Distinguishing autoimmune demyelinating diseases from MS
The importance of an accurate diagnosis
While the most common central nervous system (CNS) demyelinating disease is multiple sclerosis (MS), other CNS demyelinating diseases are associated with antibodies to aquaporin-4 (AQP4-IgG) and myelin oligodendrocyte glycoprotein (MOG-IgG), which drive harmful autoimmune responses. Identifying the antibodies associated with autoimmune CNS demyelinating disease enables diagnostic certainty and implementation of an appropriate treatment regimen, which minimizes the risk of relapse and patient disability.
Neuromyelitis optica spectrum disorders
Neuromyelitis optica (NMO) is an inflammatory, CNS demyelinating disease, characterized by severe relapsing attacks of optic neuritis and transverse myelitis. Unlike attacks associated with MS, NMO attacks commonly spare the brain in the early stages.
The spectrum of NMO was traditionally restricted to the optic nerves and the spinal cord. In 2004, Mayo Clinic scientist Vanda Lennon, M.D., Ph.D., reported an antibody that targets aquaporin-4 (AQP4), the water channel on astrocytes, which is a sensitive and specific biomarker for NMO. Since that discovery, a much broader category called NMO spectrum disorders (NMOSD) has evolved.
Myelin oligodendrocyte glycoprotein (MOG)-opathy
Detection of MOG-IgG is diagnostic of MOG-IgG associated disease (MOGAD), which is a CNS inflammatory demyelinating disease where the clinical phenotype (NMO, optic neuritis, transverse myelitis, acute disseminated encephalomyelitis [ADEM]) may be similar, but the immunopathology (astrocytopathy vs. oligodendrogliopathy) and clinical outcome (worse vs. better) are different. Detection of MOG-IgG also predicts disease relapse.
More importantly, MOGAD is distinct from MS and treated differently, with many MS treatments reported to worsen MOGAD.
By the numbers
90%
of patients with NMOSD are initially misdiagnosed with MS1
1/3
of AQP4-IgG-negative patients are positive for MOG-IgG2
50%
probability of second optic neuritis episode within one year of initial attack in AQP4-positive patients3
Ensuring better patient outcomes
Due to significant overlap in the clinical phenotypes, we recommend testing for AQP4-IgG and MOG-IgG at the same time. This synchronized testing method allows for a faster diagnosis and treatment plan decision for your patient.
Elevating outcomes through early detection
Learn more about NMOSD and the critical importance of sensitive and specific AQP4-IgG laboratory testing for early diagnosis and treatment in this “Hot Topic” presentation given by Sean Pittock, M.D.
Similar characteristics, different treatment
Early diagnosis
is critical
FACS: A superior method of testing
Mayo Clinic has developed the only fluorescence activated cell sorting (FACS) live cell-binding assay currently available in the U.S. for antibody detection of AQP4 and MOG. FACS is recommended by international leaders in neuroimmunology for its increased sensitivity and specificity.
Key testing
Additional resources
In this month’s “Hot Topic,” Sean Pittock, M.D., explains Neuromyelitis Optica Spectrum Disorder (NMOSD) and the critical importance of a sensitive and specific AQP4-IgG laboratory test for early diagnosis and treatment.
Antibodies to aquaporin-4 and myelin oligodendrocyte glycoprotein (MOG) are recently described biomarkers seen in a subset of atypical optic neuritis which have revolutionized our understanding of the condition. In this “Hot Topic,” my colleague, Dr. John Chen, will review these advances and how they impact the clinical care of our patients with optic neuritis.
"This study offers hope to patients, since each attack in NMO can cause loss of visual or motor function," says Sean Pittock, M.D., a Mayo Clinic neurologist and first author.
This "Specialty Testing" webinar will provide an overview of novel biomarker discoveries and advances being made in the study of autoimmune gliopathies.
References