Seizures in autoimmune encephalitis: Findings from an EEG pooled analysis

Morano, Alessandra; Fanella, Martina; Irelli, Emanuele Cerulli; Barone, Francesca A; Fisco, Giacomo; Orlando, Biagio; Albini, Mariarita; Fattouch, Jinane; Manfredi, M.; Casciato, Sara; Gennaro, Giancarlo Di; Giallonardo, Anna Teresa; Bonaventura, Carlo Di

doi:10.1016/j.seizure.2020.10.019

Cited by 20 publications

(24 citation statements)

References 39 publications

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“…Two types of seizures were reported in our study: generalized tonic–clonic seizure (2/5) and focal seizure with or without impaired awareness (3/5). No patients presented more than one type of seizure in the acute stage, which is in contrast to Morano’s report ( 28 ). Subclinical seizures occurred at a relatively higher rate, which motivated us to consider that widespread use of EEGs might be beneficial to the diagnosis of AE and epilepsy.…”

Section: Discussioncontrasting

confidence: 95%

Clinical Character of CASPR2 Autoimmune Encephalitis: A Multiple Center Retrospective Study

et al. 2021

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ObjectiveTo examine the clinical characteristics of autoimmune encephalitis associated with the contactin-associated protein-2 (CASPR2) antibody.Materials and MethodsMedical records of all patients diagnosed with CASPR2 antibody-associated encephalitis were retrospectively analysed. Data regarding demographic features, neurological symptoms and signs, laboratory tests, imaging results, treatments, and prognosis were collected.ResultsA total of 25 patients aged from 3 to 79 years old were enrolled in this study, with a median age of 43. Eight of 25 (32%) were female, and 17 of 25 (68%) were male. The median age of symptom onset was 42 years old with the course of disease from onset to hospital admission ranging from 2 days to 6 months (median was 17 days). Six patients (6/25) had fever as an onset symptom. During the course of disease, cognitive disturbance was the most common symptom, which was observed in 17 patients (17/25) in total. Eight patients (8/25) met the criteria for limbic encephalitis. Epileptic seizure occurred in six of these eight patients. Four patients (4/25) were diagnosed as Morvan syndrome. All patients were positive for anti-CASPR2 antibody in the serum (1:10–1:300). In six patients, antibodies were detected both in the blood and CSF (1:32–1:100). White blood cell (WBC) counts in the CSF were elevated in eight patients (8/25). The concentration of proteins in CSF increased in 10 patients (ranging from 480 to 1,337.6 mg/dl), decreased in seven patients (ranging from 23.2 to 130.5 mg/dl) and remained at a normal range in the other eight patients (ranging from 150 to 450 mg/dl). Abnormal electroencephalogram (EEG) activities included slow background activity and epileptic patterns. Abnormal signals in the bilateral hippocampus were detected by magnetic resonance imaging (MRI) in three patients presenting cognitive disturbance. In one patient who had limbic encephalitis, increased metabolism of bilateral basal ganglia and the mesial temporal lobe was revealed by PET-CT. Eleven of 15 patients receiving immunotherapy experienced varying degrees of improvement. Relapse occurred in four of 25 patients (4/25) after 2 months.ConclusionCASPR-antibody-mediated autoimmune encephalitis is characterized by diverse clinical manifestations. The most prominent conclusion revealed by this retrospective analysis is the involvement of both central and peripheral nerve systems, as well as a lower relapse rate, a good response to immunotherapy, and favorable short-term prognosis after treatment was also demonstrated. Besides, additional work is necessary to evaluate the long-term prognosis.

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Section: Discussioncontrasting

confidence: 95%

Clinical Character of CASPR2 Autoimmune Encephalitis: A Multiple Center Retrospective Study

et al. 2021

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“…Finally, smell impairment was detected more frequently in cases where mesial temporal involvement was presumably bilateral, based on ictal/interictal EEG findings (which could reveal a wider brain participation than detected through structural neuroimaging studies alone) [ 17 ]. This observation leads to further reflection upon the controversial topic of olfactory function lateralization.…”

