Pınar BEKDİK, Tuba CERRAHOĞLU ŞİRİN, Serdar ARSLAN, Nurten UZUN ADATEPE

Amyotrofik lateral sklerozlu hastalarda klinik bulgular ile F yanıtları ve difüzyon tensör traktografi arasındaki ilişkinin değerlendirilmesi

Amaç: Bu çalışmanın amacı, Amiyotrofik Lateral Skleroz (ALS) hastalığında radyolojik olarak difüzyon traktografi görüntüleme (DTG) ve elektrofizyolojik olarak F yanıtlarının klinik muayeneyi destekleyip desteklemediğini göstermektir. Gereç ve Yöntem: İstanbul Üniversitesi Cerrahpaşa Cerrahpaşa Tıp Fakültesi Nöroloji Kliniği Elektromiyografi (EMG) laboratuvarına başvuran, hastalığın herhangi bir evresindeki Revize-El Escorial ve Awaji kriterlerine göre kesin ALS tanısı alan hastalar prospektif, kesitsel olarak çalışmaya alındı. Revize ALS fonksiyonel derecelendirme ölçeği (ALSFRS-R) uygulandı. Elektrofizyolojik incelemelerde rutin sinir iletim çalışmaları, F yanıtları ve iğne EMG’si incelendi. Radyolojik incelemelerde, Radyolojik incelemelerde beyin ve servikal manyetik rezonanas görüntülemede (MRG) difüzyon traktografi görüntüleme (DTG) haritaları elde edildi ve difüzyon ve fraksiyonel anizotropi (FA) değerleri karşılaştırıldı. Bulgular: Çalışmaya alınan 12 gönüllü hastanın yaş ortalaması 55.92±9.68 (43-72) idi ve 4’ü kadın, 8’i erkek bireyden oluşmaktaydı. Ortalama ALSFRS-R puanı 35.75±7.86 (24-46) idi. Hastaların 11’ine MR görüntülemesi yapıldı, bir hastanın spinal görüntülemesi yapılamadı. Görüntüleme yapılan 11 hastanın ortalama yaş 56,4±9,8 ve hastalık süresi 2,7±1,9 yıldı. Düşük DTG-FA değerlerinin, lineer regresyon analizi ile değerlendirildiğinde kötü solunum ve konuşma skorları ile ilişkili olduğu bulundu. Ulnar sinirlerdeki tekrarlayan F yanıtları da yüksek bir ALSFRS skoru ile ilişkilendirildi. Sonuç: Çalışmamızda düşük DTG-FA değerlerinin kötü solunum ve konuşma skorları ile ilişkili olduğunu gözlemledik. Düşük FA'nın solunum gibi hayati bir fonksiyonla ilişkisinin gösterilmesi, bu tekniğin hastalığın prognozunda ve ilerlemesinde yardımcı olacağına işaret edebilir. ALSFRS-R skoru yüksek olanlarda ulnar sinirlerde tekrarlayan F yanıtlarının artması ALS'nin diğer ön boynuz hastalıklarından ayırıcı tanısında kullanılıp kullanılamayacağını düşündürmektedir. Bu çalışmanın sonuçları, ileri radyolojik bir değerlendirme olan DTG-FA ve elektrofizyolojik bir yöntem olarak tekrarlayan F yanıtlarının ALS'de erken tanıyı destekleyebileceğini düşündürmektedir.

Anahtar Kelimeler:

Amiyotrofik Lateral Skleroz, F yanıtı, Difüzyon Tensor Görüntüleme, Fraksiyonel Anizotropi

Evaluation of the relationship between clinical findings and F responses and diffusion tensor tractography in patients with amyotrophic lateral sclerosis

Purpose: The aim of this study is to show whether radiologically diffusion tractography imaging (DTI) and electrophysiologically F responses methods support clinical examination in Amyotrophic Lateral Sclerosis (ALS). It is to determine the relationships between these two methods. Materials and Methods: Patients with a definite diagnosis of ALS according to the Revised-El Escorial and Awaji criteria at any stage of the disease, who applied to the Electromyography (EMG) laboratory of the Neurology Clinic of Istanbul University Cerrahpaşa, Cerrahpaşa Medical Faculty, prospectively and cross-sectionally, were included in the study. The revised ALS functional rating scale (ALSFRS-R) was scored. Electrophysiological studies included routine nerve conduction studies, F responses and needle EMG. In radiological examinations, diffusion tractography imaging (DTI) maps were obtained in brain and cervical Magnetic Resonance Imaging (MRI) and diffusivity and fractional anisotropy (FA) values were compared. Results: The mean age of the 12 volunteer patients included in the study was 55.92±9.68 (43-72), and consisted of 4 female and 8 male individuals. The mean ALSFRS-R score was 35.75±7.86 (24-46). MRI was performed in 11 of the patients, spinal imaging could not be performed in one patient. The mean age of the 11 patients who were imaging was 56.4±9.8 years and the disease duration was 2.7±1.9 years. Low values of DTI-FA were found to be associated with poor respiratory and speech scores as assessed by linear regression analysis. Repeater F responses in the ulnar nerves were also associated with a high ALSFRS-R score. Conclusion: In our study, we observed that low values of DTI-FA were associated with poor respiratory and speech scores. Showing the correlation of low FA with a vital function such as respiration may indicate that this technique will be helpful in the prognosis and progression of the disease. The increase of repeater F responses in the ulnar nerves in those with high ALSFRS-R scores suggests that it is worth investigating whether ALS can be used in differentiating from other anterior horn diseases. The results of this study suggest that DTI-FA, which is an advanced radiological evaluation, and repeater F responses as an electrophysiological method may support early diagnosis in ALS.

Keywords:

Amyotrophic Lateral Sclerosis, F response, Diffusion Tensor Imaging, Fractional Anisotropy,

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