Università degli studi di Modena e Reggio Emilia

Cataplexy: a new therapeutic path is the result of a study carried out by the University Hospital of Modena and the IRCCS of Bologna

A study carried out by neurologists of the City Hospital of Baggiovara and researchers of the Department of Biomedical Science, Metabolic Science and Neuroscience (BMN), Centre for Neuroscience and Neurotechnologies (CfNN) of Unimore, in collaboration with the researchers and expert clinicians of Sleep Medicine of the Institution of Neurological Science of Bologna (IRCCS), revealed the brain circuits that are active during spontaneous laughter in children suffering from narco-cataplexy, a rare sleep pathology characterised by daytime uncontrollable sleepiness and sudden loss of muscle tone, often triggered by strong emotions. Thanks to state-of-the-art neuroimaging methodologies, the study developed between Modena and Bologna served to demonstrate the functional involvement of a brain region, the undefined area, the activity of which might help prevent cataplexy attacks caused by strong emotions. The study was published on the prestigious scientific journal Neurology, edited by the American Academy of Neurology Journals and was awarded by appearing on the cover of the issue of 29 January 2019.    

The research was carried out and realised thanks to the advanced neuroimaging experience of Doctor Anna Elisabetta Vaudano, Medical Executive at the unit of Neurology of the University Hospital of Modena, and coordinated by professor Stefano Meletti of the Department of Biomedical Science, Metabolic Science and Neuroscience, Centre for Neuroscience and Neurotechnologies (DBMN - CfNN) of UniMoRe and Professor  Giuseppe Plazzi of the University of Bologna and IRCCS - Institute for Neurological Science of Bologna.

The relationship between positive emotions and cataplexy has long been known and is already the object of several publications. For the first time, the new study has proven the involvement of specific brain nodes, whose activity affects and probably inhibits the activation of the pathological network that triggers cataplexy attacks. In the future, the study carried out by the University Hospital of Modena, UniMoRe and the IRCCS of Bologna may pave the way for new therapeutic targets for this rare still debilitating pathological condition. 

Professor Stefano Meletti explained that “Like other emotions, laughter may trigger a cataplectic attack in patients suffering from narco-cataplexy, an event featuring a sudden and temporary loss of muscle tone. These attacks may be quite debilitating for patients, who are often children and teenagers”. The study exploited non-invasive techniques of advanced neurological diagnostics imaging: Electroencephalogram (EEG) recorded with Functional Magnetic Resonance, EEG-fMRI, carried out in the laboratory of Brain Functional Investigation financed by the University Hospital of Modena and the DBMN within the project of the Departments of Excellence of the Ministry of Education, University and Research. Researchers were able to explore the brain circuits of 21 children/teen-agers suffering from narco-cataplexy, and 21 healthy children/teen-agers involved in spontaneous laughter raised after watching funny videos. Doctor Anna Elisabetta Vaudano, Neurologist at the Civil Hospital, pointed out that “the results show the involvement of brain networks that partially differ in patients compared with the checks. More specifically, the analyses let us identify the involvement of an area of the brain, the so called “undefined area”, the activity of which had never been related to narcolepsy/cataplexy except for in experimental animals. The results of the study show that an increased activation of the undefined area may help prevent the cataplexy attacks raised by a stimulus and/or positive emotion”.

“With our fellow researchers of the University of Modena, we have built a successful cooperation that allowed us to achieve ambitious goals and significantly helped broaden our knowledge on narcolepsy with cataplexy”, professor Giuseppe Plazzi eventually underlined. “My warm thanks definitely go to voluntary patients and children, to their families who have made possible the implementation of the project, up to its final results”. 

The main author of the project is Doctor Anna Elisabetta Vaudano, a researcher from Rome, where she obtained the specialisation in Neurology and the PhD in Neuroscience, with a sound background of experience in the field of functional neuroimaging applied to neurological diseases, in particular epilepsy and sleep diseases. She has spent part of her scientific career abroad, at the University College of London (UCL) under the supervision of professor Louis Lemieux. Doctor Vaudano has been working and cooperating with UniMoRe for many years. Doctor Vaudano remarked that “In line with recent data obtained from experimental animals, our results confirm for the first time in human beings the functional role of the undefined area in cataplexy, and help identify this brain region as a possible therapeutic target”

Professor Stefano Meletti added that “this data opens up new perspectives both for the study of cataplexy and how it relates to emotions, both to identify new drug targets in this pathologic condition that mainly affects the quality of life of young patients”.

The study lasted nearly two years and was made possible thanks to the cooperation between the neurologists of the Civil Hospital of Baggiovara and the DBMN - CfNN of Unimore and the fellow researchers and expert clinicians of of Sleep Medicine of the IRCCS, Institution of Neurological Science of Bologna and the Department of Biomedical and Neuromotor Science of the University of Bologna. In fact, the children and teen-agers suffering from narco-cataplexy have been entirely selected by the Centre of Sleep Medicine driven by Professor Plazzi. The centre has a focused and consolidated interest in research, diagnosis and treatment of this pathological disease.  All the acquisitions and analyses of EEG-fMRI data both in narco-cataplexic and healthy children were carried out at the Civil Hospital of Baggiovara (Modena). Doctor Anna Elisabetta Vaudano and Professor Stefano Meletti, together with the other members of the team, are a national and international point of reference as regards the use of this method employing the “fusion” of two investigation methods, EEG and Magnetic Resonance, to obtain live information on how the brain of patients affected by different neurologic pathologies work, and in particular for patients suffering from epilepsy and sleep diseases.

Categorie: International - english