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PubMed2006

Neuromyelitis optica.

Neuromyelitis optica (NMO), otherwise known as Devic's disease, is an idiopathic, severe, inflammatory disorder that preferentially affects the optic nerves and spinal cord. Clinical, laboratory and immunopathological evidence suggests that NMO is a humorally mediated disease distinct from MS. A spinal cord lesion extending contiguously over three or more vertebral segments is characteristic of NMO and, in combination with a cranial magnetic resonance imaging scan that does not meet radiological criteria for MS, is over 94% sensitive and 96% specific for NMO diagnosis. The serum autoantibody marker, neuromyelitis optica-immunoglobulin G (NMO-IgG), appears specific for NMO and suggests that the disease spectrum includes cases of recurrent longitudinally extensive transverse myelitis and Japanese opticospinal MS. Its target antigen is the water channel aquaporin-4, suggesting that NMO may represent a novel autoimmune channelopathy. Relapsing NMO has a poor prognosis; therapy, typically with immunosuppression, is necessary as early as possible in the disease course to prevent attack-related disability.

Autoantibodies↗
PubMed2006

Evidence for humoral autoimmunity in neuromyelitis optica.

Neuromyelitis optica (NMO) is an idiopathic central nervous system (CNS) demyelinating syndrome that may be distinguished from typical multiple sclerosis (MS). Although the cause of the disorder is not known, several lines of evidence suggest that the fundamental immunological process is driven by humoral mechanisms. These observations include the frequent coexistence of systemic autoimmune disease or positive serum autoantibodies with NMO, 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. Furthermore, clinical experience suggests that plasmapheresis and immunosuppressive therapies are beneficial for treatment and prevention of acute attacks but that standard MS immunomodulatory drugs may not alter the course of NMO. This evidence is reviewed in the context of its implications for future laboratory and clinical research in NMO.

Animals↗
PubMed2001

Detection of brain-specific autoantibodies to myelin oligodendrocyte glycoprotein, S100beta and myelin basic protein in patients with Devic's neuromyelitis optica.

Neuromyelitis optica (NMO) is a rare syndrome characterized by the combination of acute optic neuritis and transverse myelitis, usually not seen in Multiple Sclerosis (MS) and other demyelinating syndromes of the central nervous system (CNS). A high prevalence of various autoantibodies has been described in patients with NMO suggesting a polyclonal activation of the humoral immune system. We examined autoantibody responses to myelin (MBP, MOG with isotypes and epitopes) and astroglial (S100beta) antigens in four patients with NMO by ELISA and Immunoblot. All patients showed a positive anti-MOG response, with one showing reaction to the MOG epitope corresponding to amino acid sequence 63-87. MBP-autoantibodies were only detected in two and S100beta-autoantibodies in one patient. Despite the limited number of samples, these findings suggest a predominant anti-MOG rather than anti-MBP or anti-S100beta autoantibody response in NMO, though no NMO-specific antibody pattern was found, which is in keeping with a widespread acute immune activation, including a strong B-cell response.

Adult↗
PubMed2006

NMO-IgG: a specific biomarker for neuromyelitis optica.

Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that principally targets the optic nerves and spinal cord and often leads to severe disability and occasionally life threatening respiratory failure. Although its clinical manifestations overlap with those of multiple sclerosis (MS), in established cases these two conditions can be distinguished on the basis of clinical, radiological, and routine spinal fluid studies. The diagnosis in early cases or limited forms of NMO is difficult. We recently discovered a unique IgG autoantibody (NMO-IgG) that is highly specific to patients with NMO and thus a valuable diagnostic aid. Its antigen, aquaporin-4 (AQP4), is the central nervous system's predominant water channel protein. This antibody has not yet been proven to be pathogenic, but several facts suggest that it might be, including the similarity of the immunohistochemical pattern of NMO-(AQP4) IgG binding to mouse CNS tissues to the pattern of immune complex deposition in autopsied patients' spinal cord tissue. The spectrum of diseases identified by NMO-IgG is broader than has previously been recognized clinically and includes incomplete forms of NMO, such as recurrent transverse myelitis without optic neuritis and recurrent optic neuritis without myelitis.

