Publication: Amyotrofik Lateral Skleroz hastalığında motor nöron kaybının erken dönem tanısında yardımcı olabilecek yeni elektrofizyolojik incelemeler
Abstract
GirişveAmaç:Amyotrofik lateral skleroz(ALS)'de motor ünite(MU) kaybını öngörmek için birçok klinik nörofizyolojik teknikgeliştirilmiştir. Son on yılda görece daha kapsamlı olan motor unitnumber indeks (MUNIX)ve CMAP scan bazlı MUNE metodu (MScan-fit MUNE) hızlı ve pratik bir şekilde MU sayımı amacıyla ortaya atıldı. Bu çalışmada bu teknikleri kullanarak ALS hastalığında görülenMU kaybını erken dönemde saptamayı vehangi nörodejenerasyonpaterniyle uyumlu ilerlediğinianlamayı amaçladık.Metot: Çalışmamıza 25hasta ve 25 sağlıklı kontrol alındı. Ulnar sinir inervasyonlufleksörkarpi ulnaris (FCU), abductor digiti minimi (ADM) ve first dorsal interosseöz (FDI) kaslarında BKAP, MUNIX ve CMAP scanning (MScan) testleri yapıldı. Bulgular: Ulnar sinir tarafından innerve edilen FCU, ADM ve FDI kaslarından kaydedilen elektrofizyolojik biyobelirteçlerin (BKAP amplitüdü ve alan, MScan-fit MUNE ve MUNIX) ortalama değerleri proksimalden distale kademeli olarak azalmaktaydı. Çalışılan her üç kas için de normal sınırlardaBKAP amplidütü olan hastalarda (n =13 kişi)BKAP alanı, MScan-fit MUNE ve MUNIX erken dönemdeki MU kaybını sırasıyla % 30,7, %53,8,%38,4 oranında gösterebildiler. Sonuç: Çalışmamızda BKAP alanı, MScan-fit MUNE ve MUNIX testleri BKAP amplitüdünün düşmeye başlamasından daha önceki dönemde bile bozulma göstermekteydi. Ayrıca,MUNIX ve Mscan-fit MUNE’deki MU kaybı dying-back nörodejenerasyona benzer bir patern ile uyumluydu. MUNIX ve MScan-fit MUNE benzer sonuçlar gösterirken, reinnervasyon belirteçlerinden MUSIX, MScan-fit MeanUnit ve LargetsUnit‘dan daha iyi sonuçlar vermekteydi.
Introduction: Several neurophysiological methods, i.e., motor unit number estimation (MUNE) were used to predict motor unit loss in ALS. In the last decade, relatively sophisticated methods, e.g., motor unit number index (MUNIX) and MUNE with MScan-fit software using compound muscle action potential (CMAP) scanning recordings were introduced for the same purpose. In this study, we tried to find out neurodegeneration pattern in ALS and to detect early MU loss.Methods: Twenty five patients and 25 healthy control were enrolled for this study. CMAPs, MUNIX and CMAP scanning (MScan) tests were recorded in ulnar nerve innervated flexor carpi ulnaris (FCU), abductor digiti minimi (ADM), and first dorsal interosseous (FDI) muscles. MUNE was obtained after processing MScantraces with MScan-fit software.Results: We found that the neurophysiological biomarkers (i.e., CMAP amplitude, CMAP area, MScan-fit MUNE and MUNIX) recorded from FCU, ADM, and FDI muscles innervated by ulnar nerve reduced gradually from proximal to distal. And, MUSIX values increased gradually from proximal to distal (respectively in FCU,ADM and FDI) muscles while MScan-fit LU and MeU values did not change, significantly. In patients with relatively unaffected CMAP amplitude ( n=13), CMAP area, MScan-fit MUNE, and MUNIX were able to show loss of subtle or early MU loss in 30,7%, 53,8%, 38,4%, respectively. Conclusion:CMAParea, MScan-fit MUNE, and MUNIX showed neurodegeneration earlier than reduction in CMAP amplitude.MUNIX and MScan-fit MUNE demonstrated a dying-back neurodegeneration pattern in ALS. MUNIX was considered to define denervation processes similar to MScan-fit MUNE, but MUSIX was better in showing re-innervation process than MScan-fit Mean Unit and Largest Unit.
Introduction: Several neurophysiological methods, i.e., motor unit number estimation (MUNE) were used to predict motor unit loss in ALS. In the last decade, relatively sophisticated methods, e.g., motor unit number index (MUNIX) and MUNE with MScan-fit software using compound muscle action potential (CMAP) scanning recordings were introduced for the same purpose. In this study, we tried to find out neurodegeneration pattern in ALS and to detect early MU loss.Methods: Twenty five patients and 25 healthy control were enrolled for this study. CMAPs, MUNIX and CMAP scanning (MScan) tests were recorded in ulnar nerve innervated flexor carpi ulnaris (FCU), abductor digiti minimi (ADM), and first dorsal interosseous (FDI) muscles. MUNE was obtained after processing MScantraces with MScan-fit software.Results: We found that the neurophysiological biomarkers (i.e., CMAP amplitude, CMAP area, MScan-fit MUNE and MUNIX) recorded from FCU, ADM, and FDI muscles innervated by ulnar nerve reduced gradually from proximal to distal. And, MUSIX values increased gradually from proximal to distal (respectively in FCU,ADM and FDI) muscles while MScan-fit LU and MeU values did not change, significantly. In patients with relatively unaffected CMAP amplitude ( n=13), CMAP area, MScan-fit MUNE, and MUNIX were able to show loss of subtle or early MU loss in 30,7%, 53,8%, 38,4%, respectively. Conclusion:CMAParea, MScan-fit MUNE, and MUNIX showed neurodegeneration earlier than reduction in CMAP amplitude.MUNIX and MScan-fit MUNE demonstrated a dying-back neurodegeneration pattern in ALS. MUNIX was considered to define denervation processes similar to MScan-fit MUNE, but MUSIX was better in showing re-innervation process than MScan-fit Mean Unit and Largest Unit.