Ignite Creation Date:
2024-10-26 @ 3:35 PM
Last Modification Date:
2024-10-26 @ 3:35 PM
Study NCT ID:
NCT06515106
Status:
RECRUITING
Last Update Posted:
None
First Post:
2024-03-26
Brief Title:
Antibody-mediated LGI1 Encephalitis Symptoms Biomarkers and Mechanisms of the Chronic Phase of the Disease
Sponsor:
None
Organization:
None
Study Overview
Official Title:
Antibody-mediated LGI1 Encephalitis Symptoms Biomarkers and Mechanisms of the Chronic Phase of the Disease
Status:
RECRUITING
Status Verified Date:
2024-07
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
No
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
None
Brief Summary:
The encephalitis mediated by antibodies against Leucine-rich glioma inactivated 1 protein anti-LGI1 encephalitis predominantly affects men MF 64 and mostly older than 60 years The disease has two distinct clinical phases The acute phase in which the majority of patients develop severe short-term memory deficits unable to remember events or experiences that occurred a few minutes earlier This memory impairment can be preceded or accompanied by one or more of the following hyponatremia 60 of patients a highly distinctive type of seizures called facio-brachial dystonic seizures 40 of patients along with confusion irritability and other types of focal seizures or less frequently generalized seizures In addition many patients at this stage have symptoms of REM sleep behavior disorder In this stage the CSF may show pleocytosis or mild increase of proteins the EEG is usually abnormal and in 60 of the patients the MRI shows typical increased FLAIR signal in medial temporal lobes 11 There is a clinical sub-phenotype 13 of patients in which the disease presents as a rapidly progressive cognitive decline without the indicated FLAIR MRI changes About 70 of patients improve rapidly with corticosteroids and immunotherapy eg intravenous immunoglobulins andor plasma exchange but the improvement is often partial After the acute phase there is a chronic or residual phase which represents the interval from improvement of initial symptoms until the disease is considered no longer active and the remaining symptoms are thought to be irreversible This chronic phase may take several months it has been less well studied and is characterized by the absence of CSF pleocytosis and inflammatory MRI changes albeit this may show residual hippocampal atrophy and very low or undetectable titers of serum antibodies Most patients are unable to return to their job or previous activities due to residual irreversible memory or cognitive deficits accompanied by signs of moderate brain atrophy In addition we and others have shown that about 27-35 of patients have relapsing symptoms after improving from the acute phase Although acute symptomatic seizures facio-brachial dystonic and others occur in 90 of patients during the acute phase of the disease less than 10 of patients develop chronic epilepsy often associated with hippocampal sclerosis Therefore the prevailing concept on this disease suggests a syndrome and clinical course in which the acute phase shows rapid albeit partial response to immunotherapy and the symptoms of the chronic phase represent a burnout or irreversible process in which the disease is no longer active and the potential improvement of remaining symptoms is uncertain
Here investigators postulate that a better knowledge of this stage will improve treatment decisions and outcome
In Aim 1 the post-acute stage will be clinically characterized
In Aim 2 the impact of cognitive rehabilitation will be assessed
In Aim 3 a mouse model of anti-LGI1 encephalitis will be used to determine the underlying mechanisms and treatment of the postacute stage
Here investigators postulate that a better knowledge of this stage will improve treatment decisions and outcome
In Aim 1 the post-acute stage will be clinically characterized
In Aim 2 the impact of cognitive rehabilitation will be assessed
In Aim 3 a mouse model of anti-LGI1 encephalitis will be used to determine the underlying mechanisms and treatment of the postacute stage
Detailed Description:
None
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
None
Is a FDA Regulated Device?:
None
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None