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PubMed1990

Neuromyelitis optica (Devic's syndrome) and pulmonary tuberculosis.

Neuromyelitis optica and acute necrotic myelopathy occur in association with pulmonary tuberculosis. We studied 8 patients with either neuromyelitis optica (6), acute myelopathy (1), or acute optic neuropathy (1) in close temporal association with pulmonary tuberculosis, but with no evidence for CNS tuberculosis. Neurologic symptoms preceded the use of antituberculosis medication in 5 patients. Different patients showed similar clinical features, suggesting a consistent disease pattern. Autopsy examination (1 patient) revealed extensive spinal cord and optic nerve demyelination. We identified only 5 additional patients seen over the same period with idiopathic neuromyelitis optica, thus suggesting that the close temporal relationship to pulmonary tuberculosis is not coincidental. The syndrome is most likely due to an immune reaction to tuberculosis rather than the use of antituberculosis medication.

Adolescent↗
PubMed2004

Cerebrospinal fluid findings in Devic's neuromyelitis optica.

Relapsing Devic's neuromyelitis optica (DNO) may be clinically undistinguishable from multiple sclerosis (MS), thus the differential diagnosis relies mainly on neuroimaging and cerebrospinal fluid (CSF) findings. We studied CSF samples from 44 patients with DNO submitted to at least one lumbar puncture. Pleocytosis, IgG synthesis and blood brain barrier damage were the most frequent abnormalities, pleocytosis being very suggestive of DNO in patients fulfilling clinical and MRI diagnostic criteria. Pleocytosis > or =50 cells/mm3 is more frequent in the active phases of the disease. Oligoclonal bands (OBs) should be re-considered within the diagnostic criteria of DNO for possible variations in time: at variance with MS they may also disappear. Thus, more than one CSF examination should be done in the presence of suspected DNO, preferably in different disease phases. Although uncommon, OBs do not exclude DNO if optic nerve and spinal cord are the only sites of white matter damage, provided that cerebral MRI is normal at onset and during follow up.

Adolescent↗
PubMed2006

Loss of aquaporin-4 in active perivascular lesions in neuromyelitis optica: a case report.

Neuromyelitis optica (NMO) is clinically characterized by severe optic neuritis and transverse myelitis. In Japan, NMO has been named optic-spinal multiple sclerosis (OSMS) and it has been thought to be a subtype of multiple sclerosis (MS). However, several clinical and laboratory findings suggest NMO or OSMS is distinct from MS. Recently, the disease-specific antibody (NMO-IgG) was found in the serum from NMO patients, and its target antigen was identified as aquaporin-4 (AQP4) water channel protein which is mainly expressed in astroglial foot processes. However, the pathogenetic role of AQP4 in NMO remains unknown. We herein report a typical case of NMO in which immunohistochemical analysis showed a lack of AQP4 in the spinal cord lesions. The loss of AQP4 was evident in the central gray matter, especially in the perivascular lesions where immunoglobulins and complements were deposited, and glial fibrillary acidic protein (GFAP) staining was weak in those lesions. However, GFAP was strongly stained at the reactive astrogliosis surrounding the lesions. Myelin basic protein (MBP)-stained myelinated fibers were relatively preserved in the lesions where AQP4 was lost. In contrast to these NMO lesions, AQP4 was expressed predominantly in the gray matter in control spinal cords, and AQP4 was preserved in demyelinating MS lesions. Our findings suggest that astrocytic impairment associated with humoral immunity against AQP4 may be primarily involved in the lesion formation of NMO, and that the pathomechanisms of NMO are different from those of MS in which demyelination is the primary pathology.

Adult↗
PubMed2002

A role for humoral mechanisms in the pathogenesis of Devic's neuromyelitis optica.

