Brain MRI Longitudinal Volumetric Characteristics Associated With Outcomes of CASPR2-Limbic Encephalitis

NCT ID: NCT06245447

Last Updated: 2024-02-07

Basic Information

• Recruitment Status: RECRUITING
• Total Enrollment: 27 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2023-10-10
• Study Completion Date: 2024-10-31

Brief Summary

Anti-CASPR2 limbic encephalitis (CASPR2-LE) is a rare neurological disorder primarily affecting males over the age of 50. It is mediated by an autoimmune antibody response in the central nervous system (CNS) against the cellular adhesion molecule contactin-associated protein-like 2 (CASPR2). This protein plays an important role in the trafficking of KV1 channels under the myelin sheath in the juxtaparanodal region of myelinated axons. It is mostly present in the neurons of the limbic system, basal ganglia, and other motor related and sensation areas (Qin, Yang, Zhu, Wang, & Shan, 2021). This distribution explains the diverse clinical manifestations of the disease, primarily characterized by cognitive impairment. Other manifestations include cerebellar ataxia, hyperkinetic movement disorders (HMDs), seizures, and neuropathic pain, which all typically develop around 10.4 months after onset. At last visit, memory impairment is seen in 69% of the patients, cerebellar ataxia in 42% of the patients, and functional dependency in 25% of the patients. Even though most patients' symptoms improve with immune-active treatments, up to 69% of them have long-term memory impairments due to damage to hippocampal structures (Benoit et al., 2023). Research has primarily focused on understanding the disease's clinical features, underlying mechanisms, and potential treatment options. On the other hand, it is shown that MRIs performed at baseline show signal changes in the hippocampus in 62-71% of the patients, and these changes are subject to variations in subsequent follow-up scans, that differ widely among patients as mentioned before (Bien et al., 2017). And since the dynamics of hippocampal volume changes and its association with the development of hippocampal atrophy and long-term cognitive impairment are not well studied yet in CASPR2-LE, we primarily aim to examine the longitudinal changes of hippocampal volume in anti-CASPR2 Limbic Encephalitis (CASPR2-LE) patients to examine whether it correlates to the development of anterograde amnesia and hippocampal atrophy on follow-up.

Conditions

Autoimmune Encephalitis Associated With Anti-CAPR2 Antibodies

Study Design

• Observational Model Type: COHORT
• Study Time Perspective: PROSPECTIVE

Study Groups

CASPR2- Patients

Retrospective evaluation of specific brain MRI features

Intervention Type: OTHER

• Brain MRI changes in the acute stage, i.e. < 3 months from symptoms onset, including abnormal signal intensity, altered diffusion and/or contrast enhancement in the hippocampus (unilateral/bilateral), abnormal signal intensity in basal ganglia, insula, and/or perisylvian cortex, presence of global brain atrophy, presence of hippocampal atrophy and/or MTS, hippocampal volumetry
• Brain MRI changes after acute phase resolution (> 6 months after symptoms onset) including abnormal signal intensity in the hippocampus (unilateral/bilateral), presence of global brain atrophy, presence of hippocampal atrophy and/or MTS, hippocampal volumetry

Patients affected by autoimmune encephalitis with antibodies against CASPR2

Retrospective evaluation of specific brain MRI features

Intervention Type: OTHER

• Brain MRI changes in the acute stage, i.e. < 3 months from symptoms onset, including abnormal signal intensity, altered diffusion and/or contrast enhancement in the hippocampus (unilateral/bilateral), abnormal signal intensity in basal ganglia, insula, and/or perisylvian cortex, presence of global brain atrophy, presence of hippocampal atrophy and/or MTS, hippocampal volumetry
• Brain MRI changes after acute phase resolution (> 6 months after symptoms onset) including abnormal signal intensity in the hippocampus (unilateral/bilateral), presence of global brain atrophy, presence of hippocampal atrophy and/or MTS, hippocampal volumetry

Interventions

Retrospective evaluation of specific brain MRI features

• Brain MRI changes in the acute stage, i.e. < 3 months from symptoms onset, including abnormal signal intensity, altered diffusion and/or contrast enhancement in the hippocampus (unilateral/bilateral), abnormal signal intensity in basal ganglia, insula, and/or perisylvian cortex, presence of global brain atrophy, presence of hippocampal atrophy and/or MTS, hippocampal volumetry
• Brain MRI changes after acute phase resolution (> 6 months after symptoms onset) including abnormal signal intensity in the hippocampus (unilateral/bilateral), presence of global brain atrophy, presence of hippocampal atrophy and/or MTS, hippocampal volumetry

Intervention Type: OTHER

Eligibility Criteria

Inclusion Criteria

• Patients with a definite diagnosis of CASPR2-antibody-associated encephalitis
• available MRI records
• Ascertained positivity for CASPR2-antibodies in CSF

Exclusion Criteria

• Positivity for another antibody against neural or glial antigens
• MRI images not available
• Not enough clinical data to ascertain outcome

• Minimum Eligible Age: 18 Years
• Maximum Eligible Age: 100 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Hospices Civils de Lyon

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Locations

Hospices Civils de Lyon

Bron, , France

Site Status: RECRUITING

Countries

France

Central Contacts

Bastien Joubert, professor

Role: CONTACT

Bastien.joubert@chu-lyon.fr

+334 78 86 15 53

Marine Villard, doctor

Role: CONTACT

marine.villard@chu-lyon.fr

+33 4 27 85 54 60

Facility Contacts

Bastien Joubert, professor

Role: primary

Bastien.joubert@chu-lyon.fr

+334 78 86 15 53

Other Identifiers

69HCL24_0106

Identifier Type: -

Identifier Source: org_study_id