Neuromyelitis optica research

Neuromyelitis optica research

(Devic's disease research)

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Recent Neuromyelitis optica research publications are scanned daily from major neurology journals and updated here"

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NMO research archive July 2008:-

To test or not to test: NMO-IgG and optic neuritis. Posted: 2008-07-01 02:45:36 UTC+01:00 Neurology. 2008 Jun 3; 70(23): 2192-3 Giovannoni G

Relapsing inflammatory opticneuritis: is it neuromyelitis optica? Posted: 2008-07-01 02:45:36 UTC+01:00 Neurology. 2008 May 27; 70(22): 2075-6 de Seze J, Arndt C, Jeanjean L, Zephir H, Blanc F, Labauge P, Bouyon M, Ballonzoli L, Fleury M, Vermersch P, Speeg C

Aquaporin-4 autoantibodies in a paraneoplastic context. Posted: 2008-07-01 02:45:36 UTC+01:00 Arch Neurol. 2008 May; 65(5): 629-32 Pittock SJ, Lennon VA

BACKGROUND: The neuromyelitis optica IgG autoantibody (NMO-IgG) is a validated biomarker for NMO and an emerging spectrum of inflammatory central nervous system-demyelinating disorders. Its antigen is the astrocytic water channel aquaporin-4; NMO-IgG has not been described in a cancer context. OBJECTIVES: To report (1) neurologic and oncologic correlates for patients incidentally identified as NMO-IgG seropositive in a blinded evaluation for paraneoplastic autoantibodies and (2) the frequency of cancer in NMO-IgG-seropositive patients. DESIGN: Observational, retrospective case series. SETTING: Neuroimmunology Laboratory and Neurology Clinical Practice, Mayo Clinic College of Medicine. PATIENTS AND METHODS: From 1998 to 2007, we detected NMO-IgG in 2 patient groups: (1) 31 patients (88% female) identified incidentally among 180 000 patients evaluated for paraneoplastic autoantibodies and (2) 141 patients identified through physician-requested serological evaluation for a suspected NMO-spectrum disorder. RESULTS: In the first group, clinical information was available for 28 patients (90%). An NMO-spectrum disorder was diagnosed in 26 patients (93%), of whom 6 had a neoplasm (5 carcinomas [2 breast, 1 lung, 1 thymic, and 1 uterine cervical] and 1 B-cell lymphoma) and 1 had monoclonal gammopathy. In 4 patients, NMO-related symptoms followed neoplasia detection (median, 14 [range 3-18] months), and in 2 patients, symptoms preceded neoplasia detection (by 5 and 3 months). Two patients had carcinoma (1 breast and 1 lung) without neurological evidence of an NMO-spectrum disorder. In the second group, neoplasms were recorded in 7 seropositive patients (5.0%) with a clinically diagnosed NMO-spectrum disorder: 3 carcinomas (all breast), 1 thyroid H�rthle cell, 1 carcinoid, 1 pituitary somatotropinoma, and 1 B-cell lymphoma. An eighth patient had monoclonal gammopathy. CONCLUSIONS: Aquaporin-4-specific IgG in some cases of NMO may reflect a paraneoplastic immune response. The clinical utility of this autoantibody as a cancer marker warrants prospective investigation.

Interferon-beta increases BAFF levels in multiple sclerosis: implications for B cell autoimmunity. Posted: 2008-07-01 02:45:36 UTC+01:00 Brain. 2008 Jun; 131(Pt 6): 1455-63 Krumbholz M, Faber H, Steinmeyer F, Hoffmann LA, K�mpfel T, Pellkofer H, Derfuss T, Ionescu C, Starck M, Hafner C, Hohlfeld R, Meinl E

B cells are increasingly recognized as major players in multiple sclerosis pathogenesis. The BAFF/APRIL system is crucial for B cell homoeostasis and may drive B cell-dependent autoimmunity. We asked whether this system is affected by Interferon (IFN)-beta therapy. We analysed transcription of the ligands (BAFF, APRIL, TWE-PRIL) and the corresponding receptors (BAFF-R, TACI and BCMA) by TaqMan-PCR ex vivo in whole blood and in immune cell subsets purified from IFN-beta-treated multiple sclerosis patients. Serum BAFF concentrations were determined by ELISA. This cross-sectional study involved 107 donors. IFN-beta therapy strongly induced BAFF transcription proportionally to the IFN-beta biomarker MxA in monocytes and granulocytes in vivo. BAFF serum concentrations were elevated in IFN-beta-treated multiple sclerosis patients to a similar level as observed in SLE patients. In cultured PBMC, neutrophils, fibroblasts and astrocytes, BAFF was induced by IFN-beta concentrations similar to those reached in vivo in treated multiple sclerosis patients. BAFF turned out to be the main regulated element of the BAFF/APRIL system. In untreated multiple sclerosis patients, there was no BAFF increase as compared to healthy controls. Our study reveals a complex situation. We show that IFN-beta therapy induces a potent B cell survival factor, BAFF. However, B cell depletion would be desirable at least in some multiple sclerosis patients. The systemic induction of BAFF by IFN-beta therapy may facilitate the production of various autoantibodies and of IFN-neutralizing antibodies. Individual MS/NMO patients who have major B cell involvement may benefit less than others from IFN-beta therapy, thus explaining interindividual differences of the therapeutic response.

NMO-IgG predicts the outcome of recurrent optic neuritis. Posted: 2008-07-01 02:45:36 UTC+01:00 Neurology. 2008 Jun 3; 70(23): 2197-200 Matiello M, Lennon VA, Jacob A, Pittock SJ, Lucchinetti CF, Wingerchuk DM, Weinshenker BG

OBJECTIVE: To determine the prognostic value of neuromyelitis optica (NMO)-immunoglobulin G (IgG) in patients with recurrent optic neuritis (ON). The aquaporin-4-specific serum autoantibody, NMO-IgG, is a biomarker for NMO and relapsing transverse myelitis. Recurrent ON may herald multiple sclerosis (MS) or NMO, or it may occur as an isolated syndrome. The prognosis and response to therapy differs in each of these contexts. METHODS: We evaluated 34 patients who were tested for NMO-IgG between 2000 and 2007 and who had two or more episodes of ON without satisfying a diagnosis of MS or NMO prior to serologic testing. Clinical data were available for 25 Mayo Clinic patients (5 NMO-IgG positive and 20 NMO-IgG negative) and for an additional 9 seropositive patients whose serum was referred to the Mayo Clinic Neuroimmunology laboratory for testing. RESULTS: Twenty percent of the patients with recurrent ON seen at Mayo Clinic were seropositive. All NMO-IgG-positive patients (vs 65% NMO-IgG-negative patients) had at least one attack with visual acuity in the affected eye worse than 20/200 (p = 0.05). In seropositive patients for whom long-term follow-up was possible (median 8.9 years after the initial ON), 6 of 12 (50%) experienced an episode of myelitis and fulfilled criteria for NMO. In contrast, 1 of 15 seronegative patients (6.7%) fulfilled McDonald criteria for MS (p = 0.03). Seropositive patients had a final visual score which was worse than that of seronegative patients (p = 0.02). CONCLUSIONS: Neuromyelitis optica (NMO)-immunoglobulin G seropositivity predicts poor visual outcome and development of NMO. Seropositive recurrent optic neuritis is a limited form of NMO.

NMO antibody-positive recurrent optic neuritis without clear evidence of transverse myelitis. Posted: 2008-07-01 02:45:36 UTC+01:00 Arch Ophthalmol. 2008 Apr; 126(4): 566-70 Dinkin MJ, Cestari DM, Stein MC, Brass SD, Lessell S

Acute disseminating encephalomyelitis in neuromyelitis optica: closing the floodgates. Posted: 2008-07-01 02:45:36 UTC+01:00 Arch Neurol. 2008 Feb; 65(2): 267-71 Eichel R, Meiner Z, Abramsky O, Gotkine M

OBJECTIVE: To report the clinical and radiological features of 2 patients with neuromyelitis optica (NMO) associated with severe acute disseminating encephalomyelitis. The first patient had anti-aquaporin 4 antibodies (NMO-IgG) but no lesion enhancement, in contrast to the second patient who was seronegative for NMO-IgG but had clear lesion enhancement on magnetic resonance imaging. DESIGN: Clinical, laboratory, and radiological analysis of 10 patients presenting with features compatible with an NMO-spectrum disorder, 2 of whom developed acute disseminating encephalomyelitis. SETTING: Inpatient ward at the Department of Neurology, Hadassah University. PATIENTS: Patients admitted during a 1-year period with features compatible with an NMO-spectrum disorder. INTERVENTIONS: Medical histories and imaging data were reviewed and serum samples were analyzed for the presence of NMO-IgG. MAIN OUTCOME MEASURES: Clinical and paraclinical evidence of brain involvement. RESULTS: Of 10 patients tested, 5 were positive for NMO-IgG. One seropositive and 1 seronegative patient had an acute disseminating encephalomyelitis-like episode. In both cases, the clinical, laboratory, and electroencephalographic findings supported a diagnosis of acute disseminating encephalomyelitis. Magnetic resonance imaging demonstrated extensive bilateral white matter lesions in both patients. Lesions in the seropositive patient were notably lacking in enhancement following gadolinium injection, whereas robust lesion enhancement was observed in the seronegative patient. CONCLUSIONS: Acute disseminating encephalomyelitis without lesion enhancement on magnetic resonance imaging may represent a childhood manifestation of seropositive NMO. The lack of enhancement suggests an intact blood-brain barrier and supports a unique mechanism of edema induction due to dysfunction of water channels.

