Elisa Visani , Laura Canafoglia , Lorenzo Nanetti , Davide Rossi Sebastiano , Dunja Duran , Paola Anversa , Deborah Bonfoco , Sara Dotta , Davide Tabarelli , Anna Castaldo , Gloria Marchini , Alessia Mongelli , Caterina Mariotti
{"title":"弗里德赖希共济失调对多模态刺激的神经磁反应","authors":"Elisa Visani , Laura Canafoglia , Lorenzo Nanetti , Davide Rossi Sebastiano , Dunja Duran , Paola Anversa , Deborah Bonfoco , Sara Dotta , Davide Tabarelli , Anna Castaldo , Gloria Marchini , Alessia Mongelli , Caterina Mariotti","doi":"10.1016/j.clinph.2025.2110738","DOIUrl":null,"url":null,"abstract":"<div><h3>Objective</h3><div>This study explores the utility of various evoked fields in elucidating the pathophysiology of Friedreich’s ataxia (FA) and potentially contributing to developing more targeted diagnostic and therapeutic strategies.</div></div><div><h3>Methods</h3><div>Thirty-seven patients with FA aged 27.6 ± 7.4 years and a control group of 17 healthy subjects were enrolled in the study. The neuromagnetic response to auditory, tactile, visual, somatosensory, auditory and tactile oddball stimulation were acquired. For all the components of interest, latency and amplitude were measured and correlated with clinical data.</div></div><div><h3>Results</h3><div>Neuromagnetic responses were identifiable in more than 90% of cases. A significant response delay was observed in all tested modalities (auditory, somatosensory, tactile and visual responses). P300 responses were comparable in patients and healthy subjects. Latencies of visual and auditory responses correlated with SARA scores. Moreover, latencies of auditory responses correlated with disease onset age, whereas latencies of visual responses correlated with disease severity.</div></div><div><h3>Conclusions</h3><div>Auditory and visual responses correlated with the severity of the disease, whereas alterations in somatosensory responses represent an intrinsic characteristic of the disease.</div></div><div><h3>Significance</h3><div>In FA the study of evoked visual fields could provide a possible biomarker of disease progression and treatment efficacy.</div></div>","PeriodicalId":10671,"journal":{"name":"Clinical Neurophysiology","volume":"175 ","pages":"Article 2110738"},"PeriodicalIF":3.7000,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Neuromagnetic responses to multimodal stimuli in Friedreich’s ataxia\",\"authors\":\"Elisa Visani , Laura Canafoglia , Lorenzo Nanetti , Davide Rossi Sebastiano , Dunja Duran , Paola Anversa , Deborah Bonfoco , Sara Dotta , Davide Tabarelli , Anna Castaldo , Gloria Marchini , Alessia Mongelli , Caterina Mariotti\",\"doi\":\"10.1016/j.clinph.2025.2110738\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Objective</h3><div>This study explores the utility of various evoked fields in elucidating the pathophysiology of Friedreich’s ataxia (FA) and potentially contributing to developing more targeted diagnostic and therapeutic strategies.</div></div><div><h3>Methods</h3><div>Thirty-seven patients with FA aged 27.6 ± 7.4 years and a control group of 17 healthy subjects were enrolled in the study. The neuromagnetic response to auditory, tactile, visual, somatosensory, auditory and tactile oddball stimulation were acquired. For all the components of interest, latency and amplitude were measured and correlated with clinical data.</div></div><div><h3>Results</h3><div>Neuromagnetic responses were identifiable in more than 90% of cases. A significant response delay was observed in all tested modalities (auditory, somatosensory, tactile and visual responses). P300 responses were comparable in patients and healthy subjects. Latencies of visual and auditory responses correlated with SARA scores. Moreover, latencies of auditory responses correlated with disease onset age, whereas latencies of visual responses correlated with disease severity.</div></div><div><h3>Conclusions</h3><div>Auditory and visual responses correlated with the severity of the disease, whereas alterations in somatosensory responses represent an intrinsic characteristic of the disease.</div></div><div><h3>Significance</h3><div>In FA the study of evoked visual fields could provide a possible biomarker of disease progression and treatment efficacy.</div></div>\",\"PeriodicalId\":10671,\"journal\":{\"name\":\"Clinical Neurophysiology\",\"volume\":\"175 \",\"pages\":\"Article 2110738\"},\"PeriodicalIF\":3.7000,\"publicationDate\":\"2025-05-13\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Clinical Neurophysiology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S1388245725005905\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Clinical Neurophysiology","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S1388245725005905","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
Neuromagnetic responses to multimodal stimuli in Friedreich’s ataxia
Objective
This study explores the utility of various evoked fields in elucidating the pathophysiology of Friedreich’s ataxia (FA) and potentially contributing to developing more targeted diagnostic and therapeutic strategies.
Methods
Thirty-seven patients with FA aged 27.6 ± 7.4 years and a control group of 17 healthy subjects were enrolled in the study. The neuromagnetic response to auditory, tactile, visual, somatosensory, auditory and tactile oddball stimulation were acquired. For all the components of interest, latency and amplitude were measured and correlated with clinical data.
Results
Neuromagnetic responses were identifiable in more than 90% of cases. A significant response delay was observed in all tested modalities (auditory, somatosensory, tactile and visual responses). P300 responses were comparable in patients and healthy subjects. Latencies of visual and auditory responses correlated with SARA scores. Moreover, latencies of auditory responses correlated with disease onset age, whereas latencies of visual responses correlated with disease severity.
Conclusions
Auditory and visual responses correlated with the severity of the disease, whereas alterations in somatosensory responses represent an intrinsic characteristic of the disease.
Significance
In FA the study of evoked visual fields could provide a possible biomarker of disease progression and treatment efficacy.
期刊介绍:
As of January 1999, The journal Electroencephalography and Clinical Neurophysiology, and its two sections Electromyography and Motor Control and Evoked Potentials have amalgamated to become this journal - Clinical Neurophysiology.
Clinical Neurophysiology is the official journal of the International Federation of Clinical Neurophysiology, the Brazilian Society of Clinical Neurophysiology, the Czech Society of Clinical Neurophysiology, the Italian Clinical Neurophysiology Society and the International Society of Intraoperative Neurophysiology.The journal is dedicated to fostering research and disseminating information on all aspects of both normal and abnormal functioning of the nervous system. The key aim of the publication is to disseminate scholarly reports on the pathophysiology underlying diseases of the central and peripheral nervous system of human patients. Clinical trials that use neurophysiological measures to document change are encouraged, as are manuscripts reporting data on integrated neuroimaging of central nervous function including, but not limited to, functional MRI, MEG, EEG, PET and other neuroimaging modalities.