Normal-appearing white matter demyelination in neuromyelitis optica spectrum disorder

2017 ◽  
Vol 24 (4) ◽  
pp. 652-658 ◽  
Author(s):  
I. H. Jeong ◽  
J. Y. Choi ◽  
S.-H. Kim ◽  
J.-W. Hyun ◽  
A. Joung ◽  
...  
2016 ◽  
Vol 22 (12) ◽  
pp. 1616-1620 ◽  
Author(s):  
In Hye Jeong ◽  
Joon Yul Choi ◽  
Su-Hyun Kim ◽  
Jae-Won Hyun ◽  
AeRan Joung ◽  
...  

Multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) are inflammatory autoimmune diseases of the central nervous system. We hypothesized that the degree of demyelination within lesions in MS and NMOSD would differ as the pathophysiology of the two diseases do. We used myelin water imaging to compare the myelin water fraction (MWF) in 106 periventricular white matter (PVWM) lesions in 27 MS patients and 51 PVWM lesions in 20 NMOSD patients. The MWF was significantly reduced in the MS compared with the NMOSD lesions, suggesting that myelin loss was more severe in MS than in NMOSD.


2021 ◽  
Vol 3 (2) ◽  
pp. e000174
Author(s):  
Ivo Bekavac ◽  
Matea Matejic ◽  
Riley J Woods ◽  
John I Halloran

ObjectiveTo present a case of two identical twins presenting concurrently with symptoms and subsequent initial diagnosis of neuromyelitis optica spectrum disorder (NMOSD).MethodsClinical, laboratory and MRI findings for both twins were reviewed and presented here.ResultsTwin A presented with right eye pain and subsequent blurred vision in right eye. MRI of the brain and spine demonstrated pre-chiasmal right optic nerve enhancement and T2 hyperintense lesions in the spinal cord at T7 and T9 levels. Cerebrospinal fluid (CSF) analysis was remarkable for NMO/aquaporin-4 (AQP4) fluorescence-activated cell sorting (FACS) titre of 1:32 and a serum NMO/AQP4-IgG positive titre of 1:10 000. Twin B presented with diplopia. MRI of the brain and spine demonstrated T2 hyperintense lesions in the periventricular cerebral white matter, in the periaqueductal white matter of the pons, in the midbrain and the cervical spinal cord. Neurological examination findings revealed incomplete right trochlear palsy, rotatory nystagmus, an incomplete left internuclear ophthalmoplegia and hyper-reflexia. CSF analysis was remarkable for NMO/AQP4 FACS titre of 1:256 and a serum NMO-IgG positive titre of 1:10 000. Both twins responded well to intravenous steroid therapy. There was no adverse environmental exposure present.ConclusionWe present an interesting and rare case of identical twins presenting concurrently and for the first time with NMOSD.


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.


2014 ◽  
Vol 20 (10) ◽  
pp. 1413-1416 ◽  
Author(s):  
Jun Sawada ◽  
Ryosuke Orimoto ◽  
Tatsuro Misu ◽  
Takayuki Katayama ◽  
Hitoshi Aizawa ◽  
...  

A woman with Sjögren syndrome manifesting as aphasia with a left deep cerebral white matter lesion tested positive for anti-aquaporin 4 (AQP4) antibody. Open biopsy of the lesion revealed active demyelination with edematous changes and the preservation of most axons, indicating a non-necrotic demyelinating lesion. Immunostaining for AQP4 was diffusely lost, whereas the loss of glial fibrillary acidic protein immunostaining was limited but with highly degenerated astrocytic foot processes in perivascular areas. These results suggested neuromyelitis optica spectrum disorder (NMOSD) pathology rather than Sjögren-related vasculitis. Only cerebral cortical symptoms with a cerebral white matter lesion could be observed in NMOSDs.


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