Brain abnormalities in Sjogren syndrome with recurrent CNS manifestations: association with neuromyelitis optica

2009 ◽  
Vol 15 (9) ◽  
pp. 1069-1076 ◽  
Author(s):  
JH Min ◽  
HJ Kim ◽  
BJ Kim ◽  
KW Lee ◽  
IN Sunwoo ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Vasogenic Edema ◽  
Brain Mri ◽  
Internal Capsule ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Brain Abnormalities ◽  
Posterior Limb ◽  
Brain Involvement

Background and objectives Optic neuritis or longitudinally extensive myelitis in Sjogren syndrome (SS) suggests a neuromyelitis optica spectrum disorder (NMOSD). However, brain abnormalities of SS remain to be elucidated for the association with neuromyelitis optica (NMO). Methods Twelve primary SS patients (all women, 42 ± 13.2 years) who had recurrent central nervous system (CNS) manifestations with brain involvement were retrospectively identified. Brain MRI, and neurologic and serologic findings were analyzed with the measurement of anti-aquaporin-4 antibody (AQP4-Ab). Results All patients showed brain lesions characteristic of NMO as follows: 1) the involved sites adjacent to the third and fourth ventricles and in the posterior limb of the internal capsule, 2) unique configurations, such as the longitudinal course from the internal capsule to the midbrain, large cerebral or cerebellar lesions over 3 cm, and cavity-like formations. AQP4-Ab was positive in six of eight patients tested, and all the seropositive patients showed lesions with increased diffusion, suggestive of vasogenic edema. Four patients met the revised criteria of NMO, and nine had features of NMOSDs. Of the remaining three patients showing only brain involvement, one had AQP4-Ab. Conclusions This study demonstrates that SS patients with recurrent CNS involvement have brain abnormalities characteristic of NMO and AQP4-Ab in Korea. The presence of AQP4-Ab in one SS patient with only brain involvement may suggest that the coexistence of NMO should be explored in SS patients with recurrent CNS manifestations, even without optic neuritis or myelitis.

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 NMO in pediatric patients: brain involvement and clinical expression

2011 ◽  
Vol 69 (1) ◽  
pp. 34-38 ◽  
Author(s):  
Joaquín A. Peña ◽  
María Elena Ravelo ◽  
Eduardo Mora-La Cruz ◽  
Cecilia Montiel-Nava
Keyword(s):  
Multiple Sclerosis ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Optic Neuropathy ◽  
Pediatric Patients ◽  
Brain Mri ◽  
Water Channel ◽  
Suggested Method ◽  
Clinical Expression ◽  
Brain Involvement

OBJECTIVE: To analyze the clinical, neuroimaging characteristics and positivity of the acquaporin water channel (NMO-IgG) in pediatric patients with neuromyelitis optica (NMO). This disorder could have a variable clinical expression. To address such variability, the term NMO spectrum has been suggested. METHOD: We evaluated six pediatric patients, with a median age of 11 years at the time of the study, with the diagnosis of NMO by the Wingerchuck criteria. RESULTS: All the cases exhibited bilateral optic neuritis (ON). Four patients had abnormalities on brain MRI from the onset,although only three of them developed symptoms correlated to those lesions during the course of their disorder. NMO-IgG was positive in 80%. CONCLUSION: Optic neuropathy is the most impaired feature in NMO patients. Brain MRI lesions are not compatible with multiple sclerosis and positivity of the NMO-IgG are also present in NMO pediatric patients, confirming the heterogeneity in the expression of this disorder.

scholarly journals Seronegative Neuromyelitis Optica: A Case Report of a Hispanic Male

2016 ◽  
Vol 8 (2) ◽  
pp. 102-107 ◽  
Author(s):  
Nabeel Badri ◽  
Mohamed Teleb ◽  
Saad Syed ◽  
Miraie Wardi ◽  
Mateo Porres-Aguilar ◽  
...  
Keyword(s):  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Brain Mri ◽  
Sudden Onset ◽  
Interesting Case ◽  
Imaging Studies ◽  
Thoracic Spinal Cord ◽  
Lower Extremity Weakness ◽  
Thoracic Spinal ◽  
Hispanic Male

