Defining the Initial Clinical Symptoms and Characterizing Those Leading to the Discovery of CASPR2
Overview
- Phase
- Not Applicable
- Intervention
- Not specified
- Conditions
- CASPR2-Antibody
- Sponsor
- Hospices Civils de Lyon
- Enrollment
- 48
- Locations
- 1
- Primary Endpoint
- Initial cognitive impairment or clinical presentation of first epileptic seizures.
- Status
- Completed
- Last Updated
- 4 years ago
Overview
Brief Summary
Auto-immune encephalitides involve auto-antibodies targeting the central nervous system, and particularly the synapse and its structure, such as protein CASPR2.
CASPR2 antibody-associated auto-immune encephalitides lead to an inflammation of the limbic system and generate focal temporal seizures and cognitive impairment. Most patients are initially hospitalized because of the temporal seizures (Joubert et al., JAMA Neurology 2016). However, many already show at that time cognitive impairment, which has failed to elicit the appropriate investigations, therefore delaying the diagnosis.
The study will hence investigate precisely the initial, sometimes neglected, clinical symptoms and those leading to the diagnosis, in the cohort of patients suffering from a CASPR2 antibody-associated encephalitis, from the French reference center on paraneoplastic neurological diseases and autoimmune encephalitis.
This way, the study aims to delineate the symptoms that should trigger suspicions of a CASPR2 antibody-associated encephalitis
Investigators
Eligibility Criteria
Inclusion Criteria
- •Positive CASPR2-Ab in serum and/or CSF tested by immunohistochemistry on mouse brain slices and confirmed by cell-based assay (CBA) of HEK293 cells expressing CASPR
- •Diagnosis and follow-up in France
- •From 18 to unlimited age
Exclusion Criteria
- •Patients CASPR2-IgG negative in serum and CSF
- •Foreign follow-up
Outcomes
Primary Outcomes
Initial cognitive impairment or clinical presentation of first epileptic seizures.
Time Frame: 8 months
The retained information will depend on the very first declared symptom: if the patient first showed focal seizures, we will precise the clinical presentation of this seizure. On the other hand, if the patient first showed cognitive symptoms, we will best define this impairment.