Development of extensive brain lesions following fingolimod (FTY720) treatment in a patient with neuromyelitis optica spectrum disorder

2011 ◽  
Vol 18 (1) ◽  
pp. 113-115 ◽  
Author(s):  
Ju-Hong Min ◽  
Byoung Joon Kim ◽  
Kwang Ho Lee

We report the case of a patient who developed extensive brain lesions during fingolimod (FTY720) treatment in the TRANSFORMS study. His initial diagnosis was multiple sclerosis, but after encephalopathy anti-aquaporin4 antibody (anti-AQP4 Ab) was detected, it was changed to neuromyelitis optica spectrum disorder. After treatment with fingolimod, he developed bilateral extensive brain lesions. The brain MRI showed lesions predominantly involving the right frontal and parietal lobes, with vasogenic edema and enhancement. He had residual encephalomalacia and no recurrence with steroid treatment over 3 years following withdrawal of fingolimod.

2018 ◽  
Vol 24 (9) ◽  
pp. 1256-1258 ◽  
Author(s):  
Jonathan Ciron ◽  
Olivier Colin ◽  
Marie-Pierre Rosier ◽  
Soline Lapeyrie ◽  
Damien Biotti ◽  
...  

Brain MRI was originally considered to appear normal in neuromyelitis optica spectrum disorders (NMO-SD). Typical brain lesions are now well described and have been integrated in the latest revision of NMO-SD criteria, but the NMO-SD MRI pattern remains not yet comprehensive. We report here extensive white matter lesions (EWML) mimicking leukodystrophy in a 50-year-old woman with long-lasting anti-AQP4+ NMO-SD. We suggest that EWML could be a possible brain MRI presentation of NMO-SD patients.


2017 ◽  
Vol 9 (2) ◽  
pp. 127-130
Author(s):  
Yoshiaki Takahashi ◽  
Yasuhiro Manabe ◽  
Ryuta Morihara ◽  
Hisashi Narai ◽  
Toru Yamashita ◽  
...  

We report the unusual case of a 63-year-old man with spinocerebellar ataxia (SCA) type 31 who developed neuromyelitis optica spectrum disorder (NMOSD) 14 years after the onset of cerebellar symptoms. In addition to cerebellar atrophy, magnetic resonance imaging showed multiple high-intensity areas in the brain and a long thoracic cord lesion from Th1/2 to Th11. The combination of NMOSD and SCA31 is accidental. However, our case suggests that inflammatory processes could be involved in the pathogenesis of NMOSD and SCA31.


2018 ◽  
Vol 19 ◽  
pp. 73-78 ◽  
Author(s):  
Edgar Carnero Contentti ◽  
Vanessa Daccach Marques ◽  
Ibis Soto de Castillo ◽  
Veronica Tkachuk ◽  
Amilton Antunes Barreira ◽  
...  

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 12 ◽  
pp. 117957352097381
Author(s):  
Braeden D Newton ◽  
Orhun Kantarci ◽  
Darin T Okuda

The recent expansion of the radiological criteria and the use of a highly specific biomarker, anti-aquaporin 4-IgG (AQP4 IgG), has significantly improved the ability of clinicians to provide a timely and accurate diagnosis for neuromyelitis optica spectrum disorder (NMOSD), especially when faced with an abnormal disease presentation. Here, we report on the 5-year clinical experience of a 69-year-old right-handed African American woman who initially presented following symptoms suggestive of transient global amnesia. Her clinical history was only remarkable for a single episode of visual decline with poor recovery experienced 35 years prior, with prior unrevealing serological investigations. Brain MRI features were significant for diffuse, bilateral white matter abnormalities throughout the supratentorial, deep gray matter, and infratentorial regions. Spinal cord imaging studies were within normal limits with no intramedullary high-signal abnormalities identified. Serological studies were significant for the presence of anti-aquaporin 4-IgG. The clinical features were supportive of the diagnosis of NMOSD. The data provided here highlight both the clinical and radiological heterogeneity of NMOSD.


2016 ◽  
Vol 27 (5) ◽  
pp. 2206-2215 ◽  
Author(s):  
Kaspar-Josche Streitberger ◽  
Andreas Fehlner ◽  
Florence Pache ◽  
Anna Lacheta ◽  
Sebastian Papazoglou ◽  
...  

2017 ◽  
Vol 23 (13) ◽  
pp. 1748-1756 ◽  
Author(s):  
Jin Myoung Seok ◽  
Hye-Jin Cho ◽  
Suk-Won Ahn ◽  
Eun Bin Cho ◽  
Min Su Park ◽  
...  

Background: There are currently few studies regarding late-onset neuromyelitis optica spectrum disorder (LO-NMOSD). Objective: We aimed to describe the characteristic features of patients with LO-NMOSD in Korea. Methods: Anti-aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder (NMOSD) from nine tertiary hospitals were reviewed retrospectively. The patients were divided into two groups based on age of onset: LO-NMOSD (⩾50 years of age at onset) versus early-onset neuromyelitis optica spectrum disorder (EO-NMOSD) (<50 years of age at onset). Clinical, laboratory, and magnetic resonance imaging (MRI) parameters were investigated. Results: Among a total of 147 patients (125 female; age of onset, 39.4 ± 15.2 years), 45 patients (30.6%) had an age of onset of more than 50 years. Compared to patients with EO-NMOSD, patients with LO-NMOSD had more frequent isolated spinal cord involvement at onset (64.4% vs 37.2%, p = 0.002), less frequent involvement of the optic nerve (40.0% vs 67.7%, p = 0.002), and less frequent brain MRI lesions (31.1% vs 50.0%, p = 0.034). Furthermore, there was a significant positive correlation between age of onset and Expanded Disability Status Scale (EDSS) score at last follow-up ( r = 0.246, p = 0.003). Conclusion: Age of onset could be an important predictor of lesion location and clinical course of patients with NMOSD.


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