Antibody-Positive Autoimmune Encephalitis and Paraneoplastic Neurological Syndrome: Epidemiology and Outcome of Neuronal Antibody Testing in Sweden

Kosek, Sonja; Persson, Bo‐Eric; Rodrigues, Rui Da Silva; Malmeström, Clas; Punga, Anna Rostedt; Burman, Joachim

doi:10.1155/2023/6993615

Cited by 2 publications

(2 citation statements)

References 25 publications

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“…Considering that the population of North Karelia is nearly entirely Caucasian, this difference in ethnic backgrounds might explain the difference in incidence. Indeed, recent data from Sweden suggest an annual incidence of 0.3/100,000 person-years for autoimmune encephalitis and paraneoplastic neurological syndrome with a tendency to increase during the study period (2015-2019) [18]. Recent data from Denmark show an incidence of 0.17/100,000 for NMDAR encephalitis alone [19], suggesting that 0.3 certainly is too low and 0.6/100,000 is more likely to reflect true occurrence rate.…”

Section: Discussionmentioning

confidence: 99%

Adult-Onset Encephalitis over Twelve Years in Easternmost Finland

Sipilä

2024

Neuroepidemiology

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Introduction: The epidemiology of encephalitis varies by region and time. Available Finnish data are outdated and there are no data from eastern parts of the country nor concerning the occurrence autoimmune encephalitides. Material and methods: Patients with encephalitis were identified from mandatory administrative registries in North Karelia Central Hospital. The diagnoses were verified, and data extracted by reviewing the patient records. Study period was 2010-2021. Only patients >16 years of age were included. Results: 51 patients with a clinical encephalitis were identified (55 % men) identified with a median age of 65 years [interquartile range (IQR) 45, 73; total age range 16-88 years] indicating a crude incidence of 3.1/100,000 person-years for the entire study period. A specific aetiology could be identified in 31 cases (61 %) with Tick-borne encephalitis (TBE) being the most common one (20 % of all 51 cases), followed by Herpes simplex virus type 1 (HSV-1, 16 %) and Varicella Zoster virus (VZV, 14 %). Autoimmune aetiology was confirmed in 10 %. TBE was most often found in the youngest age group (16-52 years of age) and the herpes viruses in the oldest group (71 years or older). A specific cause was most often identified in the oldest patients (78 %). TBE patients were younger than patients with VZV (p=0.0009) or HSV-1 (p=0.0057) but there was no difference when they were compared to patients with autoimmune (p=0.27) or unknown (p=0.074) aetiology. At presentation, there were differences in the occurrence of some clinical signs and symptoms between aetiologies but nothing specific. Eight patients (16 %) were immunosuppressed. Inpatient seizures occurred in 10 patients (20 %). In these cases, the etiology was HSV-1 in 50 % and TBE or VZV in none. A full recovery was observed in 51 % of all patients while three patients (6 %) had died of the encephalitis while in hospital or shortly after discharge. Conclusions: Adult-onset encephalitis was more common and the patients older in easternmost Finland than previously reported in other parts of the country. TBE, HSV-1 and VZV are the most commonly identified specific aetiologies whereas a fifth of the cases are probably caused by autoimmunity. Prognosis depended on aetiology but was very good in the majority of cases

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Section: Discussionmentioning

confidence: 99%

Adult-Onset Encephalitis over Twelve Years in Easternmost Finland

Sipilä

2024

Neuroepidemiology

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“…The study population consisted of 37 patients recruited from our previous epidemiological study of AE and PNS (Kosek et al, 2023), in which all patients in Healthcare region Mid Sweden who tested positive for a neuronal antibody between 2015 and 2019 were included.…”

Section: Study Populationmentioning

confidence: 99%

Antibody‐positive autoimmune encephalitis and paraneoplastic neurological syndrome: A Swedish case series

Kosek,

Burman,

Punga

2024

Brain and Behavior

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ObjectiveThis study aimed to explore the clinical characteristics and temporal disease course of patients with autoimmune encephalitis (AE) and paraneoplastic neurological syndrome (PNS) in Sweden.MethodsThirty‐seven antibody‐positive AE and PNS cases were identified in the Healthcare region Mid Sweden between 2015 and 2019. Clinical data were collected through a retrospective review of electronic health records. Patients were divided into three subgroups based on antibody type: neuronal surface antibodies (NSAbs), onconeural antibodies, and anti‐GAD65 antibodies.ResultsNineteen patients had NSAbs, 11 onconeural antibodies, and seven anti‐GAD65 antibodies. Anti‐LGI1 and anti‐NMDAR were the most frequently detected NSAbs, with anti‐NMDAR cases having an older‐than‐expected age distribution (median age 40, range 17–72). Only 11 of 32 (30%) of patients had findings suggesting encephalitis on initial MRI, but 28 of 31 (90%) had pathological findings on initial cerebrospinal fluid analysis. All patients but one had abnormal EEG findings. Median time to immunotherapy was comparable among the three subgroups, whereas patients with anti‐LGI1, anti‐CASPR2, and anti‐IgLON5 had an eightfold longer time to immunotherapy than anti‐NMDAR and anti‐GABA‐B (p = .0016). There was a seasonal variation in onset for patients with non‐tumor‐related NSAbs and anti‐GAD65 antibodies, with most patients (72%) falling ill in spring or summer.ConclusionSwedish patients with AE and PNS had similar clinical characteristics as previously described cohorts from other geographical regions except for anti‐NMDAR encephalitis, with older onset than expected. The onset of non‐tumor‐related AE occurred predominantly in the warm seasons, and AE with a more insidious onset was associated with delayed treatment initiation.

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Antibody-Positive Autoimmune Encephalitis and Paraneoplastic Neurological Syndrome: Epidemiology and Outcome of Neuronal Antibody Testing in Sweden

Cited by 2 publications

References 25 publications

Adult-Onset Encephalitis over Twelve Years in Easternmost Finland

Adult-Onset Encephalitis over Twelve Years in Easternmost Finland

Antibody‐positive autoimmune encephalitis and paraneoplastic neurological syndrome: A Swedish case series

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