The symptoms of autoimmune encephalitis with antibodies directed against neuronal cell surface antigens are generally reversible by removing the antibodies or the antibody-producing B cells. 1 Although phenotypically pleomorphic, autoimmune encephalitis usually presents as a rapidly progressive encephalopathy but may present with neurologic and psychiatric manifestations without an inflammatory CSF profile 2 or abnormalities on brain MRI. Antibodies directed against dopamine 2 receptor (D2R) have been described in 14 children 3,4 presenting with encephalitis and prominent movement features. While it is debated whether D2R antibodies are pathogenic or bystanders in human disease, 5,6 the response to immunotherapy reported for most patients 7 helps to build the case in favor of their pathogenicity. Clinical case A 16-month-old healthy boy was admitted to the emergency department due to sudden onset of altered mental status and abnormal posturing of his limbs. He showed marked irritability, poor eye contact, sustained flexion of his left limbs, and extension of his right limbs alternating with mixed choreic-dystonic movements of the 4 limbs and mandible. Neurologic examination revealed considerable axial hypotonia, with the patient being unable to sit unassisted, and the patient ceased to communicate verbally, despite having prior typical language development. A recent history of vomiting for the last 2 days was elicited but there was no evidence of fever and bloodwork including ammonia levels had normal results.
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