirati patients: A case series

Journal of Clinical Neuroscience ◽

10.1016/j.jocn.2019.11.045 ◽

2020 ◽

Vol 72 ◽

pp. 185-190

Author(s):

Antonia Ceccarelli ◽

Victoria Ann Mifsud ◽

Amna Dogar ◽

Syed Irteza Hussain

Keyword(s):

Neuromyelitis Optica ◽

Case Series ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder

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