Neuromyelitis optica (NMO) is a rare disease, common in white females and rarely reported in Hispanic males. It is usually associated with recurrent demyelinating spectrum that is autoimmune in nature. The diagnosis is usually confirmed by antibody biomarkers; however, they can be negative and lead to more dilemma in diagnosis. Furthermore, the course of disease and prognosis are different in seronegative as compared to seropositive NMO. Treatment is similar in both subgroups with new approaches under investigation for seronegative NMO patients. We present an interesting case of a 37-year-old Hispanic male who presented with sudden onset of lower extremity weakness, numbness, blurry vision, and urinary retention. Magnetic resonance imaging (MRI) of the thoracic spine showed multiphasic demyelinating process involving the thoracic spinal cord. His brain MRI also revealed changes suggesting optic neuritis. The patient met the criteria for diagnosis of NMO by having optic neuritis and myelitis by imaging studies despite having negative aquaporin-4 antibodies (AQP4-Ab). His condition improved after plasma exchange. NMO can be difficult to distinguish from acute multiple sclerosis in the early stages of the disease. Having AQP4-Ab testing is important for diagnosis with imaging studies; however, negative antibody results cannot exclude the diagnosis, but rather group it in seronegative subtype. Ongoing studies and research suggest that seronegative NMO might have a different pathophysiology, manifestation, and prognosis.

Association of Neuromyelitis Optica Spectrum Disease and Sjogren Syndrome in a Tunisian Patient

2021 ◽  
Vol 69 (4) ◽  
pp. 1065
Author(s):  
ThourayaBen Younes ◽  
Malek Mansour ◽  
Wafa Kacem ◽  
Ridha Mrissa
Keyword(s):  
Neuromyelitis Optica ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Tunisian Patient

scholarly journals Brain Abnormalities in Neuromyelitis Optica Spectrum Disorder

2012 ◽  
Vol 2012 ◽  
pp. 1-10 ◽  
Author(s):  
Woojun Kim ◽  
Su-Hyun Kim ◽  
So-Young Huh ◽  
Ho Jin Kim
Keyword(s):  
Neuromyelitis Optica ◽  
Diffusion Tensor ◽  
Imaging Techniques ◽  
Brain Lesions ◽  
Spectrum Disorder ◽  
Brain Abnormalities ◽  
Technical Developments ◽  
New Findings ◽  
The Central Nervous System ◽  
Brain Involvement

Neuromyelitis optica (NMO) is an idiopathic inflammatory syndrome of the central nervous system that is characterized by severe attacks of optic neuritis (ON) and myelitis. Until recently, NMO was considered a disease without brain involvement. However, since the discovery of NMO-IgG/antiaqaporin-4 antibody, the concept of NMO was broadened to NMO spectrum disorder (NMOSD), and brain lesions are commonly recognized. Furthermore, some patients present with brain symptoms as their first manifestation and develop recurrent brain symptoms without ON or myelitis. Brain lesions with characteristic locations and configurations can be helpful in the diagnosis of NMOSD. Due to the growing recognition of brain abnormalities in NMOSD, these have been included in the NMO and NMOSD diagnostic criteria or guidelines. Recent technical developments such as diffusion tensor imaging, MR spectroscopy, and voxel-based morphometry reveal new findings related to brain abnormalities in NMOSD that were not identified using conventional MRI. This paper focuses on the incidence and characteristics of the brain lesions found in NMOSD and the symptoms that they cause. Recent studies using advanced imaging techniques are also introduced.

