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.

Oligoclonal bands in Devic’s neuromyelitis optica and multiple sclerosis: differences in repeated cerebrospinal fluid examinations

2004 ◽  
Vol 10 (1) ◽  
pp. 2-4 ◽  
Author(s):  
R Bergamaschi ◽  
S Tonietti ◽  
D Franciotta ◽  
E Candeloro ◽  
E Tavazzi ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Cerebrospinal Fluid ◽  
Neuromyelitis Optica ◽  
Oligoclonal Bands ◽  
Genetic Pathways ◽  
Devic’S Neuromyelitis Optica

We studied repeated cerebrospinal fluids of patients with Devic’s neuromyelitis optica (NMO) and multiple sclerosis (MS). Variations of oligoclonal bands (OBs) had opposite trends in the two groups. In MS, O Bs were detected in 399 of 411 patients (97%) and never disappeared. In NMO, O Bs were detected in three of 11 patients (27%) and always disappeared. The hypothesis that NMO and MS follow distinct patho genetic pathways is supported by our findings, which can be useful for the differentiatio n of NMO from MS.

scholarly journals Devic’s neuromyelitis optica: a critical review

2008 ◽  
Vol 66 (1) ◽  
pp. 120-138 ◽  
Author(s):  
Marco Aurélio Lana-Peixoto
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Neuromyelitis Optica ◽  
Brain Mri ◽  
Water Channel ◽  
Demyelinating Diseases ◽  
Aquaporin 4 ◽  
Igg Antibody ◽  
Vascular Walls ◽  
Devic’S Neuromyelitis Optica

Devic's neuromyelitis optica (NMO) is an idiopathic inflammatory demyelinating and necrotizing disease characterized by predominant involvement of the optic nerves and spinal cord. In Asian countries relapsing NMO has been known as opticospinal multiple sclerosis. It has long been debated if NMO is a variant of multiple sclerosis (MS) or a distinct disease. Recent studies have shown that NMO has more frequently a relapsing course, and results from attack to aquaporin-4 which is the dominant water channel in the central nervous system, located in foot processes of the astrocytes. Distinctive pathological features of NMO include perivascular deposition of IgG and complement in the perivascular space, granulocyte and eosinophil infiltrates and hyalinization of the vascular walls. These features distinguish NMO from other demyelinating diseases such as MS and acute demyelinating encephalomyelopathy. An IgG-antibody that binds to aquaporin-4, named NMO-IgG has high sensitivity and specificity. Magnetic resonance imaging (MRI) studies have revealed that more frequently there is a long spinal cord lesion that extends through three or more vertebral segments in length. Brain MRI lesions atypical for MS are found in the majority of cases. Treatment in the acute phase includes intravenous steroids and plasma exchange therapy. Immunosupressive agents are recommended for prophylaxis of relapses.

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 Risk of Multiple Sclerosis after Idiopathic Optic Neuritis in a Pakistani Population

2010 ◽  
Vol 37 (2) ◽  
pp. 258-263 ◽  
Author(s):  
Syed Faraz Kazim ◽  
Muhammad Islam ◽  
Mustafa Khan ◽  
Bilal Hameed ◽  
Saad Shafqat
Keyword(s):  
Multiple Sclerosis ◽  
Optic Neuritis ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Oligoclonal Bands ◽  
Cumulative Probability ◽  
South Asian Population ◽  
Increased Risk ◽  
Risk Of Progression ◽  
Csf Oligoclonal Bands

Background and objective:Optic neuritis (ON) is associated with a 38% ten-year risk of developing multiple sclerosis (MS) in Western populations, but the corresponding risk in non-Western populations is unclear. We conducted this study to estimate the risk of progression to MS after an episode of ON in a South Asian population.Methods:Two hundred and fifty-three patients with idiopathic ON were identified by reviewing records of visual evoked potentials and chart notes from a single academic center spanning the years 1990-2007. A structured telephone interview was then conducted to identify patients who had subsequently received a diagnosis of MS. The diagnosis was corroborated from chart notes, where possible. Cumulative probability of conversion to MS was calculated using Kaplan-Meier survival analysis.Results:The five-year risk of developing MS was 14.6% and the ten-year risk was 24%. Patients (N=218) who had one or more typical demyelinating lesions on baseline brain magnetic resonance imaging (MRI) had a 68% 10-year risk; those with no lesions or non-typical lesions had a 14% risk (p<0.001). Female gender, recurrent ON, and occurrence of ON in winter months were also associated with increased risk (p≤ 0.001). Severity of ON and likelihood of detecting cerebrospinal fluid (CSF) oligoclonal bands were higher in patients who developed MS.Conclusion:Idiopathic ON in Pakistan carries a lower risk of progression to MS compared with Western data. As in Western populations, however, presence of abnormal baseline brain MRI and CSF oligoclonal bands correlate with increased MS risk.

