Autoimmune encephalitis with anti-NMDAR antibodies – variety of clinical manifestations

Abstract

The aim of this study was to review the literature on the topic of diversity of clinical symptoms of autoimmune encephalitis with the presence of antibodies against N-methyl-D-aspartate receptors (anti-NMDAR antibodies), the diagnostic process, and treatment. The incidence of the disease is approximately 4% of all reported cases of encephalitis. Autoimmune encephalitis with anti-NMDAR antibodies manifests as a rapidly progressive encephalopathy of acute or subacute onset. It usually develops over a period of six weeks. To diagnose the disease, it is necessary to confirm a minimum of four out of six symptoms, such as behavioural or cognitive impairment, speech impairment, epileptic seizures, movement disorders, disturbances of consciousness, and autonomic dysfunction. The diagnostic process is aided by additional examinations including electroencephalography, cerebrospinal fluid examination, magnetic resonance imaging, and laboratory tests (e.g. determination of titre of antineuronal antibodies). It is not uncommon for symptoms to indicate the possibility of co-occurrence of several psychiatric conditions at the same time, leading to a delay in making a correct diagnosis. Moreover, it should be remembered that anti-NMDAR encephalitis often displays the characteristics of a paraneoplastic syndrome, and particularly often coexists with ovarian teratoma. Consequently, this option should be included in the diagnostic process. Autoimmune encephalitis with anti-NMDAR antibodies can manifest itself in many ways, often with multiple neurological and psychiatric symptoms. Quick diagnosis, as well as early targeted treatment, increase the chance of success of the therapeutic process.