Devic's disease [neuromyelitis optica (NMO)] is an idiopathic inflammatory demyelinating disease of the CNS, characterized by attacks of optic neuritis and myelitis. The mechanisms that result in selective localization of inflammatory demyelinating lesions to the optic nerves and spinal cord are unknown. Serological and clinical evidence of B cell autoimmunity has been observed in a high proportion of patients with NMO. The purpose of this study was to investigate the importance of humoral mechanisms, including complement activation, in producing the necrotizing demyelination seen in the spinal cord and optic nerves. Eighty-two lesions were examined from nine autopsy cases of clinically confirmed Devic's disease. Demyelinating activity in the lesions was immunocytochemically classified as early active (21 lesions), late active (18 lesions), inactive (35 lesions) or remyelinating (eight lesions) by examining the antigenic profile of myelin degradation products within macrophages. The pathology of the lesions was analysed using a broad spectrum of immunological and neurobiological markers, and lesions were defined on the basis of myelin protein loss, the geography and extension of plaques, the patterns of oligodendrocyte destruction and the immunopathological evidence of complement activation. The pathology was identical in all nine patients. Extensive demyelination was present across multiple spinal cord levels, associated with cavitation, necrosis and acute axonal pathology (spheroids), in both grey and white matter. There was a pronounced loss of oligodendrocytes within the lesions. The inflammatory infiltrates in active lesions were characterized by extensive macrophage infiltration associated with large numbers of perivascular granulocytes and eosinophils and rare CD3(+) and CD8(+) T cells. There was a pronounced perivascular deposition of immunoglobulins (mainly IgM) and complement C9neo antigen in active lesions associated with prominent vascular fibrosis and hyalinization in both active and inactive lesions. The extent of complement activation, eosinophilic infiltration and vascular fibrosis observed in the Devic NMO cases is more prominent compared with that in prototypic multiple sclerosis, and supports a role for humoral immunity in the pathogenesis of NMO. Based on this study, future therapeutic strategies designed to limit the deleterious effects of complement activation, eosinophil degranulation and neutrophil/macrophage/microglial activation are worthy of further investigation.

Antibody Formation↗
PubMed2006

Natural history of the visual impairment of relapsing neuromyelitis optica.

PURPOSE: To describe the clinical features and natural history of the visual impairment of relapsing neuromyelitis optica. DESIGN: Prospective observational case series. PARTICIPANTS: Thirty patients of Afro-Caribbean origin with neuromyelitis optica and 47 patients with multiple sclerosis. METHODS: A complete ophthalmologic examination was performed with each ocular attack and during a remission period at least 6 months after the last attack. MAIN OUTCOME MEASURES: Incidence of unilateral and bilateral optic neuritis and incidence of unilateral and bilateral severe visual loss, defined as visual acuity (VA) < or = 20/200. RESULTS: Twenty-eight patients (93%) with neuromyelitis optica were female and 2 (7%) were male. The mean age of onset was 30+/-10.5 years. Mean disease duration was 9.5+/-5.4 years. Neuromyelitis optica first manifested by an episode of optic neuritis in 23 cases (76.6%), bilateral in 4. The average number of ocular attacks per patient was 2.7+/-1.6. Twenty-one patients (70%) showed impairment in both eyes. Median times from onset to unilateral optic neuritis, bilateral optic neuritis, mono-ocular severe visual loss, and binocular severe visual loss were 0+/-0.08, 1+/-1.6, 2+/-0.8, and 13+/-3 years, respectively. Fifteen patients (50%) experienced severe visual loss in both eyes and 6 (20%) in one eye. Severe visual loss occurred in as few as 2 attacks. CONCLUSION: This study is the largest series of relapsing neuromyelitis optica in a population of African descent. Neuromyelitis optica's visual impairment is very severe; it contrasts drastically with that typically observed in multiple sclerosis. We confirmed that the prognosis for optic neuritis in patients with neuromyelitis optica is worse than the prognosis in patients with multiple sclerosis.

Adult↗
PubMed1999

The clinical course of neuromyelitis optica (Devic's syndrome).

OBJECTIVES: To evaluate the spectrum of neuromyelitis optica (NMO), including characteristics of the index events (optic neuritis [ON]) and myelitis), neuroimaging, CSF, and serologic studies, and to evaluate the long-term course. METHODS: Review of 71 patients with NMO evaluated at the Mayo Clinic between 1950 and 1997. RESULTS: NMO was either monophasic or relapsing. Patients with a monophasic course (n = 23) usually presented with rapidly sequential index events (median 5 days) with moderate recovery. Most with a relapsing course (n = 48) had an extended interval between index events (median 166 days) followed within 3 years by clusters of severe relapses isolated to the optic nerves and spinal cord. Most relapsing patients developed severe disability in a stepwise manner, and one-third died because of respiratory failure. Features of NMO distinct from "typical" MS included >50 cells/mm3 in CSF (often polymorphonuclear), normal initial brain MRI, and lesions extending over three or more vertebral segments on spinal cord MRI. CONCLUSIONS: Clinical, laboratory, and imaging features generally distinguish neuromyelitis optica from MS. Patients with relapsing optic neuritis and myelitis may have neuromyelitis optica rather than MS. Patients with a relapsing course of neuromyelitis optica have a poor prognosis and frequently develop respiratory failure during attacks of cervical myelitis.