Finding NMO: neuromyelitis optica in children. Posted: 2008-07-01 02:45:36 UTC+01:00 Neurology. 2008 Jan 29; 70(5): 334-5 Levy M, Birnbaum J, Kerr D

Is neuromyelitis optica eyeing a distinct path from multiple sclerosis? Posted: 2008-07-01 02:45:36 UTC+01:00 Arch Ophthalmol. 2008 Jan; 126(1): 128-9 Glisson CC, Galetta SL

Clinical course of optic neuritis in patients with relapsing neuromyelitis optica. Posted: 2008-07-01 02:45:36 UTC+01:00 Arch Ophthalmol. 2008 Jan; 126(1): 12-6 Papais-Alvarenga RM, Carellos SC, Alvarenga MP, Holander C, Bichara RP, Thuler LC

OBJECTIVE: To describe the clinical characteristics, course, and prognosis of optic neuritis in recurrent neuromyelitis optica. METHODS: We analyzed 60 patients diagnosed using 1999 Mayo Clinic criteria who were seen between 1985 and 2004 at Hospital da Lagoa (Rio de Janeiro, Brazil). RESULTS: Optic neuritis was the initial feature in 53.3% of patients, most with unilateral disease. Recurrent optic neuritis before myelitis occurred in 18.3%. The visual impairment was severe at nadir of the visual index event in 78.3%, with a high remission rate. In the median disease duration of 8 years (range, 0.5-30 years), 380 relapses (118 optic neuritis, 223 myelitis, 39 optic neuritis and myelitis) occurred. At the last follow-up, 53.3% of patients had bilateral visual impairment and 63.3% were blind in at least 1 eye. A high mortality rate (23.3%) was due to cervical myelitis. Mortality rates were significantly higher among Afro Brazilian patients (58.3%). CONCLUSIONS: Optic neuritis in patients with recurrent neuromyelitis optica has a severe and acute onset, with predominantly unilateral lesions followed by improvement of clinical symptoms. In the long-term, the disease leads to severe bilateral visual impairment. Mortality rates are higher among patients of Afro Brazilian descent.

Cognitive functions in neuromyelitis optica. Posted: 2008-07-01 02:45:36 UTC+01:00 Arch Neurol. 2008 Jan; 65(1): 84-8 Blanc F, Z�phir H, Lebrun C, Labauge P, Castelnovo G, Fleury M, Sellal F, Tranchant C, Dujardin K, Vermersch P, de Seze J

BACKGROUND: Neuromyelitis optica (NMO) is characterized by optic neuritis and longitudinally extensive acute transverse myelitis. The brain is generally considered healthy in NMO, though very recent studies have demonstrated that magnetic resonance imaging abnormalities may be observed in various brain regions of NMO patients. To date, cognitive functions have never been investigated in NMO. OBJECTIVE: To investigate cognitive functions in a cohort of 30 patients with NMO. DESIGN: Observational, prospective study. PATIENTS: We studied 30 patients with NMO and compared them with 30 patients with multiple sclerosis and 30 healthy controls matched for age, sex, and educational level. Main Outcome Measure We applied a French translation of the Brief Repeatable Battery of Neuropsychological Tests for Multiple Sclerosis and 3 additional tests. RESULTS: Cognitive performance was significantly lower in the NMO and multiple sclerosis groups than in healthy controls for the 2-second (P< .001) and 3-second (P= .001) Paced Auditory Serial Addition Test, the digit symbol modality test (P= .005), word generation (P= .02), and forward (P= .002) and backward (P= .007) digit span test. We did not observe any difference in test performance between NMO and multiple sclerosis patients. We found no differences between the 3 groups for the other tests. We did not find any correlation between clinical, biological, or magnetic resonance imaging results and cognitive dysfunction. CONCLUSIONS: This study confirms the recent concept of a possible brain involvement in NMO. Additional studies are needed to confirm these initial results and to better understand the mechanisms of such abnormalities.

Neuromyelitis optica and non organ-specific autoimmunity. Posted: 2008-07-01 02:45:36 UTC+01:00 Arch Neurol. 2008 Jan; 65(1): 78-83 Pittock SJ, Lennon VA, de Seze J, Vermersch P, Homburger HA, Wingerchuk DM, Lucchinetti CF, Z�phir H, Moder K, Weinshenker BG

BACKGROUND: Neuromyelitis optica (NMO) is often associated with other clinical or serological markers of non-organ-specific autoimmunity. OBJECTIVE: To evaluate the relationship between NMO spectrum disorders (NMOSDs), including NMO, longitudinally extensive transverse myelitis, and recurrent optic neuritis, and autoimmune disease. We concentrated on the association with systemic lupus erythematosus (SLE), Sj�gren syndrome (SS), or serological evidence of these disorders, which commonly is a source of diagnostic confusion. DESIGN: Retrospective blinded serological survey. SETTING: Mayo Clinic College of Medicine, Rochester, and Centre Hospitalier R�gional Universitaire de Lille. METHODS: Group 1 included 153 US patients with NMOSDs (78 with NMO and 75 with longitudinally extensive transverse myelitis) and 33 control subjects with SS/SLE. Group 2 included 30 French patients with SS/SLE, 14 with NMOSDs (6 with NMO, 6 with longitudinally extensive transverse myelitis, and 2 with recurrent optic neuritis), 16 without NMOSDs, and 4 with NMO without SS/SLE. RESULTS: For group 1, NMO-IgG was detected in 66.7%, antinuclear antibodies in 43.8%, and Sj�gren syndrome A (SSA) antibodies in 15.7% of patients with NMO and longitudinally extensive transverse myelitis. Five NMO-IgG-seropositive patients with NMOSDs had coexisting SLE, SS, or both. Antinuclear antibodies and SSA antibodies were more frequent in NMO-IgG-seropositive patients than in NMO-IgG-seronegative patients (P= .001). For group 2, NMO-IgG was detected in 5 of 14 patients (35.7%) with NMOSDs and SS/SLE and in 2 of 4 patients (50.0%) with NMO without SS/SLE (P= .59). We detected NMO-IgG only in patients with NMOSDs and not in 49 controls with SS/SLE but without optic neuritis or myelitis from the 2 cohorts (P= .01). CONCLUSION: Neuromyelitis optica spectrum disorders with seropositive findings for NMO-IgG occurring with SS/SLE or non-organ-specific autoantibodies is an indication of coexisting NMO rather than a vasculopathic or other complication of SS/SLE.

Neuromyelitis optica-IgG in childhood inflammatory demyelinating CNS disorders. Posted: 2008-07-01 02:45:36 UTC+01:00 Neurology. 2008 Jan 29; 70(5): 344-52 Banwell B, Tenembaum S, Lennon VA, Ursell E, Kennedy J, Bar-Or A, Weinshenker BG, Lucchinetti CF, Pittock SJ

OBJECTIVE: To determine seroprevalence of neuromyelitis optica (NMO)-IgG in childhood CNS inflammatory demyelinating disorders. METHODS: We analyzed demographic, clinical, and radiologic data in a blinded fashion and assessed serum NMO-IgG status for 87 children: 41 with relapsing-remitting multiple sclerosis (RRMS), 17 with NMO, 13 with monophasic/recurrent optic neuritis (ON), 13 with transverse myelitis, of whom 10 were longitudinally extensive on MRI spine (LETM), and another 3 with LETM in the context of acute disseminated encephalomyelitis (ADEM). RESULTS: Ten of the 87 children (11%) were seropositive. Eight of 17 with NMO (47%) were seropositive (7 of 9 with relapsing NMO [78%], 1 of 8 with monophasic NMO [12.5%]). Two other children were seropositive: 1 of 5 with recurrent ON and one child with recurrent LETM. No seropositive case was identified among 41 with RRMS (14% of whom had LETM at some point in their clinical course), 8 with monophasic ON, 9 with monophasic LETM, or 3 with LETM in the context of ADEM. CONCLUSIONS: The similar frequency of neuromyelitis optica (NMO)-IgG in both childhood and adult cases of NMO, and its rarity in relapsing-remitting multiple sclerosis, supports the concept that these diseases have a similar pathogenesis in childhood and adulthood. It is noteworthy that none of nine children with monophasic longitudinally extensive transverse myelitis (LETM) was NMO-IgG-seropositive. Furthermore, LETM does not appear to be as predictive of an NMO spectrum disorder in children as it is in adults. Longitudinal studies of larger pediatric LETM cohorts are required to ascertain whether the absence of NMO-IgG is a negative predictor for relapse in this childhood entity.

More on multiple sclerosis and neuromyelitis optica. Posted: 2008-07-01 02:45:36 UTC+01:00 Arch Neurol. 2007 Dec; 64(12): 1802; author reply 1802-3 Franciotta D, Jarius S, Aloisi F

Pathogenesis of normal-appearing white matter damage in neuromyelitis optica: diffusion-tensor MR imaging. Posted: 2008-07-01 02:45:36 UTC+01:00 Radiology. 2008 Jan; 246(1): 222-8 Yu C, Lin F, Li K, Jiang T, Qin W, Sun H, Chan P

PURPOSE: To prospectively evaluate diffusion indexes of the corticospinal tract (CST), corpus callosum (CC), optic radiation (OR), and cingulum in patients with neuromyelitis optica (NMO) without visible lesions in the brain. MATERIALS AND METHODS: All participants provided informed consent, and the study was approved by the institutional review board. Nineteen patients with NMO (one man, 18 women; mean age, 35.1 years; range, 19-55 years) with normal brain magnetic resonance (MR) imaging findings and 19 sex- and age-matched healthy control subjects were examined with diffusion-tensor MR imaging. The CST, CC, OR, and cingulum were globally and regionally analyzed by using mean diffusivity, fractional anisotropy, and primary (lambda1) and transverse (lambda23) eigenvalues. Correlations of diffusion indexes of the CST and OR with the pyramidal and visual components of the Kurtzke Functional Systems (KFS) and Expanded Disability Status Scale scores were also investigated. Student t testing and Pearson correlation were performed. RESULTS: As compared with values in control subjects, both global and regional analyses showed significant (P < .01) increases in mean diffusivity and lambda23 of the CST and OR but not in any of the diffusion indexes of the CC and cingulum in patients with NMO. In patients with NMO, mean diffusivity (r = 0.556, P = .013) and lambda1 (r = 0.556, P = .013) of the CST were correlated with pyramidal KFS scores, and mean diffusivity (r = 0.523, P = .022) and lambda1 (r = 0.504, P = .027) of the OR were correlated with visual KFS scores. CONCLUSION: Axonal degeneration secondary to lesions in the spinal cord and optic nerves is a cause of normal-appearing white matter damage in NMO. SUPPLEMENTAL MATERIAL: http://radiology.rsnajnls.org/cgi/content/full/2461062075/DC1.

