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

Taube, Julia; Baumgartner, Tobias; Helmstaedter, Christoph

doi:10.1016/j.ebr.2022.100534

Cited by 3 publications

(4 citation statements)

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“…By tracking for 13 years, Taube et al. observed a similar outcome in a patient who was treated promptly with immunotherapy ( 29 ). As in other forms of AE, anti-amphiphysin encephalitis presenting with acute symptomatic seizures has a lower risk of subsequent unprovoked seizure development, which perhaps explains the disappearance of seizures in most patients.…”

Section: Discussionmentioning

confidence: 92%

Anti-amphiphysin encephalitis: Expanding the clinical spectrum

et al. 2023

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ObjectiveAn analysis of the clinical features of autoimmune encephalitis accompanied by anti-amphiphysin antibodies.MethodsThe data of encephalitis patients with anti-amphiphysin antibodies were retrospectively evaluated, including demographics, neurological and laboratory findings, imaging, treatment, and prognostic predictions.ResultsTen patients aged between 29 and 78 years (median age 52 years) were included. The male: female ratio was 4:6. Limbic encephalitis was found in nine patients while epileptic seizures were present in seven patients. All patients showed anti-amphiphysin antibody positivity in sera while one ninth was positive for CSF antibody. The EEG findings were abnormal, including reductions in background activity, and the presence of diffuse slow waves, sharp waves, and spikes and waves. Five patients showed signs of increased T2 signals in the medial temporal lobe on MRI while PET showed either hyper- or hypo-metabolic changes in several brain regions, including the temporal lobe, hippocampus, basal ganglia, frontal and parietal cortices. Nine of ten patients were treated with immunotherapy, with improvements of varying degrees. There was a significant reduction in seizure frequency, and all patients were seizure-free at last follow-up.ConclusionAutoimmune encephalitis with anti-amphiphysin antibodies has a variety of clinical manifestations. The most common symptom is limbic encephalitis. Although relief from seizures can be achieved relatively easily, many patients suffer psychiatric, cognitive, and sleep sequelae. The disease was found to be associated with a lower incidence of cancer than has been previously reported for paraneoplastic neurologic syndromes.

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Section: Discussionmentioning

confidence: 92%

Anti-amphiphysin encephalitis: Expanding the clinical spectrum

et al. 2023

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“…Anti‐amphiphysin encephalitis is a less common autoimmune neurological disorder. A patient presented as vestibulocerebellar syndrome with hypermetabolism in the vestibulocerebellum and adjacent vermis, while another patient developed limbic encephalitis showing hypermetabolism in the MTL 37,38 . Paraneoplastic limbic encephalitis (PLE) is a rare neurological disorder manifesting memory dysfunction, epilepsy, and psychiatric abnormalities.…”

Section: Visual Assessment Of Brain 18f‐fdg‐pet Metabolic Patterns By...mentioning

confidence: 99%

“…A patient presented as vestibulocerebellar syndrome with hypermetabolism in the vestibulocerebellum and adjacent vermis, while another patient developed limbic encephalitis showing hypermetabolism in the MTL. 37,38 Paraneoplastic limbic encephalitis (PLE) is a rare neurological disorder manifesting memory dysfunction, epilepsy, and psychiatric abnormalities. Seventy percent of PLE cases occur in association with small cell lung carcinomas and testicular germ cell tumors.…”

Section: Other Intracellular Antibody-associated Encephalitismentioning

confidence: 99%

Positron emission tomography in autoimmune encephalitis: Clinical implications and future directions

Liu

et al. 2022

Acta Neuro Scandinavica

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Autoimmune encephalitis is an immune-mediated inflammatory disease of the central nervous system caused by abnormal immune response against surface or intracellular antigens. 1 In addition to the previously discovered classical paraneoplastic encephalitisassociated antibodies, a variety of autoimmune encephalitis-related antibodies have been reported in recent years, including antibodies targeting N-methyld-aspartate receptor (NMDAR), alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), leucine-rich glioma inactivated 1 (LGI1), contactin-associated proteinlike 2 (CASPR2), gamma-aminobutyric acid receptor (GABAR) A/B, dipeptidyl-peptidase-like protein-6 (DPPX), glycine receptor (GlyR), glutamic acid decarboxylase 65 (GAD65), dipeptidyl-peptidase-like protein-6 (DPPX), IgLON5 et al. 2 The clinical manifestations, severity, and prognosis with specific antibodies are different, but in general, early detection and early treatment are the principles to handle this disease. However, it has to be admitted that there are still difficulties in the early diagnosis of autoimmune encephalitis, although we have made improvements and revision in the diagnostic criteria based on the clinical criteria defined by Graus et al. 3 in 2016 to avoid excessive reliance on the detection of autoimmune antibodies from serum or cerebrospinal fluid (CSF). The problems focus on the heterogeneity of clinical manifestations, low positive rate of imaging evidence, and lack of specificity of EEG. For example, autoimmune encephalitis may involve

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“…A number of the cases stress the importance of monitoring more than just cognitive function in a comprehensive neuropsychological evaluation. In the case presented by Taube et al [4] , it was the persisting behavioural disturbance in the context of a near complete recovery of cognitive function that indicated a relapse in a patient with limbic encephalitis, more than a decade after the initial onset of the disease. Similarly the cases presented by Drs Kalscheur [5] and Pulsipher [6] and their colleagues both illustrate the importance of behavioural changes in the diagnosis and monitoring of patients with slow wave sleep disturbance.…”

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confidence: 94%

Editorial

Baxendale¹

2023

Epilepsy & Behavior Reports

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

Cited by 3 publications

References 21 publications

Anti-amphiphysin encephalitis: Expanding the clinical spectrum

Anti-amphiphysin encephalitis: Expanding the clinical spectrum

Positron emission tomography in autoimmune encephalitis: Clinical implications and future directions

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