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, f...
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| Format: | Article |
| Language: | English |
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Elsevier
2022-01-01
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| Series: | Epilepsy & Behavior Reports |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2589986422000119 |
| _version_ | 1811302888270462976 |
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| author | Julia Taube Juri-Alexander Witt Tobias Baumgartner Christoph Helmstaedter |
| author_facet | Julia Taube Juri-Alexander Witt Tobias Baumgartner Christoph Helmstaedter |
| author_sort | Julia Taube |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-13T07:37:29Z |
| format | Article |
| id | doaj.art-e86e96cd27e64a58834abb9b38163f60 |
| institution | Directory Open Access Journal |
| issn | 2589-9864 |
| language | English |
| last_indexed | 2024-04-13T07:37:29Z |
| publishDate | 2022-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Epilepsy & Behavior Reports |
| spelling | doaj.art-e86e96cd27e64a58834abb9b38163f602022-12-22T02:56:04ZengElsevierEpilepsy & Behavior Reports2589-98642022-01-0118100534All’s well that ends well? Long-term course of a patient with anti-amphiphysin associated limbic encephalitisJulia Taube0Juri-Alexander Witt1Tobias Baumgartner2Christoph Helmstaedter3Department of Epileptology, University Hospital Bonn (UKB), Bonn, GermanyDepartment of Epileptology, University Hospital Bonn (UKB), Bonn, GermanyDepartment of Epileptology, University Hospital Bonn (UKB), Bonn, GermanyCorresponding author at: Department of Epileptology, University Hospital Bonn (UKB), Venusberg-Campus 1, D-53127 Bonn, Germany.; Department of Epileptology, University Hospital Bonn (UKB), Bonn, GermanyAnti-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.http://www.sciencedirect.com/science/article/pii/S2589986422000119Anti-amphiphysin associated limbic encephalitisAutoimmune epilepsyBehaviorLong-term outcomeAmnesia |
| spellingShingle | Julia Taube Juri-Alexander Witt Tobias Baumgartner Christoph Helmstaedter All’s well that ends well? Long-term course of a patient with anti-amphiphysin associated limbic encephalitis Epilepsy & Behavior Reports Anti-amphiphysin associated limbic encephalitis Autoimmune epilepsy Behavior Long-term outcome Amnesia |
| title | All’s well that ends well? Long-term course of a patient with anti-amphiphysin associated limbic encephalitis |
| title_full | All’s well that ends well? Long-term course of a patient with anti-amphiphysin associated limbic encephalitis |
| title_fullStr | All’s well that ends well? Long-term course of a patient with anti-amphiphysin associated limbic encephalitis |
| title_full_unstemmed | All’s well that ends well? Long-term course of a patient with anti-amphiphysin associated limbic encephalitis |
| title_short | All’s well that ends well? Long-term course of a patient with anti-amphiphysin associated limbic encephalitis |
| title_sort | all s well that ends well long term course of a patient with anti amphiphysin associated limbic encephalitis |
| topic | Anti-amphiphysin associated limbic encephalitis Autoimmune epilepsy Behavior Long-term outcome Amnesia |
| url | http://www.sciencedirect.com/science/article/pii/S2589986422000119 |
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