Rehabilitation of neuromyelitis optica (Devic syndrome): three case reports. Posted: 2008-07-01 02:45:36 UTC+01:00 Am J Phys Med Rehabil. 2008 Feb; 87(2): 144-8 Schreiber AL, Fried GW, Formal CS, DeSouza BX

We describe the inpatient clinical rehabilitation course of three patients with neuromyelitis optica (NMO; Devic syndrome). These patients had varying functional deficits. Each patient improved in several functional independence measures (FIM domains) but had minimal to no progress in other domains after acute rehabilitation stays between 1 and 1.5 mos. NMO is a severe central nervous system demyelinating syndrome distinct from MS, characterized by optic neuritis, myelitis, and at least two of three criteria: longitudinally extensive cord lesion, MRI nondiagnostic for multiple sclerosis, or NMO-IgG seropositivity. Persons with NMO may demonstrate improved function with rehabilitation efforts; though gains may be lost to relapse. Future immunomodulatory intervention may augment the benefits of rehabilitation.

Does mitochondrial DNA predispose to neuromyelitis optica (Devic's disease)? Posted: 2008-07-01 02:45:36 UTC+01:00 Brain. 2008 Apr; 131(Pt 4): e93 Hudson G, Mowbray C, Elson JL, Jacob A, Boggild M, Torroni A, Chinnery PF

Pathogenic potential of IgG binding to water channel extracellular domain in neuromyelitis optica. Posted: 2008-07-01 02:45:36 UTC+01:00 Neurology. 2007 Dec 11; 69(24): 2221-31 Hinson SR, Pittock SJ, Lucchinetti CF, Roemer SF, Fryer JP, Kryzer TJ, Lennon VA

BACKGROUND: Autoantibody specific for the aquaporin-4 astrocytic water channel is restricted to serum and CSF of patients with neuromyelitis optica (NMO) and related CNS inflammatory demyelinating disorders (relapsing optic neuritis and longitudinally extensive transverse myelitis). NMO-typical lesions are distinct from MS-typical lesions. Aquaporin-4 is lost selectively at vasculocentric sites of edema/inflammation coinciding with focal deposits of immunoglobulins (Ig) G, M, and terminal complement products, with and without myelin loss. Evidence for antigen-specific autoantibody pathogenicity is lacking. METHODS: We used confocal microscopy and flow cytometry to evaluate the selectivity and immunopathological consequences of Ig binding to surface epitopes of living target cells expressing aquaporin-4 fused at its cytoplasmic N-terminus with GFP. We tested serum, IgG-enriched and IgG-depleted serum fractions, and CSF from patients with NMO, neurologic control patients, and healthy subjects. We also analyzed aquaporin-4 immunoreactivity in myelinated adult mouse optic nerves and spinal cord, and plasma cell Ig isotypes in archived brain tissue from an NMO patient. RESULTS: Serum IgG from patients with NMO binds to the extracellular domain of aquaporin-4; it is predominantly IgG(1), and it initiates two potentially competing outcomes, aquaporin-4 endocytosis/degradation and complement activation. Serum and CSF lack aquaporin-4-specific IgM, and plasma cells in CNS lesions of NMO contain only IgG. Paranodal astrocytic endfeet highly express aquaporin-4. CONCLUSIONS: NMO patients' serum IgG has a selective pathologic effect on cell membranes expressing aquaporin-4. IgG targeting astrocytic processes around nodes of Ranvier could initiate demyelination.

Neuromyelitis optica with brainstem lesion mistaken for brainstem glioma. Case report. Posted: 2008-07-01 02:45:36 UTC+01:00 J Neurosurg. 2007 Sep; 107(3 Suppl): 251-4 Park KY, Ahn JY, Cho JH, Choi YC, Lee KS

Neuromyelitis optica (NMO) is a severe demyelinating syndrome defined principally by its tendency to affect optic nerves and the spinal cord selectively. Asymptomatic brain lesions have recently become a common finding in NMO, and symptomatic brain lesions do not exclude the diagnosis of this entity. The authors describe the case of a 12-year-old girl suffering from an unusually atypical form of NMO in which a brainstem lesion was mistaken for a brainstem glioma. Brainstem involvement in NMO exhibits variable features on neuroimaging and is confused with brainstem glioma in cases of extensive brainstem involvement in childhood. Careful differential diagnosis and proper treatment are vital for a favorable prognosis.

Neuromyelitis optica in a mother and daughter. Posted: 2008-07-01 02:45:36 UTC+01:00 Arch Neurol. 2007 Aug; 64(8): 1189-92 Braley T, Mikol DD

BACKGROUND: Neuromyelitis optica (NMO) is a rare demyelinating disease of the central nervous system that most often results in selective involvement of the optic nerves and spinal cord. Although most cases are sporadic, several familial cases have been reported. All of those patients have been siblings who experienced disease onset at similar ages. To our knowledge, there has not been a documented case between a mother-daughter pair, nor has there been a reported case in which family members developed the disease at different stages of life. OBJECTIVES: To illustrate the clinical courses of NMO in a white mother-daughter pair, which supports a hereditary predisposition to this disorder, as well as to reinforce that onset of disease can occur at different ends of the age spectrum even within the same family. Design, Setting, and Patients Case report of a mother-daughter pair with NMO treated at the University of Michigan Medical Center. RESULTS: After multiple occurrences of optic neuritis and transverse myelitis as well as extensive workups, both mother and daughter were eventually diagnosed with NMO but with different ages at onset, at ages 62 and 29 years, respectively. CONCLUSIONS: Development of NMO is in part genetically influenced. Our familial cases show that NMO may differ clinically within a family. While current diagnostic criteria include an extensive spinal cord lesion, the second case (mother) illustrates that milder cases of NMO might not fulfill that requirement, in which case NMO-IgG antibody seropositivity may confirm the diagnosis.

Water movements in the brain: role of aquaporins.

Trends Neurosci. 2007 Dec 2;

Authors: Tait MJ, Saadoun S, Bell BA, Papadopoulos MC

About 80% of the brain is water. This review discusses the importance of the three brain water-channel proteins (AQP1, AQP4, AQP9) in brain physiology. AQP1 is expressed in the choroid plexus and participates in forming cerebrospinal fluid. AQP4, found in astrocyte foot processes, glia limitans and ependyma, facilitates water movement into and out of the brain, accelerates astrocyte migration and alters neuronal activity. Recently, AQP4 autoantibodies were discovered in patients with neuromyelitis optica, a demyelinating disease, and are now being used to diagnose this condition. AQP9 is present in some glia and neurons, but its function is unclear. Finally, we discuss how the discovery of AQP activators and inhibitors will be the next major step in this field.

Research ID:- Neuromyelitis optica research 18054802 [PubMed - as supplied by publisher] The Accuracy of Prevalence Rates of Multiple Sclerosis: A Critical Review. Related Articles

The Accuracy of Prevalence Rates of Multiple Sclerosis: A Critical Review.

Neuroepidemiology. 2007 Nov 27;29(3-4):150-155

Neuromyelitis optica research. Authors: Poser CM, Brinar VV

Review of the recent Neuromyelitis optica literature raises doubts about the reliability of reported prevalence rates of multiple sclerosis (MS). Many published prevalence rates are inflated. Some studies have shown that relying on clinical information and MRI interpretation leads to one third of incorrect MS diagnoses. The most important error is failing to distinguish between the clinical and MRI characteristics of MS and of disseminated encephalomyelitis (DEM) in both their acute and relapsing forms. The diagnostic criteria in current usage, including those relating to imaging, do not differentiate between MS and other recurrent inflammatory demyelinating diseases of the central nervous system. Considering a second demyelinating episode following a clinically isolated symptom or acute DEM, as confirming MS, is another major source of error. Another is including cases with onset before they entered the study group or moved to the geographic area. Neuromyelitis optica (NMO) has long been considered an MS variant and in Far Eastern countries it is counted as the 'oriental' form of MS, falsely inflating prevalence rates of MS in those areas. Recent immunologic and radiologic evidence shows that at least some NMO cases represent instances of DEM. Copyright (c) 2007 S. Karger AG, Basel.

Research ID:- Neuromyelitis optica research 18042998 [PubMed - as supplied by publisher] Pathogenesis of Normal-appearing White Matter Damage in Neuromyelitis Optica: Diffusion-Tensor MR Imaging. Related Articles

Pathogenesis of Normal-appearing White Matter Damage in Neuromyelitis Optica: Diffusion-Tensor MR Imaging.

Radiology. 2007 Nov 21;

Neuromyelitis optica research. Authors: Yu C, Lin F, Li K, Jiang T, Qin W, Sun H, Chan P

Purpose: To prospectively evaluate diffusion indexes of the corticospinal tract (CST), corpus callosum (CC), optic radiation (OR), and cingulum in patients with neuromyelitis optica (NMO) without visible lesions in the brain. Materials and Methods: All participants provided informed consent, and the study was approved by the institutional review board. Nineteen patients with NMO (one man, 18 women; mean age, 35.1 years; range, 19-55 years) with normal brain magnetic resonance (MR) imaging findings and 19 sex- and age-matched healthy control subjects were examined with diffusion-tensor MR imaging. The CST, CC, OR, and cingulum were globally and regionally analyzed by using mean diffusivity, fractional anisotropy, and primary (lambda(1)) and transverse (lambda(23)) eigenvalues. Correlations of diffusion indexes of the CST and OR with the pyramidal and visual components of the Kurtzke Functional Systems (KFS) and Expanded Disability Status Scale scores were also investigated. Student t testing and Pearson correlation were performed. Results: As compared with values in control subjects, both global and regional analyses showed significant (P < .01) increases in mean diffusivity and lambda(23) of the CST and OR but not in any of the diffusion indexes of the CC and cingulum in patients with NMO. In patients with NMO, mean diffusivity (r = 0.556, P = .013) and lambda(1) (r = 0.556, P = .013) of the CST were correlated with pyramidal KFS scores, and mean diffusivity (r = 0.523, P = .022) and lambda(1) (r = 0.504, P = .027) of the OR were correlated with visual KFS scores. Conclusion: Axonal degeneration secondary to lesions in the spinal cord and optic nerves is a cause of normal-appearing white matter damage in NMO. Supplemental material: http://radiology.rsnajnls.org/cgi/content/full/2461062075/DC1 (c) RSNA, 2007.