Section: Discussionmentioning

confidence: 99%

Olfactory impairment in autoimmune encephalitis: another piece of the puzzle

et al. 2022

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Background Despite being long neglected, olfaction has recently become a focus of intense research in neuroscience, as smell impairment has been consistently documented in both neurodegenerative and neuroinflammatory diseases. Considering the close anatomo-functional correlations between the limbic system and the central olfactory structures, we investigated olfaction in a population of patients with autoimmune encephalitis (AE). Methods Nineteen adult subjects (14 males, median age 64 years) diagnosed with definite (14/19) or possible (5/19) AE and followed for ≥ 6 months were enrolled. The Brief Smell Identification Test (B-SIT), a 12-item, forced-choice, scratch-and-sniff measure, was used to assess the patients’ olfactory function in comparison with a group of sex- and age-matched healthy controls (HC). According to the B-SIT score, subjects were classified as anosmic (< 6), hyposmic (6–8) and normal (≥ 9). Electro-clinical, laboratory and neuroimaging findings were reviewed. Results Smell impairment was revealed in 15/19 patients (9 hyposmic, 6 anosmic), compared with 5/19 HC ( p = 0.0029). Age, gender and smoking habits did not affect the participants’ performance at B-SIT. Olfactory dysfunction appeared more common among patients with definite AE ( p = 0.0374), regardless of autoantibody status. Subjects with higher modified Rankin Scale (mRS) scores at AE onset more likely presented hyposmia/anosmia ( p = 0.033), and so did those with bilateral ictal/interictal EEG abnormalities ( p = 0.006). Conclusions We found olfaction to be impaired in a significantly large proportion of AE cases. Smell deficits appeared more common in subjects with severe AE (as indicated by both definite diagnosis and higher mRS score), and might represent an additional feature of immune-mediated encephalitis. Supplementary Information The online version contains supplementary material available at 10.1007/s00415-022-10959-6.

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“…Epileptiform discharges appeared in 21.6% of the patients [ 5 ]. Reports of some other studies have described that when epileptic discharges appear in EEG, they are most frequently distributed over the temporal region [ 7 , 8 ]. Furthermore, it is noteworthy that in AE, changes in seizure semiology and EEG findings over time have often been seen [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

“…Although we measured conventional EEG repeatedly, we were unable to record the ictal EEG because of the lack of continuous EEG monitoring. For AE, subclinical seizures (SCs) are reportedly observed occasionally on continuous EEG monitoring in addition to clinical seizures (CSs) [ 7 , 8 ]. Furthermore, comparison of the relative proportions of CSs and SCs between antibody-positive and negative AE showed that the proportion of SCs was significantly higher in the antibody-negative cases [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

EEG Contribution to the Diagnosis of Antibody-Negative Autoimmune Encephalitis: A Case Report

et al. 2021

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Autoimmune encephalitis (AE) is a group of inflammatory brain diseases that are characterized by prominent neuropsychiatric symptoms. Early therapeutic intervention is important for AE. Therefore, without waiting for autoantibody test results, clinicians must consider the possibility of AE based solely on clinical symptoms and conventional test results. The case described herein is of antibody-negative encephalitis with abnormalities shown only by EEG, which contributed to the diagnosis and treatment. The patient, a 20-year-old woman, showed autonomic seizures in addition to movement disorders, psychiatric symptoms, and cognitive dysfunction, which worsened subacutely. Her seizures and movement disorders were not responsive to antiepileptic medications. Results obtained from MRI and cerebrospinal fluid (CSF) were normal; EEG findings showed repeated spikes in the right temporal area, with changes over time. Based on the clinical course and EEG, along with administered immunotherapy, which resolved seizures, movement disorders, and psychiatric symptoms, we suspected AE. For diagnosis of AE and for evaluating treatment responsiveness, EEG was useful. Results indicate that EEG can assist clinicians even with AE cases for which MRI and CSF findings are normal.

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Seizures in autoimmune encephalitis: Findings from an EEG pooled analysis

Cited by 20 publications

References 39 publications

Clinical Character of CASPR2 Autoimmune Encephalitis: A Multiple Center Retrospective Study

Clinical Character of CASPR2 Autoimmune Encephalitis: A Multiple Center Retrospective Study

Olfactory impairment in autoimmune encephalitis: another piece of the puzzle

EEG Contribution to the Diagnosis of Antibody-Negative Autoimmune Encephalitis: A Case Report

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