TY - JOUR
T1 - Overlapping demyelinating syndrome (Neuromyelitis optica spectrum disorders NMOSD with anti-NMDA receptor encephalitis); A case report
AU - Sinani, Ahmed Al
AU - Maawali, Said Al
AU - Alshekaili, Jalila
AU - Kindi, Mahmood Al
AU - Ramadhani, Khalsa Al
AU - Khabouri, Jaber Al
AU - Nadeem, Ahmed
AU - Salti, Abdullah Al
N1 - Funding Information:
We would like to express our sincere gratitude and appreciation to Khoula hospital Radiology Department and to Sultan Qaboos University Hospital, Microbiology and Immunology Department for their valuable contribution and guidance that greatly assisted the completion of this case report.
Publisher Copyright:
© 2020 Elsevier B.V.
PY - 2020/7
Y1 - 2020/7
N2 - Neuromyelitis optica spectrum disorder (NMOSD) represents an evolving spectrum of inflammatory demyelinating central nervous system-based autoimmune diseases; while anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is another severe immune-mediated syndrome that occurs in association with IgG antibodies against the GluN1 subunit of the NMDAR and has been predominantly reported in young females (Dalmau et al., 2008 Dec). Although It has been recognized that anti-NMDAR encephalitis can coexist in the same patient who has serological markers of another autoimmune disease (e.g. neuronal autoantibodies and demyelination AQP4 or MOG antibodies) (Titulaer et al., 2014), rare cases are reported with anti-NMDAR encephalitis that overlap with a demyelinating syndrome; such as neuromyelitis optica spectrum disorders associated with Anti- Aquaporin 4 (AQP4) antibodies (Titulaer et al., 2013). We report here an unusual and rare overlapping autoimmune syndrome in a young Omani female who first presented in 2011 with the clinical and radiological presentations of neuromyelitis optica spectrum disorder (NMOSD) and had a complete recovery. Five years later, she was admitted with the diagnosis of anti-NMDAR encephalitis and was found to have positive serum as well as CSF analyses results for AQP4 and anti-NMDAR antibodies. The patient showed significant improvement, for both clinical syndromes with good response to steroid and immunomodulating therapy.
AB - Neuromyelitis optica spectrum disorder (NMOSD) represents an evolving spectrum of inflammatory demyelinating central nervous system-based autoimmune diseases; while anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is another severe immune-mediated syndrome that occurs in association with IgG antibodies against the GluN1 subunit of the NMDAR and has been predominantly reported in young females (Dalmau et al., 2008 Dec). Although It has been recognized that anti-NMDAR encephalitis can coexist in the same patient who has serological markers of another autoimmune disease (e.g. neuronal autoantibodies and demyelination AQP4 or MOG antibodies) (Titulaer et al., 2014), rare cases are reported with anti-NMDAR encephalitis that overlap with a demyelinating syndrome; such as neuromyelitis optica spectrum disorders associated with Anti- Aquaporin 4 (AQP4) antibodies (Titulaer et al., 2013). We report here an unusual and rare overlapping autoimmune syndrome in a young Omani female who first presented in 2011 with the clinical and radiological presentations of neuromyelitis optica spectrum disorder (NMOSD) and had a complete recovery. Five years later, she was admitted with the diagnosis of anti-NMDAR encephalitis and was found to have positive serum as well as CSF analyses results for AQP4 and anti-NMDAR antibodies. The patient showed significant improvement, for both clinical syndromes with good response to steroid and immunomodulating therapy.
KW - Anti-N-methyl-D-aspartate receptor (anti-NMDAR)
KW - Encephalitis
KW - Intravenous immunoglobulin IVIG
KW - Neuromyelitis optica spectrum disorders (NMOSD)
KW - overlap demyelinating syndrome
KW - Rituximab
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U2 - 10.1016/j.msard.2020.102153
DO - 10.1016/j.msard.2020.102153
M3 - Article
C2 - 32413838
AN - SCOPUS:85084424457
SN - 2211-0348
VL - 42
JO - Multiple Sclerosis and Related Disorders
JF - Multiple Sclerosis and Related Disorders
M1 - 102153
ER -