Research ID:- Neuromyelitis optica research 18033757 [PubMed - as supplied by publisher] Development of extensive brain lesions following interferon beta therapy in relapsing neuromyelitis optica and longitudinally extensive myelitis. Related Articles

Development of extensive brain lesions following interferon beta therapy in relapsing neuromyelitis optica and longitudinally extensive myelitis.

J Neurol. 2007 Nov 21;

Neuromyelitis optica research. Authors: Shimizu Y, Yokoyama K, Misu T, Takahashi T, Fujihara K, Kikuchi S, Itoyama Y, Iwata M

Research ID:- Neuromyelitis optica research 18004636 [PubMed - as supplied by publisher] Rehabilitation of Neuromyelitis Optica (Devic Syndrome): Three Case Reports. Related Articles

Rehabilitation of Neuromyelitis Optica (Devic Syndrome): Three Case Reports.

Am J Phys Med Rehabil. 2007 Oct 29;

Neuromyelitis optica research. Authors: Schreiber AL, Fried GW, Formal CS, Desouza BX

Schreiber AL, Fried GW, Formal CS, DeSouza BX: Rehabilitation of neuromyelitis optica (Devic syndrome): three case reports. Am J Phys Med Rehabil 2007;86:000-000.We describe the inpatient clinical rehabilitation course of three patients with neuromyelitis optica (NMO; Devic syndrome). These patients had varying functional deficits. Each patient improved in several functional independence measures (FIM domains) but had minimal to no progress in other domains after acute rehabilitation stays between 1 and 1.5 mos. NMO is a severe central nervous system demyelinating syndrome distinct from MS, characterized by optic neuritis, myelitis, and at least two of three criteria: longitudinally extensive cord lesion, MRI nondiagnostic for multiple sclerosis, or NMO-IgG seropositivity. Persons with NMO may demonstrate improved function with rehabilitation efforts; though gains may be lost to relapse. Future immunomodulatory intervention may augment the benefits of rehabilitation.

Research ID:- Neuromyelitis optica research 17993984 [PubMed - as supplied by publisher] Neuromyelitis optica (Devic's disease) in a patient with syphilis. Related Articles

Neuromyelitis optica (Devic's disease) in a patient with syphilis.

Mult Scler. 2007 Nov 6;

Neuromyelitis optica research. Authors: Wilcox R, Burrow J, Slee M, Craig J, Thyagarajan D

The patient initially presented with bilateral optic neuritis and periventricular cranial MRI abnormalities in the context of syphilis. Blood was positive but cerebrospinal fluid testing was negative for specific syphilis markers and he was oligoclonal cerebrospinal fluid (CSF) band negative. He initially responded well to penicillin and corticosteriod treatment, but went on to develop the clinical syndrome of neuromyelitis optica (NMO). Testing for the presence of the serum autoantibody for aquaporin- 4 was negative. This patient appears to represent another case of post-infectious NMO. Possible pathogenesis of this post-syphilis NMO syndrome in the patient is discussed.

Research ID:- Neuromyelitis optica research 17986508 [PubMed - as supplied by publisher] Immune agents for the treatment of Devic's neuromyelitis optica. Related Articles

Immune agents for the treatment of Devic's neuromyelitis optica.

Neurol Sci. 2007 Oct;28(5):238-40

Neuromyelitis optica research. Authors: Bergamaschi R

Research ID:- Neuromyelitis optica research 17972036 [PubMed - in process] Does mitochondrial DNA predispose to neuromyelitis optica (Devic's disease)? Related Articles

Does mitochondrial DNA predispose to neuromyelitis optica (Devic's disease)?

Brain. 2007 Oct 29;

Neuromyelitis optica research. Authors: Hudson G, Mowbray C, Elson JL, Jacob A, Boggild M, Torroni A, Chinnery PF

Research ID:- Neuromyelitis optica research 17967805 [PubMed - as supplied by publisher] Devic's neuromyelitis optica: clinical and imaging findings. Related Articles

Devic's neuromyelitis optica: clinical and imaging findings.

JBR-BTR. 2007 Jul-Aug;90(4):284-7

Neuromyelitis optica research. Authors: Vandevyver V, Lemmerling M, De Potter R, Verstraete K

Devic's neuromyelitis optica is an uncommon but severe monophasic or relapsing demyelinating disease. Current evidence supports the concept that it is a distinct disorder from multiple sclerosis, with some specific imaging characteristics. The aim of this paper is to report the clinical and imaging features of this rare entity and to discuss its differential diagnosis.

Research ID:- Neuromyelitis optica research 17966247 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Pathogenic potential of IgG binding to water channel extracellular domain in neuromyelitis optica. Related Articles

Pathogenic potential of IgG binding to water channel extracellular domain in neuromyelitis optica.

Neurology. 2007 Oct 10;

Neuromyelitis optica research. Authors: Hinson SR, Pittock SJ, Lucchinetti CF, Roemer SF, Fryer JP, Kryzer TJ, Lennon VA

BACKGROUND: Autoantibody specific for the aquaporin-4 astrocytic water channel is restricted to serum and CSF of patients with neuromyelitis optica (NMO) and related CNS inflammatory demyelinating disorders (relapsing optic neuritis and longitudinally extensive transverse myelitis). NMO-typical lesions are distinct from MS-typical lesions. Aquaporin-4 is lost selectively at vasculocentric sites of edema/inflammation coinciding with focal deposits of immunoglobulins (Ig) G, M, and terminal complement products, with and without myelin loss. Evidence for antigen-specific autoantibody pathogenicity is lacking. METHODS: We used confocal microscopy and flow cytometry to evaluate the selectivity and immunopathological consequences of Ig binding to surface epitopes of living target cells expressing aquaporin-4 fused at its cytoplasmic N-terminus with GFP. We tested serum, IgG-enriched and IgG-depleted serum fractions, and CSF from patients with NMO, neurologic control patients, and healthy subjects. We also analyzed aquaporin-4 immunoreactivity in myelinated adult mouse optic nerves and spinal cord, and plasma cell Ig isotypes in archived brain tissue from an NMO patient. RESULTS: Serum IgG from patients with NMO binds to the extracellular domain of aquaporin-4; it is predominantly IgG1, and it initiates two potentially competing outcomes, aquaporin-4 endocytosis/degradation and complement activation. Serum and CSF lack aquaporin-4-specific IgM, and plasma cells in CNS lesions of NMO contain only IgG. Paranodal astrocytic endfeet highly express aquaporin-4. CONCLUSIONS: NMO patients' serum IgG has a selective pathologic effect on cell membranes expressing aquaporin-4. IgG targeting astrocytic processes around nodes of Ranvier could initiate demyelination.

Research ID:- Neuromyelitis optica research 17928579 [PubMed - as supplied by publisher] Neuromyelitis optica with brainstem lesion mistaken for brainstem glioma. Case report. Related Articles

Neuromyelitis optica with brainstem lesion mistaken for brainstem glioma. Case report.

J Neurosurg. 2007 Sep;107(3 Suppl):251-4

Neuromyelitis optica research. Authors: Park KY, Ahn JY, Cho JH, Choi YC, Lee KS

Neuromyelitis optica (NMO) is a severe demyelinating syndrome defined principally by its tendency to affect optic nerves and the spinal cord selectively. Asymptomatic brain lesions have recently become a common finding in NMO, and symptomatic brain lesions do not exclude the diagnosis of this entity. The Neuromyelitis optica research. Authors describe the case of a 12-year-old girl suffering from an unusually atypical form of NMO in which a brainstem lesion was mistaken for a brainstem glioma. Brainstem involvement in NMO exhibits variable features on neuroimaging and is confused with brainstem glioma in cases of extensive brainstem involvement in childhood. Careful differential diagnosis and proper treatment are vital for a favorable prognosis.

Research ID:- Neuromyelitis optica research 17918537 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Intermittent intravenous immunoglobulin successfully prevents relapses of neuromyelitis optica. Related Articles

Intermittent intravenous immunoglobulin successfully prevents relapses of neuromyelitis optica.

Intern Med. 2007;46(19):1671-2

Neuromyelitis optica research. Authors: Okada K, Tsuji S, Tanaka K

Research ID:- Neuromyelitis optica research 17917332 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Neuromyelitis optica in a patient with an early onset demyelinating episode: clinical and autoantibody findings. Related Articles

Neuromyelitis optica in a patient with an early onset demyelinating episode: clinical and autoantibody findings.

Clin Neurol Neurosurg. 2007 Dec;109(10):926-30

Neuromyelitis optica research. Authors: Beyer AM, Wandinger KP, Siebert E, Zschenderlein R, Klehmet J

Recent clinical and laboratory findings have substantially advanced our understanding of neuromyelitis optica (NMO) as a humorally mediated, autoimmune disorder. We report on a patient who suffered a first episode of transverse myelitis at the age of 6 months following diphtheria-pertussis-tetanus (DPT) vaccination which had therefore been considered suggestive of acute disseminated encephalomyelitis (ADEM). Fifteen years later, the further disease course revealed typical NMO meeting all diagnostic criteria. This development points to a broad clinical and temporal heterogeneity of NMO, with ADEM probably occurring in the context of a shared autoimmune diathesis. Despite therapy response following B-cell depletion by rituximab, positive NMO-IgG autoantibody status remained unchanged, whereas direct testing for anti-aquaporin-4 (AQP-4)-antibodies was negative throughout. Our findings challenge the pathogenic relevance of NMO-IgG and indicate a varying diagnostic value of testing for NMO-IgG and AQP-4-autoantibodies.