Autoantibodies↗
PubMed2006

Establishment of a new sensitive assay for anti-human aquaporin-4 antibody in neuromyelitis optica.

Neuromyelitis optica (NMO) is a devastating neurologic disease characterized by severe optic neuritis and transverse myelitis. Recently, its disease-specific serum autoantibody, NMO-IgG, was discovered with indirect immunofluorescence. However, the substrates of the immunofluorescence assay were not human but mouse brain tissues, which could influence the sensitivity and specificity of the antibody. The target antigen of NMO-IgG was recently identified as aquaporin-4 (AQP4) water channel protein, which is mainly expressed in brain and spinal cord. In the present study, we have established human cell lines that stably express human AQP4 and used these cells to detect and titrate anti-AQP4 antibody present in the sera of patients with NMO by immunofluorescence assay. The results were compared with those of the original NMO-IgG assay. We tested the sera from 10 patients with NMO, 10 with MS and five with other neurological disorders. Among the patients with NMO, six were NMO-IgG-positive. However, using the new anti-AQP4 antibody assay, we showed that eight patients with NMO including the six NMO-IgG-positives were positive for anti-AQP4 antibody. The staining pattern of AQP4-expressing cells treated with each serum of these eight NMO patients corresponded to that with a commercially available anti-AQP4 antibody. The antibody titer (maximum serum dilution for positive staining) ranged from 64x to 16,384x. The serum dilution titers were reproducible in blinded studies. In contrast, the patients with MS or other neurological disorders showed negative for anti-AQP4 antibody. Thus, the newly developed anti-AQP4 antibody assay appears to have a higher sensitivity for NMO than the original NMO-IgG assay and is expected to be useful for the diagnosis of NMO.

Adult↗
PubMed2005

Neuromyelitis Optica.

Neuromyelitis optica (Devic's syndrome) is an uncommon, idiopathic, demyelinating syndrome of the central nervous system that preferentially affects the optic nerves and spinal cord. It frequently is misdiagnosed as severe multiple sclerosis, but usually is readily distinguished from multiple sclerosis in fully developed cases because of its severity, typical magnetic resonance imaging (MRI) findings (normal brain MRI; longitudinally extensive lesions on spinal cord MRI), and cerebrospinal fluid analysis (polymorphonuclear pleocytosis and absence of oligoclonal banding). A serum autoantibody marker, NMO-IgG, is highly specific for the disorder. Most patients have relapsing disease, and natural history studies confirm early and severe disability. We treat acute myelitis and optic neuritis exacerbations with parenteral corticosteroids and use rescue plasmapheresis for severe, refractory attacks. Immunomodulatory drugs used for typical multiple sclerosis seem ineffective for relapse prevention. We recommend systemic immunosuppression, usually with azathioprine and oral corticosteroids, for most patients. Fulminant disease and breakthrough disease may respond to other forms of humoral immunotherapy such as rituximab.

Journal Article↗
PubMedDate not supplied

Pediatric Devic's neuromyelitis optica.

PURPOSE: Neuromyelitis optica (Devic's disease) is a rare clinical syndrome of unilateral or bilateral optic neuritis (ON) and transverse myelitis (TM) occurring within an 8-week time interval. All reports mainly involve adults and describe a poor neurologic and ophthalmic clinical course without specific attention to their few pediatric cases. The purpose of this study was to develop a clinical profile in the pediatric population. METHODS: A retrospective chart review over 15 years revealed nine cases of neuromyelitis optica. A literature search was undertaken and all cases of pediatric neuromyelitis optica were tabulated and analyzed separately. RESULTS: The average age of onset was 7 years with a preceding viral prodrome in all patients. The optic neuritis was bilateral in eight of nine patients (89%). Visual loss ranged from 20/30 to light perception. Moderate anterior disc edema was seen in all 17 affected eyes. The clinical course was characterized by a rapid visual and neurologic recovery. The average follow up was 5.3 years, with five patients followed for 6 years or longer. None of the nine patients had any visual or neurologic recurrence or other significant subsequent illness. Visual system follow up showed 20/20 in each eye of all patients with mild optic atrophy, identified in 47% of eyes. CONCLUSIONS: Devic's neuromyelitis optica occurs in the pediatric population as a distinct clinical entity with an excellent prognosis for visual and systemic recovery and no future recurrence or long-term sequelae.

