Dr. Amaia Muñoz Lopetegi (Hospital Clinic of Barcelona – Spain) has been awarded the AEA Community Seed Grant to investigate the Subclinical activity in anti-LGI1 encephalitis: diagnostic tests and prognostic implications. Learn more about her study and how this will affect those affected with autoimmune encephalitis.
You have been awarded the AEA Seed Grant, can you tell us more about the study you plan to do?
The study is aimed at characterizing the post-acute stage of the encephalitis associated with LGI1 antibodies (anti-LGI1), which is the second most frequently reported AE. It affects patients usually older than 60 years, and produces a limbic encephalitis, with different types of epileptic seizures and prominent cognitive decline. The importance of recognizing anti-LGI1 encephalitis is that its prompt diagnosis and treatment usually associate with neurologic improvement and likely prevents permanent symptoms.
Even though the most acute stage of the disease is well characterized, the later stage of the disease is much less known, and it is even unclear whether these patients may have subclinical active disease with manifestations that may have an impact on cognitive recovery.
In order to better understand the disease, we will follow patients during a year with serial visits that include an extensive panel of neurologic, cognitive, and psychiatric investigations, along with comprehensive clinical and video-polysomnographic sleep assessment, electroencephalography and neuroimaging.
Preliminary results in these patients are revealing the presence of unsuspected alterations during routine outpatient clinic assessment, and also show that symptoms considered resolved, such as epileptic seizures, are in fact still present during sleep evaluations, which may negatively impact on cognitive function and memory. These symptoms and tests (e.g.V-PSG, prolonged EEG) can be used as prognostic biomarkers guiding treatment in the post-acute phase of the disease.
How will your study help patients and families affected by AE?
This project will address one of the most complicated questions in AE, that is how to assess and treat patients after they are discharged from the hospital or rehabilitation, but have not reached complete recovery (they are unable to return to work, or they have difficulties in their daily activities). Our preliminary data indicate that a substantial number of patients with anti-LGI1 encephalitis still have an active disease, rather than a slow recovery process or permanent residual deficits. This would indicate that the immune response is still causing damage, producing symptoms that may be subtle or difficult to detect, but can be demonstrated by studies not used in routine clinical practice.
This study will provide clinical and paraclinical biomarkers (e.g., sleep studies, prolonged EEG assessment) for disease follow-up, that will permit clinicians to identify patients that need adjustment of symptomatic medications or more immunotherapy, thus shortening the process of recovery and improving overall prognosis.
Tell us more about yourself and your affiliation?
I am a neurologist and PhD research student. I received my MD degree from the University of the Basque Country (Spain). During my residency program in Neurology at the Donostia University Hospital (Spain), I did a 6-month stay at the Erasmus University Medical Center in Rotterdam, The Netherlands under the mentorship of Dr. Maarten J Titulaer, who is known for his work in the field of immune mediated encephalopathies. Upon completion of my stay in Rotterdam I was accepted to the PhD program at the University of Barcelona and was also named junior faculty at its affiliated hospital, Hospital Clinic of Barcelona. At the Hospital Clinic I work in the Sleep Unit where I attend the Sleep Disorders Unit outpatient clinic and I read polysomnography and EEG recordings for both clinical and research studies. My PhD research is being carried out in the Neuroimmunology group, directed by Dr. Josep Dalmau. My studies are focused on sleep disorders in autoimmune encephalopathies. My goal is to become an independent, translational researcher.
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