All's well that ends well? Long-term course of a patient with anti-amphiphysin associated limbic encephalitis.

Anti-amphiphysin associated limbic encephalitis (LE) is a paraneoplastic autoimmune disorder. The initial clinical presentation features seizures, cognitive and neuropsychiatric symptoms. We present the case of a 25-year-old female patient hospitalized after four consecutive tonic-clonic seizures, followed by confusion, psychotic symptoms, nonconvulsive seizure series, and severe global amnesia. Diagnostic workup revealed anti-amphiphysin associated LE without a tumor. MRI and PET indicated inflammatory processes affecting the bilateral mesial temporal structures more pronounced on the left side. Antiseizure medication, benzodiazepines, and immunotherapy resulted in rapid seizure cessation. Subsequent MRI and PET indicated left hippocampal sclerosis and a left mesial temporal hypometabolism. Executive dysfunction resolved in the following weeks. Global amnesia persisted for almost three months. Two years later, episodic memory was normal with residual visual memory impairments. While this patient's seizure and cognitive outcome has been favorable, behavioral problems persisted long after disease onset. The persisting behavioral problems and subsequent MRI evidence (13 years after onset) of a swollen right amygdala indicated a possible relapse. This case report illustrates the importance of early diagnosis of LE for best clinical management. Antiseizure medication and immunotherapy led to seizure freedom and almost complete recovery of cognition. However, long-lasting neuropsychiatric symptoms and possible recurrent inflammation highlight the need for a multimodal long-term monitoring of such patients to rule out a relapse.

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© 2022 The Author(s).

Déclaration de conflit d'intérêts

The authors declare no conflict of interest with regard to the current work. J. Taube has nothing to disclose; Dr. Witt reports personal fees from Eisai, outside the submitted work; Dr. Baumgartner reports personal fees from Eisai and UCB, outside the submitted work; Prof. Dr. Helmstaedter reports personal fees from Precisis, Eisai, UCB, and GW, outside the submitted work.