Nerve conduction studies in the early diagnosis of amyotrophic lateral sclerosis and the importance of split-hand phenomenon
Abstract
Aim: The heterogeneity of the Amyotrophic Lateral Sclerosis (ALS) clinical phenotypes leads to difficulties in early diagnosis. The ‘split hand’ sign is defined as the thenar muscles that are more prominently affected by hypothenar. In this study, the results of the initial nerve conduction study of the patients were compared with those of the controls in order to increase the findings supporting early diagnosis.
Material and Method: Seventy-five patients who were diagnosed with ALS in our clinic were included in the study. The initial ENMG findings of the patients were compared with those of 70 healthy controls: Distal motor latency (DML), the compound muscle action potential (CMAP) amplitude, velocity in the motor conduction of median, ulnar, peroneal and tibial nerves; distal latency, amplitude, velocity in sensorial conduction were evaluated. Ulnar/median DML and ulnar/median CMAP amplitude ratios were examined.
Results: In ALS group, DMLs of the median, ulnar, peroneal, and tibial nerves were significantly longer, and CMAP amplitudes were significantly smaller than those of the controls. The sensory conductions of the median, ulnar, and sural nerves were not statistically different between the groups. The ulnar/median DML ratio of the patients was lower than the ratio of controls (0.73/0.80;p=0.003); while the ulnar/median CMAP amplitude ratio was greater (1.40/1.11; p=0.002).
Conclusion: Prolonged DML and reduced amplitudes were observed in the motor nerve conduction of ALS patients in the early period. The results of the present study also support the presence of split-hand phenomenon even in early period of limb-onset ALS (both upper and lower). These findings suggest that nerve conduction studies and electrophysiologically detected split-hand sign are important clues for the early diagnosis of ALS in case of heterogeneous clinical phenotype.
References
- Wijesekera LC, Leigh PN. Amyotrophic lateral sclerosis. Orphanet J Rare Dis 2009; 4: 3-7
- Kiernan MC, Vucic S, Cheah BC, et al. Amyotrophic lateral sclerosis. Lancet 2011; 377: 942–55
- Al-Chalabi A, van den Berg LH, Veldink J. Gene discovery in amyotrophic lateral sclerosis: implications for clinical management. Nat Rev Neurol 2017; 13: 96–104
- Bendotti C, Bonetto V, Pupillo E, et al. Focus on the heterogeneity of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2020; 21: 485-95
- Das K, Nag C, Ghosh M. Familial, environmental, and occupational risk factors in development of amyotrophic lateral sclerosis. N Am J Med Sci 2012; 4: 350–5
- Lomen-Hoerth C. Amyotrophic lateral sclerosis from bench to bedside. Semin Neurol 2008; 28: 205–11
- Weber, M., Eisen, A., Stewart, H., Hirota, N. The split hand in ALS has a cortical basis. J Neurol Sci 2000; 180: 66-70
- Kuwabara S, Sonoo M, Komori T, et al. Tokyo Metropolitan Neuromuscular Electrodiagnosis Study Group. Dissociated small hand muscle atrophy in amyotrophic lateral sclerosis: frequency, extent, and specificity. Muscle Nerve 2008; 37: 426-430
- Eisen A, Kuwabara S. The split hand syndrome in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 2012; 83: 399-403.
- Menon P, Kiernan MC, Vucic S. Cortical dysfunction underlies the development of the split-hand in amyotrophic lateral sclerosis PLoS One 2014; 24: e87124
- Wilbourn AJ . The "split hand syndrome". Muscle Nerve 2000; 23:138-42
- Corcia P, Bede P, Pradat PF, et al. Split-hand and split-limb phenomena in amyotrophic lateral sclerosis: pathophysiology, electrophysiology and clinical manifestations. J Neurol Neurosurg Psychiatry 2021; 92:1126–30
- Shibuya K, Misawa S, Nasu S, et al. Split hand syndrome in amyotrophic lateral sclerosis: different excitability changes in the thenar and hypothenar motor axons. Neurol Neurosurg Psychiatry 2013; 84: 969-72.
- Hu N, Wang J, Liu M. Split hand in amyotrophic lateral sclerosis: A systematic review and meta-analysis. J Clin Neurosci 2021; 90: 293-301
- Park D, Park JS. Terminal latency abnormality in amyotrophic lateral sclerosis without split hand syndrome. Neurol Sci 2017; 38: 775-81
- Susanna B. Park, Matthew C. Kiernan, Steve Vucic . Axonal Excitability in Amyotrophic Lateral Sclerosis: Axonal Excitability in ALS. Neurotherapeutics 2017; 14: 78–90.
- Fang J, Cui L, Liu M, et al. Differences in Dysfunction of Thenar and Hypothenar Motoneurons in Amyotrophic Lateral Sclerosis. Front Hum Neurosci 2016; 10: e99
- Chi`o A, Calvo A, Moglia C, et al. Phenotypic heterogeneity of amyotrophic lateral sclerosis: a population based study. J Neurol Neurosurg Psychiatry 2011; 82: 740–6.
