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

Yıl 2025, Cilt: 10 Sayı: 3, 239 - 246, 15.09.2025

Nimet Ucaroglu Can

https://doi.org/10.26453/otjhs.1682365

Öz

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.

Anahtar Kelimeler

Amyotrophic lateral sclerosis , cervical spondylotic myelopathy , syringomyelia

Etik Beyan

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

Öz

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.

Anahtar Kelimeler

Amiyotrofik lateral skleroz , servikal miyelopati , siringomiyeli

Etik Beyan

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

Kaynakça

• Feldman EL, Goutman SA, Petri S, et al. Amyotrophic lateral sclerosis. Lancet. 2022;400:1363-1380. doi:10.1016/S0140-6736(22)01272-7
• Bekdik P, Baslo MB. Investigation of ongoing denervation and reinnervation in amyotrophic lateral sclerosis by using concentric needle electrode with single fiber electromyography method. Noro Psikiyatr Ars. 2023;60:298-303. doi:10.29399/npa.28162
• Theodore N. Degenerative cervical spondylosis. N Engl J Med. 2020;383(2):159-168. doi:10.1056/NEJMra2003558
• Opara J, Odzimek M. Cervical spondylotic myelopathy-diagnostics and clinimetrics. Diagnostics (Basel). 2024;14:556. doi:10.3390/diagnostics14050556
• Emary PC, Turner AJ. Cervical spondylotic myelopathy in a 68-year-old man diagnosed with amyotrophic lateral sclerosis. J Can Chiropr Assoc. 2024;68:172-176.
• Shin JJ, Yoo SJ, Kim TW, et al. Radiological and clinical significance of cervical dynamic magnetic resonance imaging for cervical spondylotic myelopathy. Neurospine. 2024;21:443-454. doi:10.14245/ns.2448166.083
• Flint G. Syringomyelia: diagnosis and management. Pract Neurol. 2021;21:403-411. doi:10.1136/practneurol-2021-002994
• Leclerc A, Matveeff L, Emery E. Syringomyelia and hydromyelia: current understanding and neurosurgical management. Rev Neurol (Paris). 2021;177:498-507. doi:10.1016/j.neurol.2020.07.004
• Guan J, Yuan C, Zhang C, et al. A novel classification and its clinical significance in Chiari I malformation with syringomyelia based on high-resolution MRI. Eur Spine J. 2021;30:1623-1634. doi:10.1007/s00586-021-06746-y
• Meyer T. Amyotrophic lateral sclerosis (ALS) - diagnosis, course of disease and treatment options. Deutsche medizinische Wochenschrift. 2021;146:1613-1618. doi:10.1055/a-1562-7882
• Wolfson C, Gauvin DE, Ishola F, Oskoui M. Global prevalence and incidence of amyotrophic lateral sclerosis: a systematic review. Neurology. 2023;101:e613-e623. doi:10.1212/WNL.0000000000207474
• Goutman SA, Hardiman O, Al-Chalabi A, et al. Recent advances in the diagnosis and prognosis of amyotrophic lateral sclerosis. Lancet Neurol. 2022;21:480-493. doi:10.1016/S1474-4422(21)00465-8
• Wu JC, Ko CC, Yen YS, et al. Epidemiology of cervical spondylotic myelopathy and its risk of causing spinal cord injury: a national cohort study. Neurosurg Focus. 2013;35:E10. doi:10.3171/2013.4.FOCUS13122
• McCormick JR, Sama AJ, Schiller NC, Butler AJ, Donnally CJ 3rd. Cervical spondylotic myelopathy: a guide to diagnosis and management. J Am Board Fam Med. 2020;33:303-13. doi:10.3122/jabfm.2020.02.190195
• Jajeh H, Lee A, Charls R, Coffin M, Sood A, Elgafy H. A clinical review of hand manifestations of cervical myelopathy, cervical radiculopathy, radial, ulnar, and median nerve neuropathies. J Spine Surg. 2024;10:120-134. doi:10.21037/jss-23-39
• Khattak ZK, Jiao X, Hu T, Shao Q, Sun X, Zhao X, Gu D. Investigation of gait and balance function in cervical spondylotic myelopathy patients using wearable sensors. Spine J. 2023:1127-1136. doi:10.1016/j.spinee.2023.03.004
• Han C, Wang J, Wang L, Gong Q, Huang W. Sciatica-like pain caused by cervical spondylotic myelopathy: four case reports and systematic review. Front Med (Lausanne). 2024;11:1429618. doi:10.3389/fmed.2024.1429618
• Donnally CJ 3rd, Patel PD, Canseco JA, Vaccaro AR, Kepler CK. Current management of cervical spondylotic myelopathy. Clin Spine Surg. 2022;35:E68-E76. doi:10.1097/BSD.0000000000001113.
• Rosenblum JS, Pomeraniec IJ, Heiss JD. Chiari malformation (update on diagnosis and treatment). Neurol Clin. 2022;40:297-307. doi:10.1016/j.ncl.2021.11.007
• Weier K, Naegelin Y, Thoeni A, et al. Non-communicating syringomyelia: a feature of spinal cord involvement in multiple sclerosis. Brain. 2008;131:1776-82. doi:10.1093/brain/awn068
• Brickell KL, Anderson NE, Charleston AJ, Hope JK, Bok AP, Barber PA. Ethnic differences in syringomyelia in New Zealand. J Neurol Neurosurg Psychiatry. 2006;77:989-91. doi:10.1136/jnnp.2005.081240
• Keskin NK, Yurtluk MD, Başdemirci M, Başdemirci O, Taçyıldız AE, Akbarov P. Evalu-ating incidental findings in cervical MRI scans: the prevalence and clinical relevance of incidental findings. J Turk Spinal Surg. 2025 Jan 22;36(1):35-39. doi: 10.4274/jtss.galenos.2024.73792.
• Bogdanov EI, Mendelevich EG. Syrinx size and duration of symptoms predict the pace of progressive myelopathy: retrospective analysis of 103 unoperated cases with craniocervical junction malformations and syringomyelia. Clin Neurol Neurosurg. 2002;104(2):90-97. doi:10.1016/s0303-8467(01)00189-5