Research ID:- Neuromyelitis optica research 17913344 [PubMed - in process] Devic's neuromyelitis optica in an infant case. Related Articles

Devic's neuromyelitis optica in an infant case.

J Child Neurol. 2007 Sep;22(9):1143-6

Neuromyelitis optica research. Authors: Yuksel D, Senbil N, Yilmaz D, Yavuz Gurer YK

Devic's neuromyelitis optica was orginally described as an acute severe monophasic syndrome characterised by myelitis and optic neuritis. The mean age at onset was reported to be around 40 years, with a wide range. However, Devic's neuromyelitis optica has also been seen in children. Prognosis of the syndrome was poor, and no satisfactory treatment was known. This article reports a 23-month-old boy with acute myelitis and optic neuritis who was diagnosed with Devic's neuromyelitis optica. The response of the patient to therapy was poor, and he developed severe sequelae.

Research ID:- Neuromyelitis optica research 17890418 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] No evidence of misdiagnosis in patients with multiple sclerosis and repeated positive anticardiolipin antibody testing based on magnetic resonance imaging and long term follow-up. Related Articles

No evidence of misdiagnosis in patients with multiple sclerosis and repeated positive anticardiolipin antibody testing based on magnetic resonance imaging and long term follow-up.

J Neurol Neurosurg Psychiatry. 2007 Oct;78(10):1146-8

Neuromyelitis optica research. Authors: Liedorp M, Sanchez E, van Hoogstraten IM, von Blomberg BM, Barkhof F, Polman CH, Killestein J

OBJECTIVE: To determine whether patients with definite multiple sclerosis (MS) and repeated positive anticardiolipin antibody (aCL Ab) testing fulfil the recently updated criteria for the antiphospholipid syndrome (APS). Also, to determine if these patients form a separate subgroup in terms of long term follow-up and MRI characteristics. DESIGN: A blinded case control study comparing MRI patterns between aCL Ab positive and negative MS patients with a clinical follow-up of 7 years. PARTICIPANTS: 8 (5.6%; male:female ratio 2:6; 6 relapsing-remitting subtype, 1 primary progressive subtype and 1 neuromyelitis optica (NMO)) of 143 consecutive patients with definite MS or NMO (71% relapsing-remitting, 18% secondary progressive and 6% primary progressive disease course; 4% NMO) showed repeated positive aCL Ab testing. SETTING: Outpatient clinic of a tertiary MS centre in The Netherlands. RESULTS: All eight aCL Ab positive patients had levels below 40 MPL/GPL units, with the majority of intervals between tests of at least 12 weeks. After follow-up, none of the patients fulfilled the criteria for APS. No specific MRI features were present compared with 24 matched aCL Ab negative patients. CONCLUSIONS: No aCL Ab positive MS patient fulfilled the criteria for APS, arguing against a possible misdiagnosis or coexistence.

Research ID:- Neuromyelitis optica research 17878195 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Disseminated encephalomyelitis and multiple sclerosis: two different diseases - a critical review. Related Articles

Disseminated encephalomyelitis and multiple sclerosis: two different diseases - a critical review.

Acta Neurol Scand. 2007 Oct;116(4):201-6

Neuromyelitis optica research. Authors: Poser CM, Brinar VV

The practice of initiating immunomodulatory treatment immediately after a clinically isolated syndrome (CIS) suggestive of multiple sclerosis (MS) emphasizes the need to distinguish between disseminated encephalomyelitis (DEM) and MS. Their clinical, genetic, imaging, and histopathological characteristics establish that they are distinct disease entities. Acute and recurrent DEM are more common in children, but also occur in adults. DEM is polysymptomatic and includes signs and symptoms rarely encountered in MS, such as fever, alterations of the state of consciousness, cognitive and aphasic symptoms, and meningism. Cerebrospinal oligoclonal bands are rare. Magnetic resonance imaging (MRI) is the best means of distinguishing between DEM and MS. In the former, the lesion load is heavy, thalamus or basal ganglia are often affected, and early in the disease most of the lesions are usually larger than those of MS and enhance with gadolinium. The MRI spinal cord lesions are longer than three vertebral segments, and define neuromyelitis optica (NMO). Antibodies against aquaporin-4 are present in some NMO, but are also found in cases of MS and DEM. Most NMO are forms of DEM, not MS, and are identical with the 'Oriental' or 'optico-spinal' form of MS.

Research ID:- Neuromyelitis optica research 17824894 [PubMed - in process] Primary Sj�gren's syndrome mimicking neuromyelitis optica. Related Articles

Primary Sj�gren's syndrome mimicking neuromyelitis optica.

Eur J Intern Med. 2007 Oct;18(6):507

Neuromyelitis optica research. Authors: Barroso B

Research ID:- Neuromyelitis optica research 17822665 [PubMed - in process] Relapsing-remitting central nervous system disease in a young child. Related Articles

Relapsing-remitting central nervous system disease in a young child.

Rev Neurol Dis. 2005;2(4):215-6, 220-2

Neuromyelitis optica research. Authors: Fellman DM, Cook SD

Research ID:- Neuromyelitis optica research 17768787 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] The spectrum of neuromyelitis optica. Related Articles

The spectrum of neuromyelitis optica.

Lancet Neurol. 2007 Sep;6(9):805-15

Neuromyelitis optica research. Authors: Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG

Neuromyelitis optica (also known as Devic's disease) is an idiopathic, severe, demyelinating disease of the central nervous system that preferentially affects the optic nerve and spinal cord. Neuromyelitis optica has a worldwide distribution, poor prognosis, and has long been thought of as a variant of multiple sclerosis; however, clinical, laboratory, immunological, and pathological characteristics that distinguish it from multiple sclerosis are now recognised. The presence of a highly specific serum autoantibody marker (NMO-IgG) further differentiates neuromyelitis optica from multiple sclerosis and has helped to define a neuromyelitis optica spectrum of disorders. NMO-IgG reacts with the water channel aquaporin 4. Data suggest that autoantibodies to aquaporin 4 derived from peripheral B cells cause the activation of complement, inflammatory demyelination, and necrosis that is seen in neuromyelitis optica. The knowledge gained from further assessment of the exact role of NMO-IgG in the pathogenesis of neuromyelitis optica will provide a foundation for rational therapeutic trials for this rapidly disabling disease.

Research ID:- Neuromyelitis optica research 17706564 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Anti-aquaporin 4 antibody in Japanese multiple sclerosis: the presence of optic spinal multiple sclerosis without long spinal cord lesions and anti-aquaporin 4 antibody. Related Articles

Anti-aquaporin 4 antibody in Japanese multiple sclerosis: the presence of optic spinal multiple sclerosis without long spinal cord lesions and anti-aquaporin 4 antibody.

J Neurol Neurosurg Psychiatry. 2007 Sep;78(9):990-2

Neuromyelitis optica research. Authors: Tanaka M, Tanaka K, Komori M, Saida T

BACKGROUND: Anti-aquaporin 4 (AQP4) antibodies were found in patients with neuromyelitis optica (NMO) and Japanese optic-spinal multiple sclerosis (OSMS). OBJECTIVE: To review the clinical features and investigate anti-AQP4 antibodies of Japanese patients with multiple sclerosis (MS), with or without long spinal cord lesions (LCL). METHODS: Anti-AQP4 antibodies were examined in the sera of 128 consecutive Japanese patients by the immunofluorescence method using AQP4 transfected cells. RESULTS: The 45 LCL-MS patients included 28 with a long spinal cord lesion extending contiguously over three vertebral segments on sagittal T2 weighted images (long T2 lesion) and 17 with segmental cord atrophy extending more than three vertebral segments. We identified 25 patients with anti-AQP4 antibody with LCL and anti-AQP4 antibody. Anti-AQP4 antibody was found in 12/17 (70.6%) LCL-MS patients with segmental cord atrophy, and in 13/28 (46.4%) LCL-MS patients without segmental long cord atrophy (p = 0.135, Fisher's exact test). Seropositive MS patients with LCL had more relapses than seronegative patients (p = 0.0004, Mann-Whitney U test). 9 patients with OSMS were negative for anti-AQP4 antibody who did not show LCL. CONCLUSION: These results suggest that an anti-AQP4 antibody is found not only in MS patients with long T2 lesions but also in patients with segmental cord atrophy extending more than three vertebral segments. It is a marker of LCL-MS showing frequent exacerbations. Japanese OSMS cases comprised those that were identical to NMO cases and those that were more closely related to classic MS.

Research ID:- Neuromyelitis optica research 17702782 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Neuromyelitis optica in a mother and daughter. Related Articles

Neuromyelitis optica in a mother and daughter.

Arch Neurol. 2007 Aug;64(8):1189-92

Neuromyelitis optica research. Authors: Braley T, Mikol DD

Research ID:- Neuromyelitis optica research 17698711 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Variable responses to rituximab treatment in neuromyelitis optica (Devic's disease). Related Articles

Variable responses to rituximab treatment in neuromyelitis optica (Devic's disease).

Neurol Sci. 2007 Aug;28(4):209-11

Neuromyelitis optica research. Authors: Capobianco M, Malucchi S, di Sapio A, Gilli F, Sala A, Bottero R, Marnetto F, Doriguzzi Bozzo C, Bertolotto A

We have described two cases of Devic's disease patients treated with rituximab with different outcomes. The results indicate that there may be early unresponsiveness in very aggressive cases. Well designed clinical trials are needed to assess treatment effects in such a rare disease.

Research ID:- Neuromyelitis optica research 17690854 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Migration and multiple sclerosis: the French West Indies experience. Related Articles

Migration and multiple sclerosis: the French West Indies experience.

