scholarly journals Therapeutic plasma exchange for neuromyelitis optica spectrum disorder: A multicenter retrospective study by the ASFA neurologic diseases subcommittee

2019 ◽  
Vol 35 (1) ◽  
pp. 25-32 ◽  
Author(s):  
Tina S. Ipe ◽  
Jay S. Raval ◽  
Leonor P. Fernando ◽  
Amit Gokhale ◽  
Cyril Jacquot ◽  
...  
2019 ◽  
Vol 7 (20) ◽  
pp. 3433-3436
Author(s):  
Laura Tambunan ◽  
K. Ritarwan ◽  
K. P. Surbakti

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease that causes severe demyelination, especially in the optic nerve and spinal cord with typical clinical manifestations of acute optic neuritis and transverse myelitis. The symptoms can occur simultaneously or separated by a variable period. NMOSD is associated with serum aquaporin antibodies 4 immunoglobulin G (AQP4-IgG). CASE PRESENTATION: We report a case of a 22-year-old male with complaints of weakness of all four limbs, impaired vision, urinary incontinence, and dyspnea. The Expanded Disability Status Scale (EDSS) was nine. Spinal magnetic resonance imaging (MRI) showed longitudinal extensive transversal myelitis. The brain MRI showed a normal impression, whereas the brain magnetic resonance spectroscopy (MRS) examination showed a description of the mild demyelination process. The serum antibody AQP4 (AQP4-IgG) results were seronegative, the cerebrospinal fluid examination was normal, and the oligoclonal band was negative. The ophthalmoscopic examination found bilateral papillary atrophy but optical coherence tomography (OCT) was still normal. Somatosensory evoked potential and visual evoked potential examinations were abnormal. The patient was diagnosed with NMOSD and was given combination immunosuppressant therapy, corticosteroids, and therapeutic plasma exchange. The patient experienced significant improvement with EDSS decreased to six. CONCLUSION: In the case of relapsing NMOSD patient, combination therapy of immunosuppressants, corticosteroids, and TPE was used. There were significant improvements from EDSS nine to six.


2015 ◽  
Vol 7 (1) ◽  
pp. 78-83 ◽  
Author(s):  
Richard Heekin ◽  
Chetan Gandhy ◽  
Derrick Robertson

Controversy exists regarding a potential link between exposure to recombinant hepatitis B vaccine (HBV) and central nervous system demyelinating diseases. Here, we present a case of seronegative neuromyelitis optica spectrum disorder (NMOSD) following exposure to HBV. A 28-year-old man developed painful eye movements 11 days after exposure to HBV. Within 24 h, he experienced vision loss, ascending numbness, and ataxia. T-spine MRI showed a cord lesion spanning T6-T9. Brain MRI showed bilateral optic nerve contrast enhancement and a right-sided internal capsule lesion. Cerebrospinal fluid analysis was normal, including negative oligoclonal bands and normal IgG index. AQP4-IgG serology was negative. The patient's visual symptoms improved after treatment with steroids and plasma exchange. He received plasma exchange weekly for 4 weeks with decreased numbness and tingling as well as improved coordination. Treatment with mycophenolate mofetil was started, and the patient remains clinically stable with near resolution of his prior symptoms. Neuromyelitis optica is characterized by optic neuritis and/or longitudinally extensive transverse myelitis. While our patient tested seronegative for AQP4-IgG (which remains negative in 10-50% of NMOSD cases, despite testing with the most sensitive assays available), he did meet NMOSD diagnostic criteria. In a literature review, we found 7 cases of NMOSD onset or relapse associated with exposure to various vaccines, but to our knowledge this represents the first published report of NMOSD onset following exposure to HBV. While causality between vaccination and CNS demyelinating disease remains elusive, it is important to report these cases to help develop safer vaccinations and provoke further inquiry into the pathogenesis of NMOSD.