Adolescent↗
PubMedDate not supplied

[NMO-IgG antibodies in neuromyelitis optica: a report of 2 cases].

INTRODUCTION: Neuromyelitis optica is an inflammatory demyelination disease that selectively affects optic nerves and spinal cord. Recently it has been described that the NMO-IgG antibodies, are highly specific for the diagnosis, although they are also present in partial forms of the disease. The antigen responsible for this immune response seems to be aquaporin-4 water channel. CLINICAL CASE: We describe the detection in our laboratory of NMO-IgG antibodies in two patients, one of them with a neuromyelitis optica and the other one with a recurrent myelitis and subclinical involvement of the optic nerve. CONCLUSIONS: By using dual immunostaining, confocal microscopy showed that the antibodies of both patients colocalized with that of an anti-aquaporin-4.

Adult↗
PubMed2005

An open label study of the effects of rituximab in neuromyelitis optica.

Eight patients with worsening neuromyelitis optica were treated with rituximab to achieve B cell depletion. Treatment was well tolerated. Six of eight patients were relapse free and median attack rate declined from 2.6 attacks/patient/year to 0 attacks/patient/year (p = 0.0078). Seven of eight patients experienced substantial recovery of neurologic function over 1 year of average follow-up. The pretreatment median Expanded Disability Status Scale score was 7.5, and at follow-up examination was 5.5 (p = 0.013).

Adult↗
PubMed2006

Neuromyelitis optica - Devic's syndrome, update.

Neuromyelitis optica of Devic (NMO) is a syndrome which combines transverse myelitis and optic neuritis. For many years it was assumed that transverse myelitis and optic neuritis should be simultaneous, that optic neuritis should be bilateral, and that transverse myelitis must be complete. NMO was considered to be very rare. Through the 20th century there was a debate on whether NMO is a distinct nosologic entity or a form of MS. In 2005 it is now possible to distinguish NMO from MS on the basis of the clinical, imaging, serology and immunopathology profile. This distinction is necessary for the implementation of adequate treatments, as the relapsing form of the illness carries significant morbidity and mortality.

Causality↗
PubMed1984

Neuromyelitis optica with unusual vascular changes.

A case of neuromyelitis optica had strikingly increased numbers of thickened, hyalinized small blood vessels, which were associated with cystic degeneration in the cervical spinal cord and the optic chiasm. These findings support the contention that neuromyelitis optica is a clinical syndrome rather than a distinct nosologic entity.

Demyelinating Diseases↗
PubMed2004

[Neuromyelitis optica (Devic's disease): a severe variant of multiple sclerosis].

In three women aged 20, 34 and 21, with a combination of optic neuritis and transverse myelitis, the diagnosis of neuromyelitis optica (Devic's disease) was made. All three were treated with methylprednisolone. The first patient died from respiratory failure associated with tetraparesis, over two years after experiencing initial symptoms. The other two women recovered to varying extents. By employing a combination of clinical investigations and MRI supplemented by laboratory investigations, it is possible to differentiate neuromyelitis optica from multiple sclerosis. Neuromyelitis optica is treated by a stronger form of immunosuppression. Despite recorded differences it remains possible that these conditions form part of the same spectrum in which the genetic background is important. In neuromyelitis optica the underlying condition seems to be in the field of the human immune response.

Adult↗
PubMed2004

Activation of humoral immunity and eosinophils in neuromyelitis optica.