J Neurol Sci. 2007 Nov 15;262(1-2):117-21

Neuromyelitis optica research. Authors: Cabre P

The French West Indies (FWI), i.e., the islands of Martinique and Guadeloupe, have recently experienced the emergence of multiple sclerosis (MS). This epidemiological upheaval followed a return migration of the FWI population that had previously migrated to continental France. The prevalence MS was 14.8/10(5) (95% CI: 11.9-17.7) on Dec. 31, 1999 and its mean annual incidence was 1.4/10(5) (95% CI: 1.0-1.8) for the period July 1997 to June 2002. The prevalence of MS in Martinique, that received more return migration, is higher than that of Guadeloupe (21.0/10(5) vs. 8.5/10(5)). This emergence of MS has been accompanied also by an inversion of its clinical spectrum, with recurrent neuromyelitis optica accounting for only 17.8% of cases. The standardized ratio of the incidence of MS among migrants is 1.71 (95% CI: 1.19-2.38; P<0.01) and if migration to continental France occurred before the age of 15 it is 4.05 (95% CI: 2.17-6.83; P<0.0001). According to recent data, a drastic reduction in exposure to sunlight and to intestinal parasites during childhood, found preferentially among migrants, are possible environmental factors responsible for this emergence.

Research ID:- Neuromyelitis optica research 17651756 [PubMed - in process] Low-dose corticosteroids reduce relapses in neuromyelitis optica: a retrospective analysis. Related Articles

Low-dose corticosteroids reduce relapses in neuromyelitis optica: a retrospective analysis.

Mult Scler. 2007 Sep;13(8):968-74

Neuromyelitis optica research. Authors: Watanabe S, Misu T, Miyazawa I, Nakashima I, Shiga Y, Fujihara K, Itoyama Y

Neuromyelitis optica (NMO) is a relapsing neurologic disease characterized by severe optic neuritis and transverse myelitis. A disease-modifying therapy for NMO has not been established. We retrospectively analysed the effect of low-dose corticosteroid (CS) monotherapy on the annual relapse rate in nine patients with NMO. We divided the clinical course in each patient into two periods; the CS Period in which CS was administered, and the No CS Period in which CS was not administered. Periods related to other immunological therapies, such as high-dose methylprednisolone, immunosuppressants, interferon-beta, and plasma exchange, were excluded. As a result, the annual relapse rate during the CS Periods [median, 0.49 (range, 0-1.31)] was found to be significantly lower than that during the No CS Periods [1.48 (0.65-5.54)]. As for the dose of CS, relapses occurred significantly more frequently with ;10 mg/day or less' than with ;over 10 mg/day' (odds ratio: 8.75). The results of the present study suggest a beneficial effect of low-dose CS monotherapy in reducing relapses in NMO.

Research ID:- Neuromyelitis optica research 17623727 [PubMed - in process] Recent news on MS: natalizumab is beneficial, stem cell transplantation is not, and the differentiation of neuromyelitis optica and MS is complex. Related Articles

Recent news on MS: natalizumab is beneficial, stem cell transplantation is not, and the differentiation of neuromyelitis optica and MS is complex.

J Neurol. 2007 Jun;254(6):820-822

Neuromyelitis optica research. Authors: Strupp M

Research ID:- Neuromyelitis optica research 17593469 [PubMed - as supplied by publisher] Is neuromyelitis optica a distinct entity? Related Articles

Is neuromyelitis optica a distinct entity?

Arch Neurol. 2007 Jun;64(6):906

Neuromyelitis optica research. Authors: Roach ES

Research ID:- Neuromyelitis optica research 17562945 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Is neuromyelitis optica distinct from multiple sclerosis?: something for "lumpers" and "splitters".

Is neuromyelitis optica distinct from multiple sclerosis?: something for "lumpers" and "splitters".

Arch Neurol. 2007 Jun;64(6):903-5

Neuromyelitis optica research. Authors: Frohman EM, Kerr D

Research ID:- Neuromyelitis optica research 17562944 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Neuromyelitis optica is a variant of multiple sclerosis. Related Articles

Neuromyelitis optica is a variant of multiple sclerosis.

Arch Neurol. 2007 Jun;64(6):901-3

Neuromyelitis optica research. Authors: Galetta SL, Bennett J

Research ID:- Neuromyelitis optica research 17562943 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Neuromyelitis optica is distinct from multiple sclerosis.

Neuromyelitis optica is distinct from multiple sclerosis.

Arch Neurol. 2007 Jun;64(6):899-901

Neuromyelitis optica research. Authors: Weinshenker BG

Research ID:- Neuromyelitis optica research 17562942 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Gastroparesis in multiple sclerosis. Related Articles

Gastroparesis in multiple sclerosis.

Mult Scler. 2007 Jun;13(5):686

Neuromyelitis optica research. Authors: Jacob A, Pittock SJ

Research ID:- Neuromyelitis optica research 17548454 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Neuromyelitis optica with clinical and histopathological involvement of the brain. Related Articles

Neuromyelitis optica with clinical and histopathological involvement of the brain.

Mult Scler. 2007 Jun;13(5):679-82

Neuromyelitis optica research. Authors: Hengstman GJ, Wesseling P, Frenken CW, Jongen PJ

Diagnostic criteria for neuromyelitis optica (NMO) state that there should be no active disease outside the optic nerves and spinal cord. However, several cases have been described with symptomatic brain involvement. We describe an autopsy case of a patient with NMO and symptomatic involvement of the brain. The histopathology of the brain lesions is typical for NMO, with extensive macrophage infiltration, including perivascular accumulation of large numbers of eosinophils. This is the first case that clearly shows that in NMO, the histopathology of the brain lesions is identical to that of the lesions in the optic nerves and spinal cord.

Research ID:- Neuromyelitis optica research 17548452 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Optic neuritis. Related Articles

Optic neuritis.

Compr Ophthalmol Update. 2007 Mar-Apr;8(2):67-75; discussion 77-8

Neuromyelitis optica research. Authors: Vaphiades MS, Kline LB

The term optic neuritis denotes primary inflammation of the optic nerve. When the clinical history and examination suggest optic neuritis and the optic disk appears normal, the term retrobulbar optic neuritis is used. Critical elements in establishing the diagnosis are a detailed history and an accurate examination. For this reason, the ophthalmologist must be familiar with the clinical profile of optic neuritis.

Research ID:- Neuromyelitis optica research 17540123 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Relapsing neuromyelitis optica responsive to glatiramer acetate treatment. Related Articles

Relapsing neuromyelitis optica responsive to glatiramer acetate treatment.

Eur J Neurol. 2007 Jun;14(6):e12-3

Neuromyelitis optica research. Authors: Gartzen K, Limmroth V, Putzki N

Research ID:- Neuromyelitis optica research 17539924 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Neuromyelitis optica: new findings on pathogenesis. Related Articles

Neuromyelitis optica: new findings on pathogenesis.

Int Rev Neurobiol. 2007;79:665-88

Neuromyelitis optica research. Authors: Wingerchuk DM

Neuromyelitis optica (NMO) is an idiopathic CNS demyelinating disorder that preferentially involves the optic nerve and spinal cord. Diverse sources of evidence support the hypothesis that NMO is distinct from classical multiple sclerosis (MS) and that the pathogenesis of NMO is dominated by humoral mechanisms. Such evidence includes clinical observations that systemic autoimmune diseases often coexist with NMO and that therapeutic plasmapheresis may provide meaningful rescue therapy for severe clinical attacks, immunopathologic studies that demonstrate prominent complement activation and immunoglobulin deposition, and the discovery of the serum autoantibody NMO-IgG, a potential NMO biomarker that targets aquaporin-4 (AQP4). The NMO-IgG marker is present in a majority of patients with "NMO-spectrum disorders," including isolated or recurrent longitudinally extensive transverse myelitis, recurrent optic neuritis with negative brain imaging, and the Asian optic-spinal form of MS. Preliminary experiments demonstrate that NMO-IgG can modulate AQP4 function and fix complement, characteristics that suggest it has the potential to be pathogenic in NMO. Other immunologic differences among NMO, NMO-spectrum disorders, and classical MS are reviewed.

Research ID:- Neuromyelitis optica research 17531863 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Differential diagnosis of multiple sclerosis. Related Articles

Differential diagnosis of multiple sclerosis.

Int Rev Neurobiol. 2007;79:393-422

Neuromyelitis optica research. Authors: Fadil H, Kelley RE, Gonzalez-Toledo E

There are a number of illnesses that can mimic multiple sclerosis (MS). This pretty much includes any pathological process that can reflect injury to the central nervous system either in a transient or progressive basis. Typically, MS presents itself in individuals in their teens up to their late 30s. On occasion, however, one can see MS present in patients in their 60s. However, in retrospect, many of these patients might have had subtle manifestations of MS in their younger years. Visual obscuration or visual loss can be a manifestation of retinal ischemia, retinal migraine, or optic neuritis which might or might not evolve into a clinical picture compatible with MS. Cranial neuropathy, long tract signs, sensory disturbance, and/or gait ataxia can be related to a number of different processes such as illicit drug use, neurosarcoidosis, neuro-Behcet's disease, neuroborreliosis, HIV-related disease, neurosyphilis, vascular occlusive disease including vasculitis, connective tissue disorders, acute disseminated encephalomyelitis (ADEM), idiopathic transverse myelitis, neuromyelitis optica (NMO), or tropical spastic paraparesis. In addition, a constellation of symptoms, with questionable objective findings, along with normal MRI imaging, normal CSF results, and normal evoked response testing, when indicated, might identify a conversion disorder or possibly malingering. There are now established criteria for the diagnosis of MS, but initial presentations can be less than "textbook" in nature. With the advent of immunomodulating therapy, it has become more important to diagnose MS more effectively earlier on in the course of the illness. Prior to specific therapy for MS, astute clinicians did not necessarily move with alacrity to establish the diagnosis in patients with subtle or transient manifestations. This was in recognition of the fact that little could be offered to alter the course of the illness and a number of patients might never experience further problems if they were lucky enough to have their illness go into permanent remission after one minor exacerbation.

