Overlapping demyelinating syndrome (Neuromyelitis optica spectrum disorders NMOSD with anti-NMDA receptor encephalitis); A case report

Autor: Mahmood Al Kindi, Khalsa Al Ramadhani, Abdullah Al Salti, Ahmed Al Sinani, Jaber Al Khabouri, Jalila Alshekaili, Ahmed Sameer Nadeem, Said M. Al Maawali
Rok vydání: 2020
Předmět:
Zdroj: Multiple Sclerosis and Related Disorders. 42:102153
ISSN: 2211-0348
DOI: 10.1016/j.msard.2020.102153
Popis: 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.
Databáze: OpenAIRE
Externí odkaz: