Ignite Creation Date:
2024-05-06 @ 3:39 PM
Last Modification Date:
2024-10-26 @ 1:54 PM
Study NCT ID:
NCT04708626
Status:
COMPLETED
Last Update Posted:
2022-11-09
First Post:
2021-01-11
Brief Title:
Epidemiology of Autoimmune Encephalitides and Paraneoplastic Neurological Syndromes in Sweden
Sponsor:
Uppsala University
Organization:
Uppsala University
Study Overview
Official Title:
Epidemiology of Autoimmune Encephalitides and Paraneoplastic Neurological Syndromes in Sweden
Status:
COMPLETED
Status Verified Date:
2022-11
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
None
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 ie 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 21 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
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 21 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
Detailed Description:
Methods
1 Identification of extended cohort
The extended cohort consists of all patients in Sweden tested for any neuronal antibody in serum or CSF between 2015 and 2019 Patients will be identified by the only five laboratories that perform tests for neuronal antibodies in Sweden The following neuronal antibodies will be included AMPA 1 Anti-Glutamate Receptor 1 AMPA 2 Anti-Glutamate Receptor 2 Amphiphysin CARP VIII Carbonic Anhydrase-Related Protein VIII CASPR2 Contactin-associated protein-like 2 CV2CRMP5 collapsin response mediator protein 5 DPPX dipeptidyl-peptidase-like protein 6 GABA B γ-Aminobutyric acid-B receptor GAD65 glutamic acid decarboxylase 2000 IUml by ELISA in serum or detected in CSF glycine receptor Homer 3 Hu antineuronal nuclear antibody-type 1 ANNA-1 IgLON5 immunoglobulin-like cell adhesion molecule 5 ITPR1 inositol 145-trisphophate receptor type 1 LGI-1 Leucine-rich glioma-inactivated 1 Ma2Ta NMDAR anti-N-methyl-D-aspartate receptor PCA-2 Purkinje cell cytoplasmic antibody type 2 Tr Trotter Ri SOX1SRY-Box Transcription Factor 1 VGCC Voltage-gated calcium channels Yo Zic4 Zinc finger protein
2 Identification of geographical region
Patients testing positive for any neuronal antibody in serum or CSF will be stratified according to which Swedish health care region that requested the test Patients whose tests where requested by health care providers in the Uppsala-Örebro health care region consisting of 7 smaller health care regions with a total population of approximately 21 million will be selected
3 Core cohort
Patients with a positive test result that belong to the Uppsala-Örebro health care region will be contacted and asked to participate in the study Written informed consent must be signed to be included in the core cohort If the patient is deceased consent will be presumed
4 Case ascertainment
Medical records from patients included in the core cohort will be reviewed to obtain clinical laboratory and radiological data Ascertainment of a case is defined as the patient either fulfilling criteria of 1 definite PNS according to Graus et al 2021 or 2 definite autoimmune limbic encephalitis according to Graus et al 2016 or 3 definite anti-NMDA receptor encephalitis according to Graus et al 2016
1 Identification of extended cohort
The extended cohort consists of all patients in Sweden tested for any neuronal antibody in serum or CSF between 2015 and 2019 Patients will be identified by the only five laboratories that perform tests for neuronal antibodies in Sweden The following neuronal antibodies will be included AMPA 1 Anti-Glutamate Receptor 1 AMPA 2 Anti-Glutamate Receptor 2 Amphiphysin CARP VIII Carbonic Anhydrase-Related Protein VIII CASPR2 Contactin-associated protein-like 2 CV2CRMP5 collapsin response mediator protein 5 DPPX dipeptidyl-peptidase-like protein 6 GABA B γ-Aminobutyric acid-B receptor GAD65 glutamic acid decarboxylase 2000 IUml by ELISA in serum or detected in CSF glycine receptor Homer 3 Hu antineuronal nuclear antibody-type 1 ANNA-1 IgLON5 immunoglobulin-like cell adhesion molecule 5 ITPR1 inositol 145-trisphophate receptor type 1 LGI-1 Leucine-rich glioma-inactivated 1 Ma2Ta NMDAR anti-N-methyl-D-aspartate receptor PCA-2 Purkinje cell cytoplasmic antibody type 2 Tr Trotter Ri SOX1SRY-Box Transcription Factor 1 VGCC Voltage-gated calcium channels Yo Zic4 Zinc finger protein
2 Identification of geographical region
Patients testing positive for any neuronal antibody in serum or CSF will be stratified according to which Swedish health care region that requested the test Patients whose tests where requested by health care providers in the Uppsala-Örebro health care region consisting of 7 smaller health care regions with a total population of approximately 21 million will be selected
3 Core cohort
Patients with a positive test result that belong to the Uppsala-Örebro health care region will be contacted and asked to participate in the study Written informed consent must be signed to be included in the core cohort If the patient is deceased consent will be presumed
4 Case ascertainment
Medical records from patients included in the core cohort will be reviewed to obtain clinical laboratory and radiological data Ascertainment of a case is defined as the patient either fulfilling criteria of 1 definite PNS according to Graus et al 2021 or 2 definite autoimmune limbic encephalitis according to Graus et al 2016 or 3 definite anti-NMDA receptor encephalitis according to Graus et al 2016
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
False
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None