2020 ◽  
Vol 35 (4) ◽  
pp. 291-296 ◽  
Author(s):  
Ariel Dahan ◽  
Fabienne Brilot ◽  
Richard Leventer ◽  
Andrew J. Kornberg ◽  
Russell C. Dale ◽  
...  

Neuromyelitis optica spectrum disorder is uncommon in children, and often seronegative for aquaporin-4 immunoglobulin G (AQP4-IgG). We conducted a retrospective study of 67 children presenting to a single Australian center with acquired demyelinating syndromes over a 7-year period. All patients were tested for AQP4-IgG. Five children (7.5%) had neuromyelitis optica spectrum disorder. One child was seropositive for AQP4-IgG (1.5%) and had a relapsing disease course with mild residual deficits. She also had a concomitant motor axonal neuropathy that improved with immunosuppressive therapy. Of the remaining 4 children, 3 had a monophasic course and 1 a relapsing course. Two were tested for anti–myelin oligodendrocyte glycoprotein (anti-MOG) antibody and both were seropositive. This study confirms that neuromyelitis optica spectrum disorder is uncommon in children, and that AQP4-IgG seropositivity is rare. Anti-MOG antibodies should be tested in children with neuromyelitis optica spectrum disorder.


2020 ◽  
Vol 7 (11) ◽  
pp. 2094-2102
Author(s):  
Punchika Kosiyakul ◽  
Sakdipat Songwisit ◽  
Patompong Ungprasert ◽  
Sasitorn Siritho ◽  
Naraporn Prayoonwiwat ◽  
...  

2020 ◽  
Vol 103 (12) ◽  
pp. 1354-1361

Background: Painful tonic spasm (PTS) is a complication frequently observed in patients with neuromyelitis optica spectrum disorder (NMOSD). Objective: To identify the prevalence of PTS, compare the factors associated with the occurrence of PTS, define the characteristics of PTS, and correlate the medication and prognostic factors with good recovery from PTS in patients with NMOSD. Materials and Methods: A retrospective study was performed in patients with definite NMOSD in the Prasat Neurological Institute between January 1, 2014 and December 31, 2018. The prevalence and characteristics of PTS were explored. The characteristics and factors associated with the occurrence of PTS were investigated. Moreover, the factors associated with PTS recovery and pain medications were further analyzed in the present study. Results: The prevalence of PTS in patients with NMOSD was 37.81%. The factors associated with the occurrence of PTS were the presence of acute myelitis (p=0.002, OR 39.00, 95% CI 3.89 to 391.23), and tobacco use (p=0.048, OR 13.38, 95% CI 1.02 to 175.52). In the subgroup analyses of the factors associated with PTS recovery, plasma exchange (p=0.007, OR 24.70, 95% CI 2.43 to 251.57), and Expanded Disability Status Scale range 1.0 to 4.5 (p=0.008, OR 6.92, 95% CI 1.67 to 28.65) were related to the recovery from PTS. While non-recovery was correlated with older age at last visit (p=0.013, OR 1.09, 95% CI 1.02 to 1.17) and longer segments of cord lesions (p=0.016, OR 1.21, 95% CI 1.04 to 1.42). Conclusion: The present study supports that PTS is one of the common complications in patients with NMOSD in Thailand. The presence of acute myelitis and the tobacco use are associated with the presence of PTS. Plasma exchange treatment in the acute phase of NMOSD may be associated with good recovery from PTS, and longer segments of spinal cord lesions is correlated with poor recovery outcomes. The control of these factors may prevent the occurrence of PTS or at least facilitate the recovery from PTS in these patients. Keywords: Neuromyelitis optica spectrum disorder (NMOSD), Painful tonic spasm (PTS)


2016 ◽  
Vol 263 (3) ◽  
pp. 575-582 ◽  
Author(s):  
Ilya Ayzenberg ◽  
◽  
Joanna Schöllhammer ◽  
Robert Hoepner ◽  
Kerstin Hellwig ◽  
...  

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