Age of Onset↗
PubMed2006

Brain abnormalities in neuromyelitis optica.

BACKGROUND: Neuromyelitis optica (NMO) is a severe demyelinating disease defined principally by its tendency to selectively affect optic nerves and the spinal cord causing recurrent attacks of blindness and paralysis. Contemporary diagnostic criteria require absence of clinical disease outside the optic nerve or spinal cord. We have, however, frequently encountered patients with a well-established diagnosis of NMO in whom either asymptomatic or symptomatic brain lesions develop suggesting that the diagnostic criteria for NMO should be revised. OBJECTIVE: To describe the magnetic resonance image (MRI) brain findings in NMO. DESIGN: Observational, retrospective case series. Patients We ascertained patients through a clinical biospecimens database of individuals with definite or suspected NMO. We included patients who (1) satisfied the 1999 criteria of Wingerchuk et al for NMO except for the absolute criterion of lacking symptoms beyond the optic nerve and spinal cord and the supportive criterion of having a normal brain MRI at onset; (2) had MRI evidence of a spinal cord lesion extending 3 vertebral segments or more (the most specific nonserological feature to differentiate NMO from MS); and (3) were evaluated neurologically and by brain MRI at the Mayo Clinic. MAIN OUTCOME MEASURES: Magnetic resonance images were classified as normal or as abnormal with either nonspecific, multiple sclerosis-like or atypical abnormalities. We evaluated whether brain lesions were symptomatic and analyzed the neuropathologic features of a single brain biopsy specimen. RESULTS: Sixty patients (53 women [88%]) fulfilled these inclusion criteria. The mean +/- SD age at onset was 37.2 +/- 18.4 years and the mean +/- SD duration of follow-up was 6.0 +/- 5.6 years. Neuromyelitis optica-IgG was detected in 41 patients (68%). Brain MRI lesions were detected in 36 patients (60%). Most were nonspecific, but 6 patients (10%) had multiple sclerosis-like lesions, usually asymptomatic. Another 5 patients (8%), mostly children, had diencephalic, brainstem or cerebral lesions, atypical for multiple sclerosis. When present, symptoms of brain involvement were subtle, except in 1 patient who was comatose and had large cerebral lesions. CONCLUSIONS: Asymptomatic brain lesions are common in NMO, and symptomatic brain lesions do not exclude the diagnosis of NMO. These observations justify revision of diagnostic criteria for NMO to allow for brain involvement.

Adult↗
PubMed2002

Neuromyelitis optica.

Whether neuromyelitis optica (NMO), the co-occurrence of myelitis and optic neuritis, is a variant of multiple sclerosis (MS) or a unique disease is controversial. Distinct neuropathological features and a fulminant clinical course argue in favor of NMO as a distinct disease. However, the combination of neurological impairments of myelitis and optic neuritis occurs in patients with several inflammatory disorders, including multiple sclerosis and collagen vascular diseases. NMO is also associated with certain infectious diseases. The fact that the NMO phenotype occurs in a variety of disease states suggests that NMO does not represent a specific clinical entity. To better understand NMO and its associations with recognized diseases, a systematic review of the literature using MEDLINE was conducted. The history of NMO, its nosology, associations with other diseases, and current concepts of its pathogenesis and treatment is reviewed in this article.

