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Abstract Details

Anti-NMDA Receptor Encephalitis with PSH: An Under-Recognized Association with Treatment Implications?
Autonomic Disorders
P03 - (-)
028
BACKGROUND: While autonomic instability occurs as part of anti-N-methyl D-aspartate (anti-NMDA) receptor encephalitis, anti-NMDA receptor encephalitis is not a recognized cause of the clinical syndrome of paroxysmal sympathetic hyperactivity (PSH). We present a case of anti-NMDA receptor encephalitis in which PSH was a cardinal feature.
DESIGN/METHODS: This is a case report of a single patient who developed paroxysmal sympathetic hyperactivity (PSH) after she was diagnosed with NMDA receptor encephalitis.
RESULTS: A 31-year-old woman had a generalized tonic-clonic seizure and then developed progressively worsening neuropsychiatric symptoms, including mania, hallucinations, echolalia, and suicidal ideation. Diagnostic work-up revealed anti-NMDA receptor antibodies detected in the serum and in the cerebrospinal fluid (CSF). One week after symptom onset, the patient experienced intermittent episodes of sinus tachycardia, hypertension, tachypnea, diaphoresis and extensor posturing. The episodes were both spontaneous and stimulus responsive (for example, during endotracheal suctioning). The episodes, consistent with PSH, were initially treated with dexmedetomidine, which was titrated to effect. Gabapentin and propranolol were added later for symptom control, but eventually weaned off as her symptoms abated approximately six weeks into the illness.
CONCLUSIONS: We believe that the autonomic instability associated with anti-NMDA receptor encephalitis may often be PSH. PSH often goes unrecognized in patients outside of the setting of TBI, thus specific PSH management strategies may be overlooked in other illnesses. Anti-NMDA receptor encephalitis may represent the functional companion to the structural lesion encountered in TBI. Recognition of PSH in this setting is important to guide the management of the autonomic instability, but may also have mechanistic implications.
Authors/Disclosures
Courtney Takahashi, MD, FAAN (John Muir Medical Center)
PRESENTER
Dr. Takahashi has received personal compensation in the range of $0-$499 for serving as a Consultant for Pfizer . Dr. Takahashi has received personal compensation in the range of $0-$499 for serving as an officer or member of the Board of Directors for Insight Neuro LLC . Dr. Takahashi has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Keches Law Group. Dr. Takahashi has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Brain IDX . Dr. Takahashi has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for McGowan Law. Dr. Takahashi has received personal compensation in the range of $500-$4,999 for serving as a Employee, Founder with Tora Connections. Dr. Takahashi has a non-compensated relationship as a Committee Member with 好色先生 that is relevant to AAN interests or activities.
H. E. Hinson, MD, MCR, FAAN (UCSF/Zuckerberg San Francisco General Hospital) Dr. Hinson has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology. Dr. Hinson has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for American Heart Association.
Dennis N. Bourdette, MD, FAAN Dr. Bourdette has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Magellan Health Care. Dr. Bourdette has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Best Doctor's Inc. Dr. Bourdette has stock in Authobahn Therapeutics. Dr. Bourdette has received intellectual property interests from a discovery or technology relating to health care.
Nizar Souayah, MD, FAAN (NJMS) Dr. Souayah has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Takeda. Dr. Souayah has received publishing royalties from a publication relating to health care.