Abstract
Pediatric catatonia is a rare and life-threatening syndrome. Approximately 20 percent of juvenile catatonia is associated with such organic causes as genetic, neurometabolic, and autoimmune disorders. Specific treatments are available for some of these conditions and can drastically improve catatonic symptoms if high-dose benzodiazepines or ECT are insufficient. Systemic autoimmune disorders (lupus, Hashimoto encephalopathy, anti-N-methyl-d-aspartate [anti-NMDA], and anti-gamma-aminobutyric acid [anti-GABA] autoimmune encephalitis) are likely to include catatonia associated with other severe mental illness symptoms (psychosis, mania). Some autoimmune disorders may occur in the context of genetic disorders. Autoantibody-negative but probable autoimmune encephalitis may also be associated with these symptoms. Catatonia may be a marker of a more severe and resistant form of autoimmune encephalitis. To facilitate decision making, we formulated a causality assessment score (CAUS) using a stepwise approach and an immunosuppressive therapeutic challenge. Our objectives were to validate the CAUS and define its threshold, retrospectively, for an accurate distinction between organic catatonia and nonorganic catatonia and specifically between autoimmune catatonia and nonorganic catatonia.
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