Modifications of longitudinally extensive transverse myelitis and brainstem lesions in the course of neuromyelitis optica (NMO): A population-based, descriptive study

BMC Neurology (Impact Factor: 2.04). 04/2013; 13(1):33. DOI: 10.1186/1471-2377-13-33
Source: PubMed


Neuromyelitis optica (NMO) includes transverse myelitis, optic neuritis and brain lesions. Recent studies have indicated that the brainstem is an important site of attack in NMO. Longitudinally extensive transverse myelitis (LETM) is an important component of the clinical diagnosis of NMO. The frequency of brainstem and LETM lesions, changes over time of LETM and the clinical consequences in the course of NMO have only been sparsely studied.
The study was a population-based retrospective case series with clinical and magnetic resonance imaging (MRI) follow-up of 35 patients with definite NMO and a relapsing-remitting course.
Brainstem lesions were observed in 25 patients, 18 in medulla oblongata (11 in area postrema). Lesions in the pons, mesencephalon and diencephalon occurred in 10, 7 and 7 patients, respectively. Lesions were symptomatic in medulla oblongata and pons, asymptomatic in mesencephalon and diencephalon. Brainstem lesions were observed significantly more often in anti-aquaporin-4 (AQP-4) antibody positive than in seronegative patients (p < 0.002).
LETM was demonstrated by MRI of the spinal cord in 30/36 patients, 23/30 of whom had follow-up MRI of the spinal cord. Recurrent LETM was observed in five patients. In nine patients the LETM changed into multiple lesions during remission or treatment. Spinal cord atrophy was observed in 12/23 (52%) patients, correlating to Expanded Disability Status Scale (r = 0.88, p < 0.001).
NMO patients had frequent occurrence of brainstem lesions and LETM. Brainstem lesions were associated with anti-AQP4 antibody positivity. LETM lesions differentiated over time and the outcome included relapses, fragmentation and atrophy. Correlation was observed between spinal cord atrophy and neurological disability.

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    • "In the presented case there is medulla damage resulting in prolonged nystagmus, with the seronegativity of AQP4-Ab. In NMO patients, a nystagmus is observed in 6% and cervical spine involvement in 70% of cases [16]. Further treatment was not possible, because of the unavailability of drugs. "
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    ABSTRACT: Aim Neuromyelitis optica is an autoimmune disorder connected with the effect of IgG antibodies against water channels (aquaporin-4) in the central nervous system. In the course of the disease, there is the involvement of the optic nerves and the spinal cord, resulting in a progressive disability. In the treatment, immunosuppressive agents are used as well as monoclonal antibodies such as rituximab (anti-CD20). According to the results, rituximab is the most effective, safe and recommended as a first-line treatment. Until now the duration and frequency of this therapy has not been established. The aim of this report is to provide the information necessary to create the optimal rituximab dosing regimen. Case An NMO patient, who was treated with rituximab for 6 years depending on the blood CD19+ B lymphocytes titres, is presented here. The complete relapse restrain and clinical stabilisation were achieved, with beneficial effect remaining even after the rituximab treatment was terminated – present observation period is almost 4 years without any immunosuppressive or immunomodulating treatment. Since the treatment was stopped, the CD19+ B lymphocyte population tends to rise, but the neurological state has not changed. The most recent magnetic resonance imaging (MRI) of the cervical spine, from 2014, showed a post-inflammatory lesion between the medulla and the cervical spine, which had been present before, without any new inflammatory lesions. The IgG antibodies against AQP-4 assays were performed in 2013 for the first time and were negative. Comment Temporary rituximab therapy in neuromyelitis optica may lead to a prolonged remission lasting some years.
    Full-text · Article · Nov 2015
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    • "Recently involvement of the brain has also been shown [4]. Brain lesions are localized in areas where AQP4 expression is high, and where blood-brain barrier is absent [4]. When the brain is involved, a complex and heterogenous picture characterized by endocrinopathy, posterior reversible encephalopathy, and brain stem signs occur, making the diagnosis difficult. "
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    DESCRIPTION: Relaps of NMDA Encephalitis Superimposed Upon Neuromiyelitis Optica.
    Full-text · Research · Sep 2015
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    ABSTRACT: Neuromyelitis optica (NMO) is a disease distinct from multiple sclerosis in terms of clinical and magnetic resonance imaging (MRI) manifestations. Antibody to aquaporin-4 (AQP4) has been identified as a specific biomarker and part of the diagnostic criteria for NMO. Although it is relatively common in Asia, a comprehensive clinical and imaging evaluation of NMO has not been reported in Chinese patients. Here, we reviewed data from 57 Chinese cases. The patients had an obvious female preponderance (female/male = 8.5:1), and transverse myelitis (82.5%) and optic neuritis (56.1%) were the most common manifestations. In MRI, longitudinally extensive transverse myelitis (6.9 ± 2.3 segments) dominated the spinal cord lesions, which were mainly (69.7%) distributed in cervical and thoracic cord. However, the length of the lesions was not correlated with onset age, paralysis severity, relapse rate, or duration. Among 29 patients who underwent AQP4 antibody assay, 17 (58.6%) were positive. There was no difference between seropositive and seronegative patients in terms of female preponderance, onset age, relapse rate, and Expanded Disability Status Scale score. However, seropositive patients had significantly more damaged segments (8.3 ± 3.5) than did seronegative patients (4.5 ± 1.6) (p < 0.001). The data revealed the clinical and MRI characteristics and AQP4 antibody status of NMO in Chinese patients and the correlations between them, which may have important implications for the diagnosis of the disease. © 2013 S. Karger AG, Basel.
    No preview · Article · Nov 2013 · European Neurology
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