Adult↗
PubMed2007

Diagnosis and treatment of neuromyelitis optica.

BACKGROUND: Neuromyelitis optica (NMO) is an uncommon CNS demyelinating syndrome often mistaken for severe multiple sclerosis (MS). Several clinical, laboratory, and neuroimaging characteristics may accurately distinguish NMO from MS early in the disease course. REVIEW SUMMARY: NMO is usually a relapsing disorder associated with early, severe, attack-related residual disability. It is associated with a highly specific antibody marker, NMO-IgG, which targets the water channel aquaporin-4. Revised NMO diagnostic criteria require optic neuritis, acute myelitis, and 2 of the following 3 characteristics: disease-onset brain magnetic resonance imaging (MRI) that is nondiagnostic for MS, contiguous spinal cord MRI lesion extending over 3 or more vertebral segments, and NMO-IgG seropositive status. Symptoms referable to central nervous system regions other than the optic nerve and spinal cord do not necessarily exclude the diagnosis of NMO, nor does the presence of brain MRI lesions. NMO-IgG has facilitated an appreciation that the spectrum of NMO is wider than previously recognized, and includes a proportion of patients with each of recurrent longitudinally extensive myelitis, recurrent isolated optic neuritis, and Japanese opticospinal MS. In contrast to typical MS, clinical experience and case series suggest that NMO requires long-term immunosuppressive therapy. CONCLUSIONS: NMO can be reliably differentiated from MS at an early stage using validated diagnostic criteria. The spectrum of NMO is wider than previously appreciated. Accurate, early diagnosis is critical to facilitate initiation of immunosuppressive therapy for attack prevention.

Humans↗
PubMed2006

[Neuroradiological aspects of Devic's neuromyelitis optica].

INTRODUCTION: Neuromyelitis optica (NMO) is a rare inflammatory and demyelinating disorder of the central nervous system, restricted to optical nerves and spinal cord. The main neuroradiological aspects, now summarized into a complete set of diagnosis criteria, are a normal cerebral MRI at onset and longitudinal involvement of the spinal cord concerning more than 3 vertebral segments. The clinical course and frequency of typical lesions remain unknown. OBJECTIVE: We here report neuroradiological data from patients suffering from NMO. METHODS: Brain and spinal cord MRI were systematically reviewed for 32 afro-Caribbean patients. RESULTS: A typical longitudinal spinal lesion was seen in 44.7 percent with or without edema; a lesion involving less than 3 vertebral segments in 26.3 percent and no lesion in 21.1 percent. Longitudinal study of a few bouts suggested a progressive normalisation of spinal cord appearance. Atrophy was negatively correlated with immunosuppressive treatment. Cerebral lesions usually absent at onset were correlated to the follow-up. In a non-recursive condition, patients completed diagnostic criteria for encephalic and spinal lesions in 82.8 percent and 48.1 percent. CONCLUSION: Radiology of spinal bouts showed multiple aspects besides the typical form. The notion of multiple bouts must be added to the spinal criteria to achieve good sensitivity. A typical extensive spinal lesion is usual in the follow-up, but seen after less then half of the bouts. Requiring such a lesion would delay the diagnosis.

Acute Disease↗
PubMed2005

Gluten sensitivity and neuromyelitis optica: two case reports.

Neuromyelitis optica is a clinical syndrome characterised by acute transverse myelitis plus an acute or subacute optic neuritis with or without recovery. Although once believed to be a variant of multiple sclerosis, diagnostic criteria have recently been proposed for neuromyelitis optica, making it a clinically distinct syndrome. The term gluten sensitivity refers to a state of heightened immunological responsiveness to ingested gluten in genetically susceptible individuals, as indicated by circulating antibodies to gliadin. Several neurological complications have been described associated with gluten sensitivity ranging from peripheral neuropathy and cerebellar ataxia to an increased risk of epilepsy. Although myelopathy has been described in some case reports of coeliac disease, neuromyelitis optica has never been described in association with gluten sensitivity. We describe two cases of gluten sensitivity presenting as neuromyelitis optica with no previous history of significant gastrointestinal symptoms. Gluten sensitivity was confirmed by immunological and histological studies.