Research ID:- Neuromyelitis optica research 17531852 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Relapsing neuromyelitis optica and relapsing-remitting multiple sclerosis: differentiation at diffusion-tensor MR imaging of corpus callosum. Related Articles

Relapsing neuromyelitis optica and relapsing-remitting multiple sclerosis: differentiation at diffusion-tensor MR imaging of corpus callosum.

Radiology. 2007 Jul;244(1):249-56

Neuromyelitis optica research. Authors: Yu CS, Zhu CZ, Li KC, Xuan Y, Qin W, Sun H, Chan P

PURPOSE: To prospectively assess sensitivity and specificity of diffusion indexes of the corpus callosum (CC) for differentiating relapsing neuromyelitis optica (RNMO) from relapsing-remitting multiple sclerosis (RRMS), by using final clinical diagnosis as the reference standard. MATERIALS AND METHODS: Participants provided informed consent; the study was approved by the institutional review board. Forty-six consecutive patients with RRMS (18 men, 28 women; mean age, 37.7 years; range, 18-58 years) and 26 consecutive patients with RNMO (two men, 24 women; mean age, 38.6 years; range, 19-59 years) underwent diffusion-tensor magnetic resonance imaging. Mean diffusivity (MD) and fractional anisotropy (FA) of the region of interest (ROI) of the CC in the midsagittal plane were measured and used as discriminative indexes. Bayesian classification with leave-one-out cross-validation was used to determine diagnostic accuracy. Differences in diffusion indexes of ROIs among groups were evaluated by using the Kruskal-Wallis test, followed by the Mann-Whitney U test for multiple comparisons and Bonferroni correction. RESULTS: Mean MD (8.48 x 10(-4) mm(2)/sec) and FA (0.729) of the ROI in patients with RNMO were significantly (P<.001) different from those (MD=10.64 x 10(-4) mm(2)/sec, FA=0.599) in patients with RRMS. Sensitivity and specificity for differentiation were 92.3% (24 of 26 patients with RNMO) and 93.5% (43 of 46 patients with RRMS) for FA and 88.5% (23 of 26 patients with RNMO) and 89.1% (41 of 46 patients with RRMS) for MD, respectively. CONCLUSION: Measurement of diffusion indexes of the CC may be useful for distinguishing patients with RNMO from those with RRMS.

Research ID:- Neuromyelitis optica research 17522347 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Neuromyelitis optica: changing concepts. Related Articles

Neuromyelitis optica: changing concepts.

J Neuroimmunol. 2007 Jul;187(1-2):126-38

Neuromyelitis optica research. Authors: Jacob A, Matiello M, Wingerchuk DM, Lucchinetti CF, Pittock SJ, Weinshenker BG

Neuromyelitis optica (NMO; Devic's disease) and the NMO spectrum disorders are idiopathic inflammatory demyelinating disorders that affect the central nervous system and have a predilection for optic nerves and spinal cord. The identification of NMO-IgG as a disease-specific marker and aquaporin 4 as the target antigen has renewed interest in NMO. Based on current data, we suspect that autoantibodies arising from peripheral B cells bind to aquaporin 4 expressed on astrocyte foot processes on the abluminal surface of microvessels, activate complement and initiate inflammatory demyelination and necrosis. The development of animal models and further analysis of the association of NMO-IgG with disease severity and treatment response will elucidate the pathobiology of NMO.

Research ID:- Neuromyelitis optica research 17512987 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Nosology of idiopathic transverse myelitis syndromes. Related Articles

Nosology of idiopathic transverse myelitis syndromes.

Acta Neurol Scand. 2007 Jun;115(6):371-6

Neuromyelitis optica research. Authors: Scott TF

Several terms are now commonly used to describe various presentations of idiopathic myelitis, including acute transverse myelitis, acute partial transverse myelitis, and secondary myelitis. Ideally, a classification system would be able to encompass various presentations in a manner that not only assists in prognosis, but also in treatment decisions. Unfortunately, we are limited in our ability to accurately identify those patients who will progress to develop multiple sclerosis, Devic's syndrome, relapsing myelitis, or will remain monophasic. However, general principles are emerging that assist in prognosis based on the particular presenting features of any patient. We review the most recent criteria proposed for various forms of transverse myelitis and highlight the limitations of these classification schemes.

Research ID:- Neuromyelitis optica research 17511844 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] An unusual chiasmal visual defect in a patient with neuromyelitis optica: case report. Related Articles

An unusual chiasmal visual defect in a patient with neuromyelitis optica: case report.

Arq Bras Oftalmol. 2007 Jan-Feb;70(1):153-5

Neuromyelitis optica research. Authors: Costa RM, Santos AC, Costa LS

PURPOSE: To report the unusual visual field finding due to a chiasmal neuritis in a 33-year-old female with the diagnosis of optic neuromyelitis optica (Devic's syndrome). METHODS: We report a case of a 33 years old female with limb paraesthesias, weakness in the legs, bowel and bladder dysfunction that was referred to the "Hospital das Cl�nicas da Faculdade de Medicina de Ribeir�o Preto da Universidade de S�o Paulo" in October 1995. Six years and four months later she had an acute visual involvement. Ophthalmologic examination, laboratory studies, magnetic resonance imaging (MRI) and a 24-2 threshold visual field in the Humphrey field analyzer were performed. RESULTS: The MRI scan showed enlargement and cavitation on the spinal cord and chiasmal involvement (thickening of the chiasm with contrast enhancement) and no demyelinating lesions in the brain, brainstem, or cerebellum. The central 24-degree threshold field examination showed an inferior visual field defect bitemporally, disclosing a chiasmal involvement. CONCLUSION: Chiasmal involvement may occur in neuromyelitis optica, probably due to a plaque within the chiasm. The Neuromyelitis optica research. Authors call attention to the importance of visual field examination with particular regard to quantifying the visual impairment and follow-up of these patients.

Research ID:- Neuromyelitis optica research 17505738 [PubMed - in process] Bibliography. Current world literature. Demyelinating diseases. Related Articles

Bibliography. Current world literature. Demyelinating diseases.

Curr Opin Neurol. 2007 Jun;20(3):358-67

Neuromyelitis optica research. Authors:

Research ID:- Neuromyelitis optica research 17495633 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Comparative immunopathogenesis of acute disseminated encephalomyelitis, neuromyelitis optica, and multiple sclerosis. Related Articles

Comparative immunopathogenesis of acute disseminated encephalomyelitis, neuromyelitis optica, and multiple sclerosis.

Curr Opin Neurol. 2007 Jun;20(3):343-50

Neuromyelitis optica research. Authors: Wingerchuk DM, Lucchinetti CF

PURPOSE OF REVIEW: Advanced immunopathological techniques hold promise for more precise diagnosis of idiopathic demyelinating diseases of the central nervous system. We review recent progress in differentiating and understanding the disease mechanisms of acute disseminated encephalomyelitis, neuromyelitis optica, and classical multiple sclerosis. RECENT FINDINGS: Four distinct immunopathological patterns have been described in multiple sclerosis patients, potentially implicating different inflammatory, demyelinating, and apoptotic mechanisms. A specific serum biomarker, neuromyelitis optica immunoglobulin G, is strongly associated with neuromyelitis optica and identifies patients with severe optic nerve and spinal cord lesions with specific pathological features such as eosinophilic and neutrophilic inflammatory infiltrates, necrosis, vascular hyalinization, and extensive vasculocentric immunoglobulin and complement deposition. This biomarker targets the water channel aquaporin-4, which is lost in neuromyelitis optica lesions. Acute disseminated encephalomyelitis still has no validated clinical diagnostic criteria but its perivenous pathological findings distinguish it from multiple sclerosis and neuromyelitis optica. SUMMARY: The clinically heterogeneous group of idiopathic inflammatory demyelinating diseases of the central nervous system is characterized by several immunopathological patterns that suggest the involvement of diverse pathogenic effector mechanisms. Future advances in experimental pathology, immunology, molecular genetics, and neuroimaging, as well as the discovery of specific biomarkers, will more precisely define these disorders and lead to better targeted therapies.

Research ID:- Neuromyelitis optica research 17495631 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Neuromyelitis optica. Related Articles

Neuromyelitis optica.

Curr Opin Neurol. 2007 Jun;20(3):255-60

Neuromyelitis optica research. Authors: Matiello M, Jacob A, Wingerchuk DM, Weinshenker BG

PURPOSE OF REVIEW: We review recent advances in neuromyelitis optica, an idiopathic inflammatory demyelinating disease of the central nervous system predominantly affecting optic nerves and spinal cord. We concentrate on a recently identified serum antibody biomarker, neuromyelitis optica immunoglobulin G (NMO-IgG), which distinguishes neuromyelitis optica from multiple sclerosis. RECENT FINDINGS: NMO-IgG is detected by indirect immunofluorescence. Its presence and specificity for neuromyelitis optica was confirmed in diverse populations. Seropositivity is now incorporated into new diagnostic criteria for neuromyelitis optica. Testing for this biomarker has suggested that the neuromyelitis optica spectrum is broader than previously recognized. Recently, the molecular target of NMO-IgG was identified as aquaporin-4. Immunopathologic studies suggest that loss of aquaporin-4 immunostaining is detectable in early lesions of neuromyelitis optica. A B-cell-specific monoclonal antibody, rituximab, may be an effective treatment even in patients not responding to other treatments. SUMMARY: Clinical, radiologic, and immunologic features distinguish neuromyelitis optica from other severe cases of multiple sclerosis. NMO-IgG is the first specific marker for a central nervous system demyelinating disease. The discovery of aquaporin-4 as the putative target of NMO-IgG, and recent data suggesting that aquaporin-4-specific antibodies are pathogenic may enhance our understanding of idiopathic inflammatory demyelinating diseases and their treatment.

