The most common etiology of autoimmune encephalitis (AE) in children and young adults is anti-N-methyl-D-aspartate receptor encephalitis (ANMDARE). ANMDARE commonly presents within a few weeks following a viral prodrome with acute or subacute neuropsychiatric symptoms including anxiety, agitation, mania, psychosis, and insomnia as well as neurocognitive symptoms, such as new memory deficits, speech/language disturbance, and executive dysfunction. This is soon followed by frank neurological symptoms, including movement disorder, dysautonomia, and seizures. Catatonia is very common during the course of illness and antipsychotic intolerance is a common feature. Many individuals with ANMDARE require intensive care to manage severe dysautonomia, catatonia, and neurobehavioral symptoms. Rapid identification and neuroimmunological intervention, along with neuropsychiatric symptom management, are key. Now, better-characterized, longer-term outcomes are being increasingly understood, including the presence of persistent deficits such as frontal executive dysfunction, and other neuropsychiatric symptoms. Individuals with a history of ANMDARE must be monitored for the possibility of relapse. This condition can mimic primary psychiatric disorders, the misidentification of which can delay care. This is the case of a young man and his journey through initial symptomatology, diagnostic complexity, months of intensive complex neuropsychiatric and medical treatment, and then gradual, painstaking return to function by means of multimodal neuropsychiatric and therapeutic intervention. Seven years after the initial reporting of his history, he provides a follow-up description of his experience. This case exemplifies the need for clinicians of a broad range of specialties to appreciate the presentation, course, management, and outcomes of this increasingly recognized condition and to engage in multidisciplinary, team-based care.

错误:搜索内容不能为空,请输入英文关键词
错误:关键词超出字数限制,请精简
高级检索

When the Body Attacks the Brain: Autoimmune Encephalitis in the Short-, Medium-, and Long-Term

  • Jordan L. Nordquist,
  • Owais Tirmizi,
  • Siddhartha S. Nadkarni,
  • Aaron J. Hauptman

摘要

The most common etiology of autoimmune encephalitis (AE) in children and young adults is anti-N-methyl-D-aspartate receptor encephalitis (ANMDARE). ANMDARE commonly presents within a few weeks following a viral prodrome with acute or subacute neuropsychiatric symptoms including anxiety, agitation, mania, psychosis, and insomnia as well as neurocognitive symptoms, such as new memory deficits, speech/language disturbance, and executive dysfunction. This is soon followed by frank neurological symptoms, including movement disorder, dysautonomia, and seizures. Catatonia is very common during the course of illness and antipsychotic intolerance is a common feature. Many individuals with ANMDARE require intensive care to manage severe dysautonomia, catatonia, and neurobehavioral symptoms. Rapid identification and neuroimmunological intervention, along with neuropsychiatric symptom management, are key. Now, better-characterized, longer-term outcomes are being increasingly understood, including the presence of persistent deficits such as frontal executive dysfunction, and other neuropsychiatric symptoms. Individuals with a history of ANMDARE must be monitored for the possibility of relapse. This condition can mimic primary psychiatric disorders, the misidentification of which can delay care. This is the case of a young man and his journey through initial symptomatology, diagnostic complexity, months of intensive complex neuropsychiatric and medical treatment, and then gradual, painstaking return to function by means of multimodal neuropsychiatric and therapeutic intervention. Seven years after the initial reporting of his history, he provides a follow-up description of his experience. This case exemplifies the need for clinicians of a broad range of specialties to appreciate the presentation, course, management, and outcomes of this increasingly recognized condition and to engage in multidisciplinary, team-based care.