Adult↗
PubMedDate not supplied

A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis.

BACKGROUND: Neuromyelitis optica is an inflammatory demyelinating disease with generally poor prognosis that selectively targets optic nerves and spinal cord. It is commonly misdiagnosed as multiple sclerosis. Neither disease has a distinguishing biomarker, but optimum treatments differ. The relation of neuromyelitis optica to optic-spinal multiple sclerosis in Asia is uncertain. We assessed the capacity of a putative marker for neuromyelitis optica (NMO-IgG) to distinguish neuromyelitis optica and related disorders from multiple sclerosis. METHODS: Indirect immunofluorescence with a composite substrate of mouse tissues identified a distinctive NMO-IgG staining pattern, which we characterised further by dual immunostaining. We tested masked serum samples from 102 North American patients with neuromyelitis optica or with syndromes that suggest high risk of the disorder, and 12 Japanese patients with optic-spinal multiple sclerosis. Control patients had multiple sclerosis, other myelopathies, optic neuropathies, and miscellaneous disorders. We also established clinical diagnoses for 14 patients incidentally shown to have NMO-IgG among 85000 tested for suspected paraneoplastic autoimmunity. FINDINGS: NMO-IgG outlines CNS microvessels, pia, subpia, and Virchow-Robin space. It partly colocalises with laminin. Sensitivity and specificity were 73% (95% CI 60-86) and 91% (79-100) for neuromyelitis optica and 58% (30-86) and 100% (66-100) for optic-spinal multiple sclerosis. NMO-IgG was detected in half of patients with high-risk syndromes. Of 14 seropositive cases identified incidentally, 12 had neuromyelitis optica or a high-risk syndrome for the disease. INTERPRETATION: NMO-IgG is a specific marker autoantibody of neuromyelitis optica and binds at or near the blood-brain barrier. It distinguishes neuromyelitis optica from multiple sclerosis. Asian optic-spinal multiple sclerosis seems to be the same as neuromyelitis optica.

Adult↗
PubMed2006

Study of mitoxantrone for the treatment of recurrent neuromyelitis optica (Devic disease).

BACKGROUND: Neuromyelitis optica is a severe demyelinating disease that selectively involves the optic nerves and the spinal cord but usually spares the brain. It is considered to have a B-cell-induced pathogenesis. Mitoxantrone hydrochloride, a synthetic anthracenedione approved for worsening relapsing-remitting multiple sclerosis and secondary progressive multiple sclerosis, has been shown to primarily suppress the humoral response. OBJECTIVE: To evaluate the benefit of mitoxantrone treatment in patients with relapsing neuromyelitis optica. DESIGN: Prospective 2-year study. SETTING: Academic multiple sclerosis center. PATIENTS: Five patients (3 women and 2 men) with an age range of 20 to 51 years and an Expanded Disability Status Scale score of 2.5 to 6.5 (mean +/- SD, 4.40 +/- 1.88). INTERVENTIONS: Monthly intravenous infusions of mitoxantrone hydrochloride, 12 mg/m2, for 6 months followed by 3 additional treatments every 3 months. MAIN OUTCOME MEASURES: Expanded Disability Status Scale score measured every 3 months and during relapses; findings on orbital, brain, and spinal cord magnetic resonance images performed at baseline and at 3, 6, 12, 18, and 24 months; and visual evoked potentials and results of ophthalmologic evaluations performed at baseline and annually. RESULTS: During the 2 years of treatment, 2 patients each had a relapse once within the initial 5 months of treatment (1 severe and 1 moderate). Improvement was seen clinically and on magnetic resonance images in 4 patients. Patients generally tolerated the treatment well, although 1 patient had a reversible decrease in cardiac ejection fraction. CONCLUSION: Our results suggest a beneficial effect of mitoxantrone treatment for relapsing neuromyelitis optica.