Research ID:- Neuromyelitis optica research 17495617 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Revised diagnostic criteria for neuromyelitis optica--incorporation of NMO-IgG status. Related Articles

Revised diagnostic criteria for neuromyelitis optica--incorporation of NMO-IgG status.

Nat Clin Pract Neurol. 2007 May;3(5):E1

Neuromyelitis optica research. Authors: Jarius S, Paul F, Franciotta D, Aktas O, Hohlfeld R, Zipp F, Vincent A

Research ID:- Neuromyelitis optica research 17479069 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Complexity and heterogeneity in demyelinating disease. Related Articles

Complexity and heterogeneity in demyelinating disease.

Brain. 2007 May;130(Pt 5):1178-80

Neuromyelitis optica research. Authors: Compston A

Research ID:- Neuromyelitis optica research 17472981 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] A population-based survey of multiple sclerosis in Shanghai, China. Related Articles

A population-based survey of multiple sclerosis in Shanghai, China.

Neurology. 2007 May 1;68(18):1495-500

Neuromyelitis optica research. Authors: Cheng Q, Miao L, Zhang J, Ding SJ, Liu ZG, Wang X, Sun XJ, Zhao ZX, Song YJ, Ding XY, Guo ZL, Yang Y, Chen SD, Jiang GX, Fredrikson S

OBJECTIVE: To conduct a large population-based survey on multiple sclerosis (MS) prevalence in Shanghai, China. METHODS: We established a network of physicians, mainly neurologists, for identifying prevalent patients with MS and systematically checked inpatient registers at each hospital in the study area for patients with a diagnosis of MS, neuromyelitis optica, or other demyelinating disorders. MS diagnosis in patients was validated by senior neurologists according to the McDonald criteria. RESULTS: In total, 123 patients with a validated MS diagnosis from the study population, 8.86 million inhabitants with permanent residence in Shanghai, were alive on the prevalence day. The crude MS prevalence rate was 1.39 cases per 100,000 inhabitants (95% CI: 1.16 to 1.66 cases) in the study population in Shanghai. There were 79 female and 44 male patients with MS, a female-to-male ratio of 1.8. Nearly all (96%) of the patients with validated MS had been examined by MRI. CONCLUSION: Multiple sclerosis prevalence in Shanghai is in line with that reported for other Asian populations.

Research ID:- Neuromyelitis optica research 17470752 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Anti-aquaporin 4 antibody in selected Japanese multiple sclerosis patients with long spinal cord lesions. Related Articles

Anti-aquaporin 4 antibody in selected Japanese multiple sclerosis patients with long spinal cord lesions.

Mult Scler. 2007 Aug;13(7):850-5

Neuromyelitis optica research. Authors: Tanaka K, Tani T, Tanaka M, Saida T, Idezuka J, Yamazaki M, Tsujita M, Nakada T, Sakimura K, Nishizawa M

Multiple sclerosis (MS) in Asian populations is often characterized by the selective involvement of the optic nerve (ON) and spinal cord (SP) (OSMS) in contrast to classic MS (CMS), where frequent lesions are observed in the cerebrum, cerebellum or brainstem. In Western countries, inflammatory demyelinating disease preferentially involving the ON and SP is called neuromyelitis optica (NMO). Recently, Lennon et al. discovered that NMO-IgG, shown to bind to aquaporin 4 (AQP4), could be a specific marker of NMO and also of Japanese OSMS whose clinical features were identical to NMO having long spinal cord lesions extending over three vertebral segments (LCL). To examine this antibody in larger populations of Japanese OSMS patients in order to know its epidemiological and clinical spectra, we established an immunohistochemical detection system for the anti-AQP4 antibody (AQP4-Ab) using the AQP4-transfected human embryonic kidney cell line (HEK-293) and confirmed AQP4-Ab positivity together with the immunohistochemical staining pattern of NMO-IgG in approximately 60% of Japanese OSMS patients with LCL. Patients with OSMS without LCL and those with CMS were negative for this antibody. Our results accorded with those of Lennon et al. suggest that Japanese OSMS with LCL may have an underlying pathogenesis in common with NMO.

Research ID:- Neuromyelitis optica research 17468440 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Pathogenetic role of myelitis for syringomyelia. Related Articles

Pathogenetic role of myelitis for syringomyelia.

Clin Neurol Neurosurg. 2007 Jul;109(6):541-6

Neuromyelitis optica research. Authors: Ravaglia S, Bogdanov EI, Pichiecchio A, Bergamaschi R, Moglia A, Mikhaylov IM

BACKGROUND: CSF-flow obstruction is regarded as a mandatory factor for the development of syringomyelia. However, there are conditions in which syringomyelia is not associated with evident persistent CSF-flow obstruction, as in the case of inflammatory spinal cord lesions. In these instances we hypothesize that the accumulation of vasogenic edema may play a role in the development of the syrinx. Recently proposed theories underline, even in the event of CSF-flow obstructions, a major role for the accumulation and final coalescence of interstitial spinal fluid, rather than CSF penetration through the spinal cord. AIM: To clarify the relationship between syrinx development and spinal cord inflammation, through the analysis of the role of intrinsic medullary factors versus CSF-flow block. METHODS: A prospective case series including patients with transient syringomyelia associated with different examples of non-infectious myelitis: sarcoidosis, post-infectious transverse myelitis, Devic's disease and multiple sclerosis. Cavitations resulting from cystic myelomalacia were excluded. CSF-flow block was assessed by structural MRI. RESULTS: Syringes associated with myelitis shared some common features: they developed during the acute phase of myelitis and disappeared after steroids, were all non-communicating cavitations involving the central canal, and occurred in the same spinal segment affected by myelitis. CSF-flow obstruction was detected in one patient (Chiari I malformation), while in the other three patients we could not detect anatomical predispositions. CONCLUSION: Only one patient had structural abnormalities, though without evidence of a pathogenetic role in itself: however, CSF space obstruction and reduced CSF compliance could have accelerated the development of syringomyelia triggered by intramedullary inflammation. The clinical and radiological features in this patient are consistent with the label "presyringomyelia". The absence of any anatomical predisposition in the other patients suggests a major pathophysiological role for intrinsic medullary mechanisms, including blood-spinal cord barrier breakdown, impairment of extracellular fluid drainage, and leakage of subarachnoidal CSF into the nervous tissue.

Research ID:- Neuromyelitis optica research 17467892 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Need for knowledge of local scanners for patients with morbid obesity. Related Articles

Need for knowledge of local scanners for patients with morbid obesity.

Emerg Med J. 2007 May;24(5):372

Neuromyelitis optica research. Authors: Ahmed SM, Bhamidipati KR, Ahmad R

Research ID:- Neuromyelitis optica research 17452721 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Anti-aquaporin-4 antibody is involved in the pathogenesis of NMO: a study on antibody titre.

Anti-aquaporin-4 antibody is involved in the pathogenesis of NMO: a study on antibody titre.

Brain. 2007 May;130(Pt 5):1235-43

Neuromyelitis optica research. Authors: Takahashi T, Fujihara K, Nakashima I, Misu T, Miyazawa I, Nakamura M, Watanabe S, Shiga Y, Kanaoka C, Fujimori J, Sato S, Itoyama Y

NMO-IgG is a disease-specific autoantibody for neuromyelitis optica (NMO) and its target antigen is aquaporin-4 (AQP4) water channel. Recently, we established a sensitive anti-AQP4 antibody assay using human AQP4-transfected cells, which appeared more sensitive than the original NMO-IgG assay. So far, there has been no large-scale study on anti-AQP4 antibody titre in NMO and related disorders. We tested 148 sera of patients with NMO, high-risk syndrome of NMO, multiple sclerosis (MS), clinically isolated syndrome suggestive of MS and miscellaneous diseases. We analysed the relation of anti-AQP4 antibody titres and clinical and laboratory parameters. The sensitivity of anti-AQP4 antibody assay was 91% (95% CI 79-100) for NMO and 85% (65-100) for high-risk syndrome, and the specificity was 100% (91-100) for NMO and high-risk syndrome, that is, none with the other disorders was positive. Among 21 anti-AQP4 antibody-positive cases whose NMO-IgG were tested, 15 were NMO-IgG-positive and 6 were NMO-IgG-negative. Higher anti-AQP4 antibody titres were associated with complete blindness and extensive or large cerebral lesions on MRI. The lengths of spinal cord lesions on MRI were positively correlated with the titres of anti-AQP4 antibody at the nadir of exacerbations. A few patients who had short (approx. one to two vertebral segments) spinal cord lesions on MRI were also seropositive with low anti-AQP4 antibody titres, but did have other clinical and MRI features of NMO. Anti-AQP4 antibody titres became lower after high-dose methylprednisolone, and a follow-up showed anti-AQP4 antibody titres remained low in relapse-free periods under immunosuppression. Cerebrospinal fluid (CSF)-anti-AQP4 antibody was detected when the serum-antibody titres exceeded 512x, at the ratio of 1 (CSF) to 500 (serum). Using a sensitive assay, the results of the present study suggest that NMO and high-risk syndrome may be essentially anti-AQP4 antibody-associated disorders, and that the anti-AQP4 antibody titres have significant clinical and immunological implications in NMO.

Research ID:- Neuromyelitis optica research 17449477 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ] Neuromyelitis optica following CMV primo-infection. Related Articles

Neuromyelitis optica following CMV primo-infection.

J Intern Med. 2007 May;261(5):500-3

Neuromyelitis optica research. Authors: Tran C, Du Pasquier RA, Cavassini M, Guex-Crosier Y, Meuli R, Ciuffreda D, Waeber G

We report the case of an acute optic neuromyelitis with rhabdomyolysis in a 34-year-old immunocompetent transsexual patient following a recent cytomegalovirus (CMV) infection. The combination of optic neuropathy and myelopathy is recognized as Devic's syndrome. Clinical presentation was unusual as the recent CMV infection induced rhabdomyolysis and was the suspected trigger of neuromyelitis.

Research ID:- Neuromyelitis optica research 17444889 [PubMed - indexed for MEDLINE ( Neuromyelitis optica research ) ]

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