scholarly journals A case of pathology-proven neuromyelitis optica spectrum disorder with Sjögren syndrome manifesting aphasia and apraxia due to a localized cerebral white matter lesion

2014 ◽  
Vol 20 (10) ◽  
pp. 1413-1416 ◽  
Author(s):  
Jun Sawada ◽  
Ryosuke Orimoto ◽  
Tatsuro Misu ◽  
Takayuki Katayama ◽  
Hitoshi Aizawa ◽  
...  
Keyword(s):  
White Matter ◽  
Neuromyelitis Optica ◽  
White Matter Lesion ◽  
Sjögren Syndrome ◽  
Spectrum Disorder ◽  
Cerebral White Matter ◽  
Sjogren Syndrome ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Aqp4 Antibody ◽  
Cerebral White Matter Lesion

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.

scholarly journals Recurrent optic neuritis in a patient with Sjogren syndrome and neuromyelitis optica spectrum disorder

Medicine ◽  
2020 ◽  
Vol 99 (45) ◽  
pp. e23029
Author(s):  
Wei Zheng ◽  
Xiaoping Liu ◽  
Xiujuan Hou ◽  
Yuelan Zhu ◽  
Taotao Zhang ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Sjögren Syndrome ◽  
Spectrum Disorder ◽  
Sjogren Syndrome ◽  
Neuromyelitis Optica Spectrum Disorder

scholarly journals A case of neuromyelitis optica spectrum disorder associated with a limited cutaneous systemic sclerosis and Sjögren syndrome

2013 ◽  
Vol 53 (9) ◽  
pp. 695-700 ◽  
Author(s):  
Yasutaka Iwanaga ◽  
Shintaro Hayashi ◽  
Nobutoshi Kawamura ◽  
Yasumasa Ohyagi ◽  
Jun-ichi Kira
Keyword(s):  
Systemic Sclerosis ◽  
Neuromyelitis Optica ◽  
Sjögren Syndrome ◽  
Spectrum Disorder ◽  
Sjogren Syndrome ◽  
Neuromyelitis Optica Spectrum Disorder

Neuromyelitis optica spectrum disorder complicated with Sjogren syndrome successfully treated with tocilizumab: A case report

2013 ◽  
Vol 26 (2) ◽  
pp. 294-296 ◽  
Author(s):  
Toshihiko Komai ◽  
Hirofumi Shoda ◽  
Kenichi Yamaguchi ◽  
Keiichi Sakurai ◽  
Mihoko Shibuya ◽  
...  
Keyword(s):  
Case Report ◽  
Neuromyelitis Optica ◽  
Sjögren Syndrome ◽  
Spectrum Disorder ◽  
Sjogren Syndrome ◽  
Neuromyelitis Optica Spectrum Disorder

scholarly journals Simultaneous new onset of neuromyelitis optica spectrum disorder in identical twins

2021 ◽  
Vol 3 (2) ◽  
pp. e000174
Author(s):  
Ivo Bekavac ◽  
Matea Matejic ◽  
Riley J Woods ◽  
John I Halloran
Keyword(s):  
Spinal Cord ◽  
White Matter ◽  
Neuromyelitis Optica ◽  
Cervical Spinal Cord ◽  
Identical Twins ◽  
Spectrum Disorder ◽  
Cerebral White Matter ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Csf Analysis ◽  
The Brain

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.

scholarly journals Neuromyelitis optica spectrum disorder in pediatrics. Case report

Case reports ◽  
2019 ◽  
Vol 5 (1) ◽  
pp. 11-18
Author(s):  
Jhon Camacho ◽  
Sebastian Zuleta ◽  
Maria Paula Alba ◽  
Andrea Hernandez ◽  
Carlos Navas
Keyword(s):  
Neuromyelitis Optica ◽  
Interesting Case ◽  
Spectrum Disorder ◽  
Demyelinating Diseases ◽  
Inflammatory Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
The Central Nervous System ◽  
High Doses ◽  
Aqp4 Antibody ◽  
Timely Treatment

Introduction: Neuromyelitis optica is an inflammatory disorder of the central nervous system that accounts for 5% of demyelinating diseases in pediatrics. Its clinical presentation is variable and associated to the involved area of the central nervous system.Case presentation: This is the case of a 15-year-old patient who consulted several times for nonspecific neurological symptoms. During his last visit to the Clínica Universitaria Colombia in Bogotá, he presented with bilateral optic neuritis, associated with frontal and parietal headache. Immunophenotyping studies were carried out, reporting positive IgG anti-aquaporin 4 antibodies (anti-AQP4 antibody), thus leading to a diagnosis of seropositive neuromyelitis optica spectrum disorder (NMOSD). Management with methylprednisolone pulses was initiated with subsequent outpatient management with rituximab that allowed stabilizing the disease.Discussion: This is an interesting case due to its insidious and uncertain onset in a pediatric patient. It was possible to evaluate clinical and diagnostic differences in relation to its presentation in adults. NMOSD mediated by anti-AQP4 is rare; brain and bone marrow MRI are essential for diagnosis. The treatment of choice for acute conditions consists of high doses of methylprednisolone.Conclusion: This disorder may result in irreversible neurological damage; for this reason, high suspicion is required for early diagnosis and timely treatment.

Eculizumab in the treatment of neuromyelitis optica spectrum disorder

Immunotherapy ◽  
2020 ◽  
Vol 12 (14) ◽  
pp. 1053-1066
Author(s):  
Katrin Giglhuber ◽  
Achim Berthele
Keyword(s):  
Neuromyelitis Optica ◽  
Controlled Trial ◽  
Water Channel ◽  
Spectrum Disorder ◽  
Phase Iii ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Humanized Monoclonal Antibody ◽  
The Complement System ◽  
Terminal Complement ◽  
Aqp4 Antibody

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease of the CNS which is distinct from multiple sclerosis and typically presents with a relapsing course of optic neuritis, myelitis and midline brain inflammatory lesions. In at least two-thirds of cases, antibodies against the water channel AQP4 can be found, which lead to an antibody-mediated activation of the complement system with consecutive damage to neuronal structures. Eculizumab, a humanized monoclonal antibody against the terminal complement component 5, was shown to significantly reduce the risk of NMOSD relapse in a Phase III placebo-controlled trial. Based on this, eculizumab (Soliris®) was the first drug to be formally approved for the treatment of anti-AQP4-antibody positive NMOSD in 2019.

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