Epidemiology of Neuromyelitis Optica in the United States

Abstract

Background: Rare diseases require integrated multicenter clinical networks to facilitate clinical research. Neuromyelitis optica (NMO) and NMO spectrum disorders (NMOSDs) are uncommon neuroinflammatory syndromes that are distinct from multiple sclerosis and associated with NMO-IgG, a serologic antibody against aquaporin 4.Objective: To develop a national multicenter NMO clinical consortium and report initial demographic, clinical, and radiographic features of a cohort of patients with NMO/NMOSD in the United States.Design: Review of medical records from patients undergoing evaluation during a 5-year period. We used uniform diagnostic criteria and clinical, laboratory, and neuroimaging definitions to describe the cohort.Setting: Three academic medical centers.Patients: One hundred eighty-seven patients with NMO/ NMOSD.Results: Of the 187 patients included in the analysis, 86 had NMO-IgG-seropositive NMO; 40, NMO-IgGseronegative NMO; and 61, NMO-IgG-seropositive NMOSD. Altogether, 29.4% of our patients were initially misdiagnosed with multiple sclerosis. The average age at onset of NMO/NMOSD was 41.1 years with a strong female predilection, similar to other autoimmune disorders. Nonwhite patients constituted 52.4% of the cohort. The hallmark of NMO and NMOSD is recurrent longitudinally extensive transverse myelitis, but patients with NMO tend to initially present with optic neuritis. Conclusions:A national multicenter consortium to study NMO/NMOSD is feasible and facilitates accurate clinical diagnosis. This network establishes a foundation for determining disease prevalence, translational research, and clinical trials.

Therapeutic plasma exchange for neuromyelitis optica spectrum disorder: A multicenter retrospective study by the ASFA neurologic diseases subcommittee

“…Patients diagnosed with NMO or NMOSD according to the diagnosis criteria proposed in 2006 and 2015 who were treated with TPE during the acute phase of their disease were included in this retrospective study. This study showed a female and Caucasian predisposition to the disease, similar to an epidemiological study performed in the United States by Mealy et al Our patients were slightly older (43.1 years) at presentation than those in the previous study (41.1 years). Most patients (>50%) presented with the core clinical findings of transverse myelitis and optic neuritis, as well as other parameters including contiguous spinal cord lesions, non‐MS brain imaging, and AQ4‐IgG seropositivity.…”

Therapeutic plasma exchange for neuromyelitis optica spectrum disorder: A multicenter retrospective study by the ASFA neurologic diseases subcommittee

“…Patients diagnosed with NMO or NMOSD according to the diagnosis criteria proposed in 2006 and 2015 who were treated with TPE during the acute phase of their disease were included in this retrospective study. This study showed a female and Caucasian predisposition to the disease, similar to an epidemiological study performed in the United States by Mealy et al Our patients were slightly older (43.1 years) at presentation than those in the previous study (41.1 years). Most patients (>50%) presented with the core clinical findings of transverse myelitis and optic neuritis, as well as other parameters including contiguous spinal cord lesions, non‐MS brain imaging, and AQ4‐IgG seropositivity.…”

An Observational Study of Multi-Faceted Demyelinating Disorders

“…Most patients were polysymptomatic. Our findings were similar to the studies by Mealy et al [ 12 ] and Pandit et al [ 13 ]. The studies by Liu et al [ 14 ] and Viswanathan et al [ 15 ] found that the MRI findings of patients with IIDCDs like MS, NMOSD, and ATM deteriorate with time.…”

Clinical Characteristics of Patients With Neuromyelitis Optica Spectrum Disorders With Early Onset

“…It must be highlighted that for the purpose of the present analyses, the "older patients" of this study still developed neuromyelitis optica spectrum disorder well before the expected time, that is, the late third or early fourth decade of life. 3,8 These findings are in accordance with previous publications showing that pediatric-onset neuromyelitis optica spectrum disorder is a debilitating condition requiring optimization of diagnosis and treatment strategies. 9,10 In the present study, 15% of this young population of patients with disease onset before the age of 21 years died.…”

Therapeutic plasma exchange for neuromyelitis optica spectrum disorder: A multicenter retrospective study by the ASFA neurologic diseases subcommittee

“…Patients diagnosed with NMO or NMOSD according to the diagnosis criteria proposed in 2006 and 2015 who were treated with TPE during the acute phase of their disease were included in this retrospective study. This study showed a female and Caucasian predisposition to the disease, similar to an epidemiological study performed in the United States by Mealy et al Our patients were slightly older (43.1 years) at presentation than those in the previous study (41.1 years). Most patients (>50%) presented with the core clinical findings of transverse myelitis and optic neuritis, as well as other parameters including contiguous spinal cord lesions, non‐MS brain imaging, and AQ4‐IgG seropositivity.…”

An Observational Study of Multi-Faceted Demyelinating Disorders

“…Most patients were polysymptomatic. Our findings were similar to the studies by Mealy et al [ 12 ] and Pandit et al [ 13 ]. The studies by Liu et al [ 14 ] and Viswanathan et al [ 15 ] found that the MRI findings of patients with IIDCDs like MS, NMOSD, and ATM deteriorate with time.…”

Clinical Characteristics of Patients With Neuromyelitis Optica Spectrum Disorders With Early Onset

“…It must be highlighted that for the purpose of the present analyses, the "older patients" of this study still developed neuromyelitis optica spectrum disorder well before the expected time, that is, the late third or early fourth decade of life. 3,8 These findings are in accordance with previous publications showing that pediatric-onset neuromyelitis optica spectrum disorder is a debilitating condition requiring optimization of diagnosis and treatment strategies. 9,10 In the present study, 15% of this young population of patients with disease onset before the age of 21 years died.…”

Therapeutic plasma exchange for neuromyelitis optica spectrum disorder: A multicenter retrospective study by the ASFA neurologic diseases subcommittee

“…Patients diagnosed with NMO or NMOSD according to the diagnosis criteria proposed in 2006 and 2015 who were treated with TPE during the acute phase of their disease were included in this retrospective study. This study showed a female and Caucasian predisposition to the disease, similar to an epidemiological study performed in the United States by Mealy et al Our patients were slightly older (43.1 years) at presentation than those in the previous study (41.1 years). Most patients (>50%) presented with the core clinical findings of transverse myelitis and optic neuritis, as well as other parameters including contiguous spinal cord lesions, non‐MS brain imaging, and AQ4‐IgG seropositivity.…”

An Observational Study of Multi-Faceted Demyelinating Disorders

“…Most patients were polysymptomatic. Our findings were similar to the studies by Mealy et al [ 12 ] and Pandit et al [ 13 ]. The studies by Liu et al [ 14 ] and Viswanathan et al [ 15 ] found that the MRI findings of patients with IIDCDs like MS, NMOSD, and ATM deteriorate with time.…”

Clinical Characteristics of Patients With Neuromyelitis Optica Spectrum Disorders With Early Onset

“…It must be highlighted that for the purpose of the present analyses, the "older patients" of this study still developed neuromyelitis optica spectrum disorder well before the expected time, that is, the late third or early fourth decade of life. 3,8 These findings are in accordance with previous publications showing that pediatric-onset neuromyelitis optica spectrum disorder is a debilitating condition requiring optimization of diagnosis and treatment strategies. 9,10 In the present study, 15% of this young population of patients with disease onset before the age of 21 years died.…”