Aquaporin-4 antibody positive neuromyelitis optica spectrum disorder associated with esophageal cancer
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Neuromyelitis Optica
Spectrum disorder
Aquaporin 4
Neuromyelitis Optica
Spectrum disorder
Aquaporin 4
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Until recently, neuromyelitis optica (NMO) was considered to be a clinical variant of multiple sclerosis (MS). The discovery of disease-specific anti-aquaporin-4 antibody has facilitated the differentiation of NMO from MS and led to the recognition of a broader phenotypic spectrum now referred to as neuromyelitis optica spectrum disorder (NMOSD). However, distinguishing NMOSD from MS remains challenging, as a subgroup of NMOSD patients are found to be seronegative. The ability to differentiate between NMOSD and MS is critical because these conditions have distinct treatments and prognoses, and the disease-modifying treatment for MS can actually aggravate NMOSD. This review focuses on clinically relevant guidance for diagnosing and differentiating between NMOSD and MS. Key Words: Neuromyelitis optica, Multiple sclerosis, Differential diagnosis, Magnetic resonance imaging, Optical coherence tomography
Neuromyelitis Optica
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Aquaporin 4
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Neuromyelitis Optica
Eculizumab
Spectrum disorder
Aquaporin 4
Broad spectrum
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Neuromyelitis Optica
Spectrum disorder
Aquaporin 4
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To investigate longitudinal brain atrophy in patients with neuromyelitis optica spectrum disorder (NMOSD).We investigated the longitudinal brain atrophy rate in patients with aquaporin-4 antibody-positive NMOSD (AQP4+NMOSD) and those with multiple sclerosis (MS) in a retrospective cohort study. Brain volume was calculated with statistical parametric mapping-12.We enrolled 36 patients with AQP4+NMOSD and 60 with MS. Patients with NMOSD were older and had a higher Kurtzke's expanded disability status scale score at baseline MRI compared with those with MS. Disease duration, annual relapse rate and intervals from the last attack and from disease-modifying drugs initiation were not significantly different between the two groups. Lower normalised lesion volume and higher normalised white matter volume were found in patients with NMOSD compared with those with MS at baseline MRI. However, the annualised atrophy rate of normalised brain volume was similar between the NMOSD (median 0.47; IQR 0.75; p=0.49) and MS (median 0.46; IQR 0.84) groups. After adjustment of age and the presence of clinical relapse, no differences of the annualised atrophy rate of normalised brain volume also were found for NMOSD and MS. Patients with AQP4+NMOSD with long cord lesion showed higher annualised atrophy rate of normalised grey matter volume compared with those without long cord lesion.Silent progression of brain atrophy was present in patients with AQP4+NMOSD, as shown in patients with MS, even in the clinically inactive age-matched cases. Subclinical dying back degeneration may explain the brain atrophy in patients with AQP4 +NMOSD.
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Neuromyelitis Optica
Spectrum disorder
Aquaporin 4
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OBJECTIVE:To describe a patient with neuromyelitis optica spectrum disorders (NMOSD) who presented with sudden onset of sleep (SOOS) as the initial manifestation. BACKGROUND: It is now known that NMOSD patients may develop excessive sleepiness due to hypothalamic lesions. However, there is no report of SOOS in this disorder. DESIGN/METHODS:Single case report. RESULTS:A 41-year-old Japanese woman was admitted to our hospital because of a 3-month history of SOOS. During this time, she was involved in two car accidents because she suddenly fell asleep while driving. She did not feel sleepiness beforehand at each event. In another occasion, she fell asleep holding the chopsticks while eating. She also felt slight sleepiness in working time but she did not take a nap. Neurological examinations revealed no definite abnormalities. Cerebrospinal fluid protein was 20 mg/dl and cell count was 7/mm3. FLAIR images of brain MRI showed high-intensity areas in the bilateral hypothalamic regions. AQP4 antibody was positive in the serum. We diagnosed her as NMOSD and treated her by intravenous methylprednisolone for 3 days. After the treatment, SOOS completely disappeared in a few weeks. CONCLUSIONS:SOOS is a well-known serious side effect of non-ergot dopamine agonists, antiparkinsonian agents. Excessive daytime sleepiness and hypersomnia has been reported as a frequent symptom of NMOSD patients with hypothalamic lesions. However, there has been no report of SOOS in NMOSD patients. SOOS is a serious condition that may lead to critical car accidents, just as has occurred for the present patient, because of the lack of heralding sleepiness, and should be strictly discriminated from simple hypersomnia. It is imperative for physicians to recognize SOOS as a possible symptom of NMOSD.
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Neuromyelitis Optica
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Girl
Aquaporin 4
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Aquaporin-4 antibody (AQP4-Ab) neuromyelitis optica spectrum disorder (NMOSD) is a well-recognized autoimmune disease of the CNS. The presence of AQP4-Ab in patients presenting with the core clinical characteristics required for a diagnosis of NMOSD with AQP4-Ab1 is associated with a very high risk of relapse and consequently leads to blindness and paralysis if the syndrome remains untreated.2 Natural history studies have provided the evidence that there is an attack-related stepwise accumulation of disability in patients with NMOSD with AQP4-Ab; therefore, attack prevention strategies are used as maintenance treatment after the first event. The current advice is for lifelong treatment.3
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Despite off-label treatment with immunosuppressive regimens such as azathioprine and rituximab, many individuals with neuromyelitis optica spectrum
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