Epidemiology of Autoimmune Encephalitides and Paraneoplastic Neurological Syndromes in Sweden

NCT04708626 | Completed

• 1 other identifier: DNR2019-03068
• Study Type: observational
• Target: 110
• Locations: 1 country
• Sites: 1

Brief Summary

Autoimmune encephalitis and paraneoplastic neurological syndromes are rare diseases caused by an abnormal immune response toward the nervous system. This can lead to life-threatening symptoms, but is in many cases treatable if a swift and correct diagnosis is made. Antibodies targeting neuronal proteins (i.e. "neuronal antibodies") can be detected in serum or cerebrospinal fluid (CSF) in about half of the patients suffering from these conditions. Although an important part of the diagnostical process of these conditions, diagnosis cannot be made only based on a positive antibody test, but the clinical findings have to be compatible as well. As these conditions are so rare, clinicians might struggle to interpret antibody test results. In this study the investigators aim to estimate the incidence rate of autoimmune encephalitides and paraneoplastic neurological syndromes in the Uppsala-Örebro health care region in Sweden between the years 2015 and 2019. Medical records from patients belonging to the Uppsala-Örebro health care region (a region in the middle of Sweden with a population of approximately 2.1 million), that tested positive for any neuronal antibody in serum or CSF will be studied to obtain clinical, laboratory and radiological data. This data will be used to ascertain if diagnostic criteria are fulfilled as well as to describe clinical characteristics and identifying possible comorbidities.

Conditions

Autoimmune Encephalitis; Paraneoplastic Neurological Syndrome

Keywords

autoimmune encephalitis; paraneoplastic neurological syndrome; neuronal antibody; epidemiology; incidence rate

Outcome Measures

Primary Outcomes (1)

• Incidence rate of autoimmune encephalitides and paraneoplastic neurological syndromes in the Uppsala-Örebro health care region between 2015-2019

  Incidence rate of autoimmune encephalitides and paraneoplastic neurological syndromes in the Uppsala-Örebro health care region based on detection of neuronal antibodies in serum or CSF, with case ascertainment based on review of medical records and application of diagnostic criteria (Graus et al. 2016 and 2021).

  5 years

Secondary Outcomes (4)

• Positivity rate

  5 years

• False positivity rate

  5 years

• Estimated incidence rate of autoimmune encephalitides and paraneoplastic neurological syndromes in the Swedish population

  5 years

• Incidence rates of autoimmune encephalitides and paraneoplastic neurological syndromes

  5 years

Study Arms (2)

Extended Cohort: Patients tested for any neuronal antibody in the Swedish population

All patients tested for any neuronal antibody in serum or CSF between 2015 and 2019 in Sweden.

Other: Description and analysis

Core Cohort: Patients with a positive neuronal antibody test belonging to the Uppsala-Örebro region

All patients belonging to the Uppsala-Örebro health care region (a region in the middle of Sweden with a population of approximately 2.1 million), that tested positive for any neuronal antibody in serum or cerebrospinal fluid between 2015-2019.

Other: Description and analysis

Interventions

Description and analysis OTHER

Collection of clinical, laboratory and radiological data from medical records.

Core Cohort: Patients with a positive neuronal antibody test belonging to the Uppsala-Örebro region

Eligibility Criteria

• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Extended cohort: All patients in Sweden tested for any neuronal antibody (AMPA 1, AMPA 2, Amphiphysin, CARP VIII, CASPR2, CV2/CRMP5, DPPX, GABA B, GAD65 (\>2000 IU/ml by ELISA in serum, or detected in CSF), glycine receptor, Homer 3, Hu, IgLON5, ITPR1, LGI-1, Ma2/Ta, NMDAR, PCA-2, Tr, Ri, SOX1, VGCC, Yo, Zic4), in serum or CSF between 2015 and 2019. Core cohort: All patients belonging to the Uppsala-Örebro health care region (a region in the middle of Sweden with a population of approximately 2.1 million), that tested positive for any neuronal antibody in serum or cerebrospinal fluid between 2015 and 2019. Patients will be identified by the only five laboratories that perform tests for neuronal antibodies in Sweden.

You may qualify if:

• All ages, both sexes.
• Neuronal antibody (AMPA 1, AMPA 2, Amphiphysin, CARP VIII, CASPR2, CV2/CRMP5, DPPX, GABA B, GAD65(\>2000 IU/ml by ELISA in serum, or detected in CSF), glycine receptor, Homer 3, Hu, IgLON5, ITPR1, LGI-1, Ma2/Ta, NMDAR, PCA-2, Tr, Ri, SOX1, VGCC, Yo, Zic4), detected in serum or cerebrospinal fluid between 2015-2019
• Antibody test was requested by a health care facility in the Uppsala-Örebro health care region
• Signed informed consent. If the participant is deceased consent will be presumed

You may not qualify if:

• Incomplete personal data or social security number making it impossible to identify and/or contact the patient to get written consent
• Informed consent not signed. If the participant is deceased consent will be presumed

Sponsors & Collaborators

• Uppsala University: lead

Study Sites (1)

Uppsala University

Uppsala, Uppland, 75237, Sweden

Location

MeSH Terms

Conditions

Autoimmune Diseases of the Nervous System

Condition Hierarchy (Ancestors)

Nervous System Diseases; Autoimmune Diseases; Immune System Diseases

Study Officials

• Joachim Burman, Assoc prof

  Uppsala University

  STUDY DIRECTOR

• Anna Rostedt Punga, Professor

  Uppsala University

  STUDY DIRECTOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: January 11, 2021
• First Posted: January 14, 2021
• Study Start: October 1, 2019
• Primary Completion: August 1, 2022
• Study Completion: August 1, 2022
• Last Updated: November 9, 2022

Record last verified: 2022-11

Locations

SE (1)