OBJECTIVE: To study immunologic alterations in patients with neuromyelitis optica (NMO). METHODS: The authors studied 8 patients with NMO together with 16 healthy subjects, 16 patients with relapsing remitting multiple sclerosis (RRMS), and 16 patients with secondary progressive MS (SPMS), matched for age and sex, as controls. Because recent histopathologic studies have demonstrated that active NMO lesions consist of perivascular immunoglobulin (Ig) deposition and eosinophil infiltration, IL-5, IL-6, IL-12, IgG, and IgM production by anti-myelin oligodendrocyte glycoprotein (MOG) mononuclear cells in peripheral blood and CSF were selected for study using ELISPOT. Eotaxin-2 (Eo-2) and eotaxin-3 (Eo-3) levels were also assessed using ELISA and eosinophil cationic protein (ECP) levels by radioimmunoassay. RESULTS: MOG-specific responses in CSF showed significant increase in IL-5, IL-6, IgG, and IgM secreting cells in NMO patients compared with patients with RRMS, SPMS and healthy subjects. Interestingly, numbers of IgM secreting cells were significantly higher than identical specificity IgG secreting ones. Moreover, CSF Eo-2, Eo-3, and ECP levels were also significantly higher in NMO patients compared to all three control populations. Anti-MOG IL-12 secreting cells were increased in CSF and peripheral blood from NMO, RRMS, and SPMS patients when compared to healthy subjects. CONCLUSIONS: These observations suggest that neuromyelitis optica is associated with a major humoral immune response (particularly anti-MOG IgM production) and eosinophil activation present exclusively in CSF.

Adult↗
PubMed2006

Revised diagnostic criteria for neuromyelitis optica.

BACKGROUND: The authors previously proposed diagnostic criteria for neuromyelitis optica (NMO) that facilitate its distinction from prototypic multiple sclerosis (MS). However, some patients with otherwise typical NMO have additional symptoms not attributable to optic nerve or spinal cord inflammation or have MS-like brain MRI lesions. Furthermore, some patients are misclassified as NMO by the authors' earlier proposed criteria despite having a subsequent course indistinguishable from prototypic MS. A serum autoantibody marker, NMO-IgG, is highly specific for NMO. The authors propose revised NMO diagnostic criteria that incorporate NMO-IgG status. METHODS: Using final clinical diagnosis (NMO or MS) as the reference standard, the authors calculated sensitivity and specificity for each criterion and various combinations using a sample of 96 patients with NMO and 33 with MS. The authors used likelihood ratios and logistic regression analysis to develop the most practical and informative diagnostic model. RESULTS: Fourteen patients with NMO (14.6%) had extra-optic-spinal CNS symptoms. NMO-IgG seropositivity was 76% sensitive and 94% specific for NMO. The best diagnostic combination was 99% sensitive and 90% specific for NMO and consisted of at least two of three elements: longitudinally extensive cord lesion, onset brain MRI nondiagnostic for MS, or NMO-IgG seropositivity. CONCLUSIONS: The authors propose revised diagnostic criteria for definite neuromyelitis optica (NMO) that require optic neuritis, myelitis, and at least two of three supportive criteria: MRI evidence of a contiguous spinal cord lesion 3 or more segments in length, onset brain MRI nondiagnostic for multiple sclerosis, or NMO-IgG seropositivity. CNS involvement beyond the optic nerves and spinal cord is compatible with NMO.

Adolescent↗
PubMed1997

Neuromyelitis optica (Devic's syndrome): no association with the primary mitochondrial DNA mutations found in Leber hereditary optic neuropathy.

Devic's neuromyelitis optica is a rare syndrome characterised by the combination of acute or subacute optic neuritis and transverse myelitis, in some cases considered to be a variant of multiple sclerosis. Mutations of mitochondrial DNA (mtDNA) associated with Leber hereditary optic neuropathy (LHON) have been identified in some patients with multiple sclerosis in whom optic neuritis is a prominent early feature. Using restriction enzyme digestion of mtDNA products amplified by the polymerase chain reaction, the primary LHON mtDNA mutations at positions 3460 bp, 11,778 bp, and 14,484 bp have been excluded in four women with Devic's neuromyelitis optica. A mutation at 4160 bp associated in some LHON families with more widespread neurological disease was also not detected. It is concluded that the primary mtDNA mutations currently associated with LHON are not responsible for the prominence of optic nerve disease in Devic's neuromyelitis optica.

Adult↗
PubMed1982

Neuromyelitis optica (Devic's syndrome) in two sisters.

