Evaluation of Cases Diagnosed with Cervical Myelopathy or Syringomyelia Referred with a Preliminary Diagnosis of Amyotrophic Lateral Sclerosis

Abstract

Objective: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease involving both upper and lower motor neurons. Upper motor neuron signs include spasticity and hyperreflexia, while lower motor neuron signs include weakness, muscle atrophy, and fasciculations. Because of these signs, ALS can be confused with cervical spondylotic myelopathy (CSM), syringomyelia, and similar diseases. This study aimed to evaluate the clinical and demographic characteristics of patients diagnosed with CSM or syringomyelia after initially being referred with suspected ALS. Materials and Methods: Ten patients referred to our neurology clinic between January 2018 and June 2020, with a preliminary diagnosis of ALS, were evaluated. Those patients later diagnosed with CSM or syringomyelia after neurological examination, including electromyography (EMG) and magnetic resonance imaging (MRI), were included in the study. Results: Ten patients (mean age 65.6 years) were analysed. Thenar and hypothenar atrophy was observed in 8 patients (80%). EMG revealed fasciculations and subacute denervation in cervical myotomes in all patients; 1patietnt had lumbar involvement. EMG of rectus abdominis and genioglossus muscles was normal. Thenar and hypothenar atrophy, fasciculation, and denervation in cervical and lumbar myotomes on EMG are similar to ALS signs. A normal rectus abdominis and genioglossus muscle EMG excludes the diagnosis of ALS. Conclusions: CSM and syringomyelia should be considered in the differential diagnosis of ALS. A detailed history, neurological examination, EMG, and MRI are essential for diagnostic accuracy.

Keywords

• Amyotrophic lateral sclerosis
• cervical spondylotic myelopathy
• syringomyelia

Ethical Statement

The study was approved by the Sakarya University Faculty of Medicine Ethical Committee (Date:10.07.2020, decision No: E.6172), and conducted in compliance with the Helsinki Declaration.

Amiyotrofik Lateral Skleroz Ön Tanısıyla Sevk Edilen Servikal Miyelopati veya Siringomiyeli Tanısı Almış Olguların Değerlendirilmesi

Abstract

Amaç: Amiyotrofik lateral skleroz (ALS), hem üst hem de alt motor nöronların dejenerasyonu ile karakterize, ilerleyici bir nörodejeneratif hastalıktır. Üst motor nöron bulguları arasında spastisite ve hiperrefleksi; alt motor nöron bulguları arasında ise kas güçsüzlüğü, atrofi ve fasikülasyonlar yer alır. Bu bulgular nedeniyle ALS, servikal spondilotik miyelopati (SSM), siringomiyeli ve benzeri hastalıklarla karıştırılabilir Bu çalışmada, ALS şüphesiyle sevk edilen ve CSM veya siringomiyeli tanısı alan hastaların klinik ve demografik özelliklerinin değerlendirilmesi amaçlanmıştır. Materyal ve Metot: Ocak 2018 – Haziran 2020 tarihleri arasında nöroloji kliniğimize ALS ön tanısıyla sevk edilen 10 hasta değerlendirildi. Nörolojik muayene, elektromiyografi (EMG) ve manyetik rezonans görüntüleme (MRG) sonrasında CSM veya siringomiyeli tanısı konulan hastalar çalışmaya alındı. Bulgular: On hasta (ortalama yaş 65,6 yıl) analiz edildi. 8 hastada (%80) tenar ve hipotenar atrofi gözlendi. EMG'de tüm hastalarda servikal miyotomlarda fasikülasyonlar ve denervasyon görüldü; 1 hastada lomber bölgede tutulum vardı. Rektus abdominis ve genioglossus kaslarının EMG'si normaldi. Tenar ve hipotenar atrofi gözlenmesi, EMG'de servikal ve lomber miyotomlarda fasikülasyon ve denervasyon görülmesi ALS ile benzerlik göstermektedir. Rektus abdominis ve genioglossus kaslarının EMG'sinin normal olması ALS tanısından uzaklaştırmaktadır. Sonuç: ALS ayırıcı tanısında CSM ve siringomiyeli düşünülmelidir. Ayrıntılı öykü, nörolojik muayene, EMG ve MRG tanı doğruluğu için gereklidir.

Keywords

• Amiyotrofik lateral skleroz
• servikal miyelopati
• siringomiyeli

Ethical Statement

Çalışma Sakarya Üniversitesi Tıp Fakültesi Etik Kurulu tarafından onaylandı (Tarih:10.07.2020, karar no:E.6172) ve Helsinki Deklarasyonu’na uygun olarak yürütüldü.

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