Objective: The goal is to learn from different presentations of Autoimmune Encephalitis (AIE) so that we consider the diagnosis more quickly, pursue the work-up judiciously and achieve best patient outcomes. We discuss two very different presentations of movement disorders that both led to recovery but differed significantly in the time to recovery.
Background: Autoimmune Encephalitis often presents with neuropsychiatric symptoms, cognitive deficits, altered level of consciousness, dysautonomia, psychosis and seizures. Parkinsonism and dystonia are less common presentations and as such, may not prompt an evaluator to consider AIE at initial presentation. Time to diagnosis has been shown likely most critical for prompt initiation of treatment and start to recovery. We encountered two patients at the opposite ends of the spectrum of time to diagnosis and treatment initiation and try to learn from their differences and commonalities.
Method: Review of the cases of two young women in our clinic was undertaken. The first (Patient 1) was initially evaluated elsewhere and presented to us 1 year after onset of symptoms. The second (Patient 2) presented to our Emergency Department (ED) ten days after a tetanus vaccination and precipitous development of symptoms.
Results: Patient 1 presented one year after onset of focal dystonic limb movements that progressed to a severe choreiform/ballistic disorder, eventually disabling and difficult to work up. Mild pleocytosis of cerebrospinal fluid was the only diagnostic clue of AIE and treatment with intravenous immunoglobulin (IVIG) was started empirically at 18 months after onset of symptoms. Recovery evolved over two years and the patient was relieved of all symptoms at 4.5 years after initial onset. Patient 2 presented to the ED with 4 days of confusion and subtle dystonic limb and facial movements. A high index of suspicious prompted workup and treatment with sequential high dose steroids and IVIG even before the confirmatory panel was positive for NMDA-receptor antibodies. All symptoms resolved after 6 weeks of treatment and supportive care.
Conclusion: Two vastly different time courses to diagnosis are presented here to show the complexity with which AIE can present with movement disorders. A high index of suspicion and move to early treatment is required for prompt recovery but also perseverance and patience for recovery can be rewarded over time.
To cite this abstract in AMA style:L. Jaffe. Complex movement disorder presentations of autoimmune encephalitis – recovery at 4 weeks versus 4 years [abstract]. Mov Disord. 2023; 38 (suppl 1). https://www.mdsabstracts.org/abstract/complex-movement-disorder-presentations-of-autoimmune-encephalitis-recovery-at-4-weeks-versus-4-years/. Accessed March 5, 2024.
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MDS Abstracts - https://www.mdsabstracts.org/abstract/complex-movement-disorder-presentations-of-autoimmune-encephalitis-recovery-at-4-weeks-versus-4-years/