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.

Neuromyelitis Optica

2017 ◽  
Author(s):  
Teri L. Schreiner ◽  
Jeffrey L. Bennett
Keyword(s):  
Multiple Sclerosis ◽  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Water Channel ◽  
Transverse Myelitis ◽  
Inflammatory Disorder ◽  
The Central Nervous System

Neuromyelitis optica (NMO), or Devic’s disease is an inflammatory disorder of the central nervous system that preferentially affects the optic nerves and spinal cord. Initially considered a variant of multiple sclerosis (MS), NMO is now clearly recognized to have distinct clinical, radiographic, and pathologic characteristics. Historically, the diagnosis of NMO required bilateral optic neuritis and transverse myelitis; however, the identification of a specific biomarker, NMO-IgG, an autoantibody against the aquaporin-4 (AQP4) water channel, has broadened NMO spectrum disease to include patients with diverse clinical and radiographic presentations. This chapter addresses the diagnosis, pathophysiology, and management of the disease.

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.

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.

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 Neuromyelitis optica and multiple sclerosis: Seeing differences through optical coherence tomography

2015 ◽  
Vol 21 (6) ◽  
pp. 678-688 ◽  
Author(s):  
JL Bennett ◽  
J de Seze ◽  
M Lana-Peixoto ◽  
J Palace ◽  
A Waldman ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Optical Coherence Tomography ◽  
Optic Neuritis ◽  
Neuromyelitis Optica ◽  
Water Channel ◽  
Optical Coherence ◽  
Fiber Layer ◽  
Specific Serum ◽  
The Central Nervous System ◽  
Inflammatory Autoimmune Disease

Neuromyelitis optica (NMO) is an inflammatory autoimmune disease of the central nervous system that preferentially targets the optic nerves and spinal cord. The clinical presentation may suggest multiple sclerosis (MS), but a highly specific serum autoantibody against the astrocytic water channel aquaporin-4 present in up to 80% of NMO patients enables distinction from MS. Optic neuritis may occur in either condition resulting in neuro-anatomical retinal changes. Optical coherence tomography (OCT) has become a useful tool for analyzing retinal damage both in MS and NMO. Numerous studies showed that optic neuritis in NMO typically results in more severe retinal nerve fiber layer (RNFL) and ganglion cell layer thinning and more frequent development of microcystic macular edema than in MS. Furthermore, while patients’ RNFL thinning also occurs in the absence of optic neuritis in MS, subclinical damage seems to be rare in NMO. Thus, OCT might be useful in differentiating NMO from MS and serve as an outcome parameter in clinical studies.

The usefulness of brain MRI at onset in the differentiation of multiple sclerosis and seropositive neuromyelitis optica spectrum disorders

2013 ◽  
Vol 20 (6) ◽  
pp. 695-704 ◽  
Author(s):  
So-Young Huh ◽  
Ju-Hong Min ◽  
Woojun Kim ◽  
Su-Hyun Kim ◽  
Ho Jin Kim ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Neuromyelitis Optica ◽  
Lateral Ventricle ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Cortical Lesions ◽  
Magnetic Imaging ◽  
Brain Involvement ◽  
Spectrum Disorders ◽  
Magnetic Resonance Imaging Mri

Background: Although neuromyelitis optica (NMO) is a central nervous system (CNS) autoimmune disease distinct from multiple sclerosis (MS). NMO and NMO spectrum disorder (NMOSD) sometimes show asymptomatic lesions on brain magnetic resonance imaging (MRI) at onset, and even present with symptomatic brain involvement. Objectives: We investigated whether brain MRI at onset can be helpful for the differentiation of MS and NMOSD. Methods: We retrospectively analyzed initial brain MRIs, performed within three months of onset, in patients with MS ( n = 51) and anti-aquaporin4-antibody-positive patients with NMOSD ( n = 67). Results: NMOSD patients met the Paty (37%) and Barkhof (13%) criteria, and the criteria of the European Magnetic Imaging in MS (MAGNIMS) study group (9%), for MS. Ovoid lesions perpendicular to the lateral ventricle, isolated juxtacortical lesions in U-fibers and isolated ovoid/round cortical lesions were found only in MS patients, whereas longitudinal corticospinal tract lesions, extensive hemispheric lesions, periependymal lesions surrounding the lateral ventricle and cervicomedullary lesions were found only in NMOSD patients. Conclusions: Our study suggests that it is difficult to differentiate MS from NMOSD by the fulfillment of the MRI criteria for MS on brain MRI at onset; however, the characteristic morphology of brain lesions is highly useful for the early differentiation of the two disorders.

Brief Overview and Experience of Visual Evoked Potential of First 67 cases at Referral Neuroscience Hospital in Bangladesh

2020 ◽  
Vol 6 (2) ◽  
pp. 74-77
Author(s):  
Mohammad Enayet Hussain ◽  
Bithi Debnath ◽  
AFM Al Masum Khan ◽  
Md Ferdous Mian ◽  
Md Nahidul Islam ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Optic Neuritis ◽  
Clinical Diagnosis ◽  
Optic Neuropathy ◽  
Cross Sectional Study ◽  
P Value ◽  
Pattern Reversal ◽  
Cross Sectional ◽  
The Mean ◽  
Visual Evoked

Background: The visual evoked potentials (VEP) is a valuable tool to document occult lesions of the central visual channels especially within the optic nerve. Objectives: The purpose of the present study was to observe the findings of first few cases of VEP done in the neurophysiology department of the National Institute of Neurosciences (NINS), Dhaka, Bangladesh. Methodology: This cross-sectional study was conducted in the Department of Neurophysiology at the National Institute of Neurosciences and Hospital, Dhaka, Bangladesh from September 2017 to March 2020. All patients referred to the Neurophysiology Department of NINS for VEP were included. Pattern reversal VEPs were done using standard protocol set by International Federation of Clinical Neurophysiology (IFCN). Results: The mean age of the study population was 30.70 (±12.11) years (6-68 years) with 31 (46.3%) male and 36 (53.7%) female patients. The mean duration of illness was 8.71 (±1.78) months (3 days- 120 months). Most common presenting symptom was blurring of vision (37.3%) and dimness of vision (32.8%). Patterned VEP revealed mixed type (both demyelinating and axonal) of abnormality in most cases [29(43.35)]. The most common clinical diagnosis was multiple sclerosis (29.85%) and optic neuropathy (26.87%). In the clinically suspected cases of multiple sclerosis, optic neuropathy and optic neuritis most of the cases of VEP were abnormal and the p value is 0.04 in optic neuropathy and optic neuritis. Conclusion: The commonest presentation of the patients in this series were blurring of vision and dimness of vision. The most common clinical diagnosis for which VEP was asked for, was optic neuritis and multiple sclerosis. Most abnormalities were of mixed pattern (demyelinating and axonal). Journal of National Institute of Neurosciences Bangladesh, 2020;6(2): 74-77

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.

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