Further investigations searching for tumor were normal. is shown awake without dyskinesias, but he has drowsiness and difficulty following verbal commands NVS-CRF38 to smooth pursuit during examination. Segment 3. A similar pattern of night\time oromandibular dyskinesias is observed during sleep. MDC3-4-884-s002.mpg (5.8M) GUID:?F03FD9E3-CC09-4BD4-A35B-C8CCF6D88B50 Abstract Background The syndrome of anti\ em N /em \methyl\D\aspartate receptor encephalitis is classically associated with a combination of limbic dysfunction, dysautonomia, central hypoventilation and HSP90AA1 movement disorders. On clinical grounds, the diagnosis is often supported by the presence of generalized dyskinesias in a patient with encephalopathy and catatonic signs. Orofacial dyskinesias have been recognized as characteristic of the disorder but can be absent in some patients. Pure psychiatric syndromes without movement disorders have also been described. Methods and Results The authors describe 2 male patients who presented with prominent neuropsychiatric symptoms without movement disorders during wakefulness but isolated orofacial dyskinesias during sleep. In 1 patient, this observation supported the early introduction of immunotherapy; and, in both patients, the clinical outcome was excellent. Conclusions Careful history and nocturnal examination may reveal orofacial dyskinesias in patients with em N /em \methyl\D\aspartate receptor encephalitis who have apparent pure psychiatric manifestations. strong class=”kwd-title” Keywords: autoimmune encephalitis, orofacial dyskinesias, em N /em \methyl\D\aspartate (NMDA) receptor encephalitis View Supplementary Video 1 View Supplementary Video 2 The syndrome of anti\ em N /em \methyl\D\aspartate receptor (NMDAR) encephalitis is characterized by limbic dysfunction, autonomic instability, hypoventilation, and movement disorders.1 Recently, it has been recognized that the clinical spectrum encompasses isolated psychiatric presentations.2 Orofacial dyskinesia is a distinctive feature of NMDAR encephalitis, and its occurrence increases the likelihood of the diagnosis in the context of psychosis or encephalopathy; however, not all patients have this finding.1, 3 In a series of patients with NMDAR encephalitis, we have observed that some patients can present with prominent neuropsychiatric symptoms, and orofacial dyskinesias can occur almost exclusively during the night, indicating that this sign could be overlooked. Here, we describe 2 patients and provide video documentation of this observation. Case 1 A 19\year\old male presented with subacute onset of abnormal behavior characterized by intermittent, complex auditory and visual hallucinations. He was fluctuating between periods of insomnia and somnolence, and NVS-CRF38 he had 3 generalized seizures before admission. On examination, he was drowsy but was able to follow simple commands. Cranial and motor examination was unremarkable. He scored 5 points on the Bush\Francis Catatonia Rating Scale (BFCRS), with the presence of immobility/stupor, stereotypies, staring, verbigeration, and dysautonomia. An electroencephalogram (EEG) revealed generalized slowing, and magnetic resonance imaging was normal. Cerebrospinal fluid (CSF) analysis revealed 18 monocytes and normal protein and glucose content. He was started with acyclovir to cover herpes simplex virus and methylprednisolone. Provisional videos were NVS-CRF38 obtained before and during treatment. However, the mother reported that, during the night, he had involuntary facial movement, so she documented these events (Video S1). The movements were present intermittently but were frequent during the course of sleep throughout the night and early morning. After his hospital discharge, CSF and serum antibody tests were positive for NMDAR antibody. Antibodies for \amino\3\hydroxy\5\methyl\4\isoxazolepropionic acid (AMPA), \aminobutyric acid B (GABA B), metabotropic glutamate receptors 1 and 5 (mGluR1 and mGluR5, respectively), leucine\rich glioma inactivated 1 (LGI1), and contactin\associated protein like 2 (CASPR2) were negative in both CSF and serum samples. Three months after treatment, he scored 27 on the Montreal Cognitive Assessment and 0 on the modified Rankin scale. Further investigations searching for tumor were normal. Antibody titer was not available, and we did not conduct longitudinal tests for NMDAR antibodies. Case 2 A 35\year old man presented with a 3\week history of generalized anxiety, auditory hallucinations, and insomnia. At examination, he was alert and able to follow commands; however, at times, he was also drowsy. Some phrases could possibly be repeated by him but with slurred talk. Cranial, motor power, and sensory examinations had been regular. Before treatment, his BFCRS rating was 11, and he exhibited stupor, mutism, looking, stereotypy, negativism, light rigidity, and cardiovascular dysautonomia. He could walk with incomplete assistance. The EEG uncovered moderate, generalized slowing, and human brain and CSF magnetic resonance imaging were unremarkable. Given our knowledge with the prior patient, we requested video records through the complete nighttime, which was supplied by his sibling during entrance and showed the current presence of orofacial dyskinesias (Video S2). After treatment with methylprednisolone and intravenous immunoglobulin, his BFCRS rating was 3. 90 days after hospital release, he have scored 23 over the Montreal Cognitive Evaluation and 1 over the improved Rankin scale. NMDAR antibodies were within serum and CSF. Antibodies for AMPA, GABA B, mGluR1, mGluR5, LGI1, and CASPR2 had been detrimental; and longitudinal assessment had not been performed. Debate The scientific display of NMDAR encephalitis could be tough and mixed to create on scientific grounds, early in especially.