Adult↗
PubMed2004

Neuromyelitis optica: current concepts.

Neuromyelitis optica (NMO; Devic's syndrome or Devic's disease) is a clinically defined disorder consisting of optic neuritis (ON) in combination with myelitis. Its nosological relationship to other central nervous system demyelinating diseases remains uncertain. Advances in understanding of its natural history and pathophysiology may eventually allow determination of whether idiopathic NMO represents a specific disease. This article summarizes recent progress in these areas of NMO research.

Humans↗
PubMed2004

MR imaging of Devic's neuromyelitis optica.

Devic's neuromyelitis optica (NMO) is a neurological condition characterized by a severe involvement of the optic nerves and spinal cord in a mono- or multi-phasic manner. Recent pathologic, cerebrospinal fluid and conventional magnetic resonance imaging (MRI) studies have shown abnormalities which might help in differentiating patients with NMO from those with multiple sclerosis. This review article summarizes the main results obtained using conventional MRI in NMO, which has contributed to the definition of internationally-accepted diagnostic criteria, and those obtained from the application of modem quantitative MR techniques which are significantly changing our views on NMO pathobiology.

Brain↗
PubMed2006

Neuromyelitis optica immunoglobulin G in a child.

Neuromyelitis optica or Devic's syndrome is an uncommon demyelinating disorder that preferentially attacks the spinal cord and optic nerves. Although it is well described in adults, childhood neuromyelitis optica has rarely been reported in the literature and is frequently misdiagnosed as severe multiple sclerosis. Recently, a serum immunoglobulin G test for neuromyelitis optica has become available which may clarify and accelerate the diagnosis. This report describes a child with recurrent myelitis and an elongated spinal cord lesion who was found to have positive neuromyelitis optica autoantibody. We believe that neuromyelitis optica autoantibody testing should be performed in cases of pediatric transverse myelitis with multiple vertical segments or recurrence.

Anti-Inflammatory Agents↗
PubMed1996

Clinical, CSF, and MRI findings in Devic's neuromyelitis optica.

OBJECTIVES: Since Devic's original description of neuromyelitis optica in 1894 there has been much debate regarding its aetiology. A specific cause has been identified in a minority of cases but in most the question has arisen whether or not Devic's neuromyelitis optica is a variant of multiple sclerosis. This study was undertaken to help clarify this issue. METHODS: Neuromyelitis optica was defined as (1) a severe transverse myelitis; (2) an acute unilateral or bilateral optic neuropathy; (3) no clinical involvement beyond the spinal cord or optic nerves, and (4) a monophasic or multiphasic illness. The clinical and autoantibody status was documented. Patients underwent CSF examination and MRI of brain and spinal cord. RESULTS: Twelve patients, with a mean age of presentation of 35.1 years, were seen. Eleven were women; vision was reduced to counting fingers or worse in 10 patients and seven became confined to a wheelchair. Examination of CSF showed local synthesis of oligoclonal bands in only two patients and a neutrophil pleocytosis in two. A possible aetiology was identified in five: a specific connective tissue disorder (two), pulmonary tuberculosis (one), and possible acute disseminated encephalomyelitis (two). Six had non-specific increases in various autoantibodies. Eleven patients underwent MRI of the brain and spinal cord. In 10 there were diffuse abnormalities involving cervical and thoracic cords with extensive swelling in the acute phase. Brain MRI was normal in five; in five there were multiple deep white matter lesions, and one patient had minor age related changes. CONCLUSION: It is proposed that Devic's neuromyelitis optica is a distinctive disorder with some clinical, CSF, and MRI features different from those found in classic multiple sclerosis. In most cases a specific aetiology is not identified, but an immunological mechanism of tissue damage seems likely.

Adolescent↗