NMOSD and other autoimmune conditions such as MOGAD can present as intracranial infections during early onset. This study attempts to find the key ways that these conditions differ to allow for better clinical diagnosis.
Many autoimmune diseases of the central nervous system, including NMOSD, autoimmune glial fibrillary acidic protein astrocytopathy (A-GFAP-A), and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and aquaporin-4-immunoglobulin-G-positive neuromyelitis optica spectrum disorders (AQP4-IgG+NMOSD) initially present symptoms that resemble intracranial infection. Furthermore, these diseases are difficult to distinguish from each other during these initial phases, when the detection of autoantibodies is lacking. This study, published in Multiple Sclerosis and Related Disorders, summarizes the immunological and radiological characteristics of autoantibodies identified using cell-based assays for patients exhibiting early-onset symptoms of A-GFAP-A, MOGAD, and AQP4-IgG+NMOSD.
This study relied on data from a cohort in a single center. All patients included in the study exhibited symptoms of intracranial infection as initial symptoms developed. Overall, there were 9 patients with A-GFAP-A, 17 with MOGAD, and 11 with AQP4-IgG+NMOSD. Symptom variations were found between patients with AQP4-IgG+NMOSD and the other diseases, including an increased rate of tremors and positive Kernig’s sign in the A-GFAP-A group compared to the other groups, a higher increase in white blood cell count in cerebrospinal fluid in the A-GFAP-A group compared to the AQP4-IgG+ NMOSD group, and a larger increase in lactate dehydrogenase, lactic acid, and and IgG, IgM, and IgA in the A-GFAP-A group compared to the MOGAD group. These results indicate that fine-tuned differentiation remains difficult.
The authors conclude by stating that their study demonstrates a number of distinct features of this disease, including tremor symptoms, higher cerebrospinal fluid immunological profiles, bilateral symmetrical lesions in ganglia, and diffuse meningeal enhancement are more commonly seen in A-GFAP-A, while ADEM-like lesions are more typical of MOGAD. Although these results provide insights that can be very beneficial for clinically differentiating between these conditions, they do not allow for unequivocal diagnosis during these early stages of disease.
Xiao, J., Zhang, S. Q., Chen, X., Tang, Y., Chen, M., Shang, K., Deng, G., Qin, C., & Tian, D. S. (2022). Comparison of clinical and radiological characteristics in autoimmune GFAP astrocytopathy, MOGAD and AQP4-IgG(+) NMOSD mimicking intracranial infection as the initial manifestation. Mult Scler Relat Disord, 66, 104057. https://doi.org/10.1016/j.msard.2022.104057