Anti-NMDA receptor encephalitis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Historical Perspective
Anti-NMDA encephalitis, initially documented in a case report dating back to 1830, is an autoimmune condition marked by neuropsychiatric manifestations. In some instances, the presence of an ovarian teratoma is observed. The case of Susannah Cahalan played a significant role in raising public awareness regarding this disorder.
Classification
Anti-NMDA encephalitis can be categorized into three distinct subtypes based on the timing of onset and duration of the illness.
Pathophysiology
Anti-NMDA receptor encephalitis is an autoimmune disorder characterized by the presence of autoantibodies directed against the GluN1 subunit of the NMDA receptor. The presence of tumors, particularly ovarian teratomas in women and testicular teratomas or lung carcinomas in men, is frequently associated with the condition. Dysregulation of NMDA receptors contributes to the development of diverse neurological and psychiatric symptoms.
Causes
In anti-NMDA receptor encephalitis, autoantibodies targeting the GluN1 subunit of the NMDA receptor are present. These antibodies lead to internalization of NMDA receptors, disrupting synaptic plasticity and causing a range of neurological and psychiatric symptoms. Various causes of the antibodies include , infections, vaccinations and tumors
Differentiating Anti-NMDA receptor encephalitis from other Diseases
In the differential diagnosis of anti-NMDA encephalitis, other autoimmune encephalitides with specific antibody associations should be considered, including those associated with antibodies against AMPA receptors, GABA-B receptors, LGI1, Caspr2, and MOG. Clinical and serological evaluations are crucial for accurate diagnosis and appropriate management.
Epidemiology and Demographics
Encephalitis has an annual incidence of 2-3 per 100,000, with 40% being infectious, 40% of unknown cause, and 20% immune-mediated. Anti-NMDA receptor encephalitis and VGK Complex antibody-positive encephalitis are major immune-mediated categories. It is more common in women and younger patients.
Risk Factors
Risk factors for anti-NMDA encephalitis include female predominance, higher incidence in young individuals, and increased likelihood of underlying teratoma in African-Americans and teratomas in Asians.
Screening
Natural History, Complications and Prognosis
Anti-NMDA encephalitis typically exhibits a characteristic trajectory, wherein initial neuropsychiatric manifestations advance to neurological symptoms.
The acute phase of the condition can be severe and protracted, extending over a period of weeks to months. Timely initiation of immunotherapy and surgical intervention for tumor removal have shown to enhance prognosis. Gradual amelioration of symptoms occurs, although the duration of recovery varies. Long-term neurological and psychiatric recuperation may necessitate several months to years, with the possibility of relapses. Mortality rates are associated with complications such as infections, cardiac arrest, and respiratory distress. Favorable prognostic indicators include early intervention, milder symptomatology, and resection of associated tumors.
Diagnosis
History and Symptoms
Anti-NMDA receptor encephalitis presents with neuropsychiatric manifestations, neurological symptoms, altered consciousness, and a subacute time frame. Early recognition is important for prompt intervention.
Physical Examination
The physical examination findings in Anti-NMDA encephalitis are non-specific. Patients may exhibit symptoms of widespread encephalopathy, altered states of consciousness, orofacial dysostosis, dyskinesis, dystonia, and autonomic storming. Neurological examination may reveal signs of diffuse cerebral dysfunction, including reflex abnormalities and mild ataxia.
Laboratory Findings
In Anti-NMDA encephalitis, cerebrospinal fluid (CSF) analysis often shows mildly elevated white blood cell count, modestly increased protein levels, and the presence of oligoclonal bands. CSF-specific tests like IgG index and oligoclonal band tests can provide diagnostic value.