Autoimmune Encephalopathy for Psychiatrists: When to Suspect Autoimmunity and What to Do Next

Abstract

Objective: To provide a critical review of autoimmune encephalopathy-broadly defined as neuropsychiatric features directly related to an autoimmune process-relevant for psychiatric practice.

Methods: We consulted rheumatology textbooks to define the scope of autoimmune conditions and identified recent reviews of rheumatic conditions, autoimmune vasculitis, and autoimmune encephalitis. We integrated these with primary reports to provide a clinically relevant overview of autoimmune encephalopathy. We focus on clinical features that should raise suspicion for autoimmunity.

Results: Despite outlying conditions, 2 categories of autoimmune encephalopathy are described: (1) neuropsychiatric symptoms associated with rheumatic conditions and (2) antibody-associated autoimmune encephalitis. Rheumatic conditions principally include connective tissue disease and other vasculitides. These may present variously such as with unexplained delirium, cognitive decline, or depression. Autoimmune encephalitis may be diffuse or localized as in limbic, brainstem, or basal ganglia encephalitis. Unexplained delirium, psychosis, catatonia, strokes, and seizures are among common presenting symptoms.

Conclusions: Prompt identification and management of autoimmunity are critical for optimal outcomes. The fact that undiagnosed and, therefore, untreated autoimmunity leads to debilitation demands vigilance for these conditions. Close attention to the unusual nature and course of neuropsychiatric symptoms, associated neurological features, and review of systems as reviewed here should guide the skillful clinician.

Keywords: anti-NMDA receptor; autoimmune encephalopathy; encephalitis; limbic encephalitis; paraneoplastic encephalitis.; rheumatic disease.