Neuromyelitis optica (Devic's syndrome) typically occurs in a sporadic pattern. We studied the disorder in two sisters, ages 6 and 10 years, who presented with sudden loss of vision at age 3 years, followed 5 months later by a transverse myelopathy. The older girl had only light perception in either eye, persistent paraparesis and progressive scoliosis; her sister had a visual acuity of 20/200 in both eyes and mild spinal cord dysfunction. Results of electrodiagnostic tests correlated well with the severity of clinical neurologic deficits and provided objective estimates of the extent of tissue damage in both the optic nerves and the spinal cord. The presence of Devic's syndrome in these two sisters could be coincidental; however, the possibility of a shared genetic factor(s) should be considered.

Child↗
PubMed2001

[Devics neuromyelitis optica. Disease or variants of multiple sclerosis?].

Neuromyelitis optica (NMO, or Devic's syndrome) is a rare syndrome characterized by the combination of acute or subacute optic neuritis and transverse myelitis. This strict confinement of lesions together with immunologic parameters such as frequent absence of oligoclonal banding despite increased signs of CSF inflammation suggest that NMO is distinct from multiple sclerosis (MS). Magnetic resonance imaging (MRI) data support NMO and MS as separate entities due to lesion distribution and signal characteristics. This is further supported by epidemiological and genetic evidence associating HLA-DRB1*1501 with disseminated "western" MS in contrast to NMO associated with DRB1*802, DPB1 501, and DPA1 202, the "Asian" type. Most findings suggest a heterogeneous pathogenesis of NMO and, in spite of the distinct localization, rather unspecific immune reactions seem to be involved. As to the frequent relapses of NMO, therapeutic options besides prednisolone are difficult to assess and favor long-term immune suppression or modulation.

Asia↗
PubMed1981

Multiple sclerosis and neuromyelitis optica in tropical and subtropical countries.

Among the indigenous people of tropical countries neuromyelitis optica is more common than multiple sclerosis which is exceptionally rare. It is postulated that these diseases are the result of virus-induced defects incorporated in the myelin at the time of myelination. These defects are located in those parts of the CNS which are undergoing active myelination at the time of the virus infection. A protective factor, which is capable of abolishing the defect is produced, directly or indirectly, by solar radiation. In tropical countries this factor abolishes most of the defects but it is unable to do so in the case of neuromyelitis optica because optic nerves and some parts of the spinal cord are myelinated before infants are exposed to such protective environmental factors. In temperate climates virus infections leave defects in many parts of the CNS and, lacking exposure to the protective environmental factor, these defects slowly mature to become plaques of multiple sclerosis.

Demyelinating Diseases↗
PubMed2003

Neuromyelitis optica: clinical predictors of a relapsing course and survival.

BACKGROUND: The relapsing form of neuromyelitis optica (NMO) is characterized by recurrent optic neuritis and myelitis, usually leading to severe, permanent, relapse-related neurologic impairment (e.g., blindness, paraplegia) within 5 years. Aggressive therapy aimed at relapse prevention initiated soon after disease onset may be expected to have a relatively greater impact on early relapse-related disability in NMO than in typical MS. Early prediction of a relapsing course and subsequent disease severity would facilitate design and implementation of clinical trials of such therapies. METHODS: A database of clinical and laboratory features of patients with NMO (n = 80) was used to develop potentially useful models predictive of a relapsing disease course and of subsequent disease severity as measured by survival. RESULTS: Predictors of a relapsing course were longer interattack interval between the first two clinical events (rate ratio [RR] = 2.16; per month increase), older age at onset (RR = 1.08; per year increase), female sex (RR = 10.0, female vs male), and less severe motor impairment with the sentinel myelitis event (RR = 0.48; per severity scale point increase). A history of other autoimmune disease (RR = 4.15; presence vs absence), higher attack frequency during the first 2 years of disease (RR = 1.21; per attack), and better motor recovery following the index myelitis event (RR = 1.84; per point increase) were associated with mortality due to relapsing NMO. CONCLUSIONS: These predictive models identify several clinical features, each available at diagnosis or early in the disease course, that predict relapsing disease and survival. These results may be useful to identify patients at high risk for severe, relapsing neuromyelitis optica in order to initiate early therapy for relapse prevention and to design clinical trials to study such interventions.

Adolescent↗