Intrathecal synthesis of virus-specific oligoclonal IgG, and of free kappa and free lambda oligoclonal bands in acute monosymptomatic optic neuritis. Comparison with brain MRI

1998 ◽  
Vol 4 (1) ◽  
pp. 22-26 ◽  
Author(s):  
Jette L Frederiksen ◽  
Christian JM Sindic
Keyword(s):  
Multiple Sclerosis ◽  
Optic Neuritis ◽  
Brain Mri ◽  
Population Based ◽  
Oligoclonal Bands ◽  
Igg Antibodies ◽  
Intrathecal Synthesis ◽  
Varicella Zoster ◽  
Definite Multiple Sclerosis ◽  
Oligoclonal Igg

Twenty-seven patients with acute monosymptomatic optic neuritis were randomly selected from a population-based cohort of patients extensively screened for known etiologies of ON. Paired serum and CSF obtained median 20 days from onset were examined for oligoclonal IgG, free kappa and free lambda chains, and virus-specific oligoclonal IgG antibodies by an affinity-mediated capillary blot technique. CSF-restricted oligoclonal IgG bands, free kappa and free lambda chain bands were observed in 17, 15 and nine patients, respectively. In addition, 16 patients showed a polyspecific intrathecal synthesis of oligoclonal IgG antibodies against one or more viruses (12 measles, nine varicella zoster, six rubella, six mumps) compared to all the 18 examined patients with definite multiple sclerosis (P=0.0014). The presence of virus-specific oligoclonal IgG was significantly related to the results of oligoclonal IgG (P=0.0034), free kappa chain bands (P=0.0020), and brain MRI abnormalities (P=0.0402). At 1 year follow-up five patients had developed clinically definite multiple sclerosis; all had virus-specific oligoclonal IgG antibodies, oligoclonal IgG and abnormal MRI at onset.

Predicting multiple sclerosis at optic neuritis onset

2003 ◽  
Vol 9 (2) ◽  
pp. 135-141 ◽  
Author(s):  
Y P Jin ◽  
J de Pedro-Cuesta ◽  
Y H Huang ◽  
M Söderström
Keyword(s):  
Multiple Sclerosis ◽  
Optic Neuritis ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Oligoclonal Bands ◽  
Individual Risk ◽  
Relative Hazard ◽  
Hazard Ratios ◽  
Individual Probability

Using multivariate analyses, individual risk of clinically definite multiple sclerosis (C DMS) after monosymptomatic optic neuritis (MO N) was quantified in a prospective study with clinical MO N onset during 1990 -95 in Stockholm, Sweden. During a mean follow-up time of 3.8 years, the presence of MS-like brain magnetic resonance imaging (MRI) lesions and oligoclonal immunoglobulin (Ig) G bands in cerebrospinal fluid (CSF) were strong prognostic markers of C DMS, with relative hazard ratios of 4.68 {95% confidence interval (CI) 2.21 -9.91} and 5.39 (95% C I 1.56 -18.61), respectively. Age and season of clinical onset were also significant predictors, with relative hazard ratios of 1.76 (95% C I 1.02 -3.04) and 2.21 (95% C I 1.13 -3.98), respectively. Based on the above two strong predicto rs, individual probability of C DMS development after MO N was calculated in a three-quarter sample drawn from a cohort, with completion of follow-up at three years. The highest probability, 0.66 (95% C I 0.48 -0.80), was obtained for individuals presenting with three or more brain MRI lesions and oligoclonal bands in the C SF, and the lowest, 0.09 (95% C I 0.02 -0.32), for those not presenting with these traits. Medium values, 0.29 (95% C I 0.13 -0.53) and 0.32 (95% C I 0.07 -0.73), were obtained for individuals discordant for the presence of brain MRI lesions and oligoclonal bands in the C SF. These predictions were validated in an external one-quarter sample.

First Presentation of an Acquired Demyelinating Syndrome

2017 ◽  
Vol 16 (03) ◽  
pp. 164-170
Author(s):  
Rachel Gottlieb-Smith ◽  
Amy Waldman
Keyword(s):  
Multiple Sclerosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Differential Diagnosis ◽  
Optic Neuritis ◽  
Clinical Features ◽  
Neuromyelitis Optica ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
The Central Nervous System

AbstractAcquired demyelinating syndromes (ADS) present with acute or subacute monofocal or polyfocal neurologic deficits localizing to the central nervous system. The clinical features of distinct ADS have been carefully characterized including optic neuritis, transverse myelitis, and acute disseminated encephalomyelitis. These disorders may all be monophasic disorders. Alternatively, optic neuritis, partial transverse myelitis, and acute disseminated encephalomyelitis may be first presentations of a relapsing or polyphasic neuroinflammatory disorder, such as multiple sclerosis or neuromyelitis optica. The clinical features of these disorders and the differential diagnosis are discussed in this article.

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.

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