Anti-NMDA receptor encephalitis overview: Difference between revisions

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==Natural History, Complications and Prognosis==
==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==
==Diagnosis==

Revision as of 18:03, 13 May 2023

Anti-NMDA receptor encephalitis Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Anti-NMDA receptor encephalitis from Other Diseases

Epidemiology and Demographics

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Natural History, Complications, and Prognosis

Diagnosis

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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

Physical Examination

Laboratory Findings

Electrocardiogram

Chest X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Surgery

Medical Therapy

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

References

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