Signs of MRI affection of brain in сlassical Amyotrophic Lateral Sclerosis patients

2016 ◽  
Vol 7 (2) ◽  
pp. 69-78 ◽  
Author(s):  
Tima R Stuchevskaya ◽  
Dmitriy I Rudenko ◽  
Valery M Kazakov ◽  
Olga F Pozdnyakova ◽  
Alexandr V Pozdnyakov ◽  
...  
Keyword(s):  
Brain Mri ◽  
Disease Onset ◽  
Internal Capsule ◽  
Diagnostic Value ◽  
Cerebral Peduncle ◽  
Focal Lesions ◽  
Duration Of Symptoms ◽  
Posterior Limb ◽  
Cerebral Mri ◽  
Mri Changes

T2 hyperintensive focal lesions were found on brain MRI only in 41 patients (9.7 %) from the ALS group (n = 423). Female were 20 (48,8 %) and male - 21 (51,2 %), aged 31 to 78 years (middle age is 56 ± 4). Focal MRI lesions were found exclusively in projection of the intracranial section of the corticospinal tract. They were always bilateral and symmetrical and were located at one or two levels, most often in the posterior limb of internal capsule and in cerebral peduncle (85 % and 60 % correspondingly). They were less frequent in the radiate crown, semioval center or medulla. There was no reliable correlation between MRI changes and clinical type of ALS, age of disease onset, duration of symptoms and region of onset of muscle weakness and atrophy. Foci of hyperintensive MRI signal in the ALS did not change in terms of time and space, and did not accumulate contrast media with disease progression. This prevents from using routine T1 and T2 mode MRI for monitoring the course of the disease, its prognosis and treatment efficiency. We can recommend cerebral MRI for early diagnostics of ALS and also to rule out other diseases which may resemble ALS. However diagnostic value of 1.5 Tesla MRI is quite limited for diagnostics of classic ALS and its subtypes (PLS and PBP).

Is Devic's neuromyelitis optica a separate disease? A comparative study with multiple sclerosis

2003 ◽  
Vol 9 (5) ◽  
pp. 521-525 ◽  
Author(s):  
J de Seze ◽  
C Lebrun ◽  
T Stojkovic ◽  
D Ferriby ◽  
M Chatel ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Brain Mri ◽  
Oligoclonal Bands ◽  
Time And Space ◽  
Laboratory Findings ◽  
Therapeutic Trials ◽  
The Difference ◽  
Devic’S Neuromyelitis Optica

Devic's neuromyelitis optica (NMO) associates optic neuritis and myelopathy without other neurological signs. Many patients with NMO may be diagnosed as having multiple sclerosis (MS). However, there have been no previous studies comparing these two patho logies and it is still unclear if NMO is a separate entity or a subtype of MS. In the present study, we compared a series of NMO patients with a series of MS patients for whom optic neuritis or myelopathy was the presenting symptom, in order to determine the place of NMO in the spectrum of MS. We retrospectively studied 30 patients diagnosed with NMO and we compared these patients with 50 consecutive MS cases revealed by optic neuritis or acute myelopathy. MS patients were only included if a relapse occurred demonstrating time and space dissemination. We compared the two groups in terms of clinical presentatio n, laboratory findings (MRI and C SF) and clinical outcome. NMO patients were older and more frequently women than MS patients but the difference was not significant. C SF and MRI data were clearly different: oligoclonal bands (O C B) were found in 23% of NMO cases and 88% of MS (P B/0.001), abnormal brain MRI data were observed in 10% of NMO cases and 66% of MS (P B/0.001) and a large spinal cord lesion was observed in 67% of NMO cases and 7.4% of MS cases (P B/0.001). C linical outcome was evaluated as more severe in the NMO group (P B/0.001). O n the basis of clinical data, all NMO patients but three had dissemination in time and space. When we included MRI parameters, only two of the NMO patients met criteria for MS and one of the MS patients met criteria for NMO. O ur study demonstrates that NMO and MS should be considered as two different entities. The respective criteria for NMO and MS were able to distinguish these two patho logies but only when MRI data were applied. This finding could have implications for future therapeutic trials.

Sign in / Sign up

Export Citation Format

Share Document