- Jin X, Jiang JY, Lu FZ, Xia XL, Wang LX, Zheng CJ. Electrophysiological differences between Hirayama disease, amyotrophic lateral sclerosis and cervical spondylotic amyotrophy. BMC Musculoskelet Disord 2014; 15: e349
- Bedlack RS. Amyotrophic lateral sclerosis: current practice and future treatments. Curr Opin Neurol 2010; 23: 524–9
- Aksoy D, Cevik B, Solmaz V, Kurt SG. Clinical, demographic and prognostic features of sporadic amyotrophic lateral sclerosis in Northern Turkey. Int J Neurosci 2014; 124: 68-73
- Richards D, Morren J A, Pioro EP. Time to diagnosis and factors affecting diagnostic delay in amyotrophic lateral sclerosis. J Neurol Sci 2020; 417: e117054
Abstract
Amaç: Amyotrofik Lateral Skleroz' da (ALS) özellikle başlangıç döneminde klinik ve muayene bulgularının heterojenliği erken tanıda zorluklara yol açmaktadır. 'Ayrık el' işareti, ALS' de tenar kasların hipotenar kaslardan daha belirgin olarak etkilenmesini tanımlamak için kullanılır. Bu çalışmada erken tanıyı destekleyen bulguları artırmak için hastaların kliniğimizdeki ilk sinir iletim çalışmalarının sonuçları araştırıldı ve kontrol grubuyla karşılaştırıldı.
Gereç ve Yöntem: Kliniğimizde ALS tanısı ile takip edilen 75 hasta çalışmaya dahil edildi. Hastaların ilk elektronöromyografi bulguları 70 sağlıklı kontrol ile karşılaştırıldı: Distal motor latans (DML), bileşik kas aksiyon potansiyeli (CMAP) amplitüdü, median, ulnar, peroneal ve tibial sinirlerin motor iletim hızları; distal latanslar, amplitüdler ve duyusal iletim hızları değerlendirildi. Ayrıca ulnar/median DML ve ulnar/median CMAP amplitüd oranları karşılaştırıldı.
Bulgular: ALS grubunda kontrol grubuna göre median, ulnar, peroneal ve tibial sinirlerin DML' leri anlamlı olarak daha uzundu ve CMAP amplitüdleri anlamlı olarak daha küçüktü p˂0.05). Median, ulnar ve sural sinirlerin duyusal iletimleri gruplar arasında istatistiksel olarak farklı değildi. Hastaların ulnar/median DML oranı kontrollere göre daha düşüktü (0.73/0.80;p=0.003); ulnar/median CMAP amplitüd oranı ise daha yüksekti (1.40/1.11; p=0.002).
Sonuç: ALS hastalarının erken dönemde motor sinir iletimlerinde uzamış DML ve düşük amplitüdler gözlendi. Bu çalışmada sonuçlarımız, ekstremite başlangıçlı ALS' nin (hem üst hem de alt) erken döneminde bile Ayrık el fenomeninin varlığını ve önemini desteklemektedir. Bulgularımız, ALS' nin spesifik muayene ve elektrofizyoloji bulgularının oldukça heterojen olduğu başlangıç döneminde dahi sinir iletim çalışmaları ve Ayrık el işaretinin erken tanıda önemli ipuçları olduğunu düşündürmektedir.
References
- Wijesekera LC, Leigh PN. Amyotrophic lateral sclerosis. Orphanet J Rare Dis 2009; 4: 3-7
- Kiernan MC, Vucic S, Cheah BC, et al. Amyotrophic lateral sclerosis. Lancet 2011; 377: 942–55
- Al-Chalabi A, van den Berg LH, Veldink J. Gene discovery in amyotrophic lateral sclerosis: implications for clinical management. Nat Rev Neurol 2017; 13: 96–104
- Bendotti C, Bonetto V, Pupillo E, et al. Focus on the heterogeneity of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2020; 21: 485-95
- Das K, Nag C, Ghosh M. Familial, environmental, and occupational risk factors in development of amyotrophic lateral sclerosis. N Am J Med Sci 2012; 4: 350–5
- Lomen-Hoerth C. Amyotrophic lateral sclerosis from bench to bedside. Semin Neurol 2008; 28: 205–11
- Weber, M., Eisen, A., Stewart, H., Hirota, N. The split hand in ALS has a cortical basis. J Neurol Sci 2000; 180: 66-70
- Kuwabara S, Sonoo M, Komori T, et al. Tokyo Metropolitan Neuromuscular Electrodiagnosis Study Group. Dissociated small hand muscle atrophy in amyotrophic lateral sclerosis: frequency, extent, and specificity. Muscle Nerve 2008; 37: 426-430
- Eisen A, Kuwabara S. The split hand syndrome in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 2012; 83: 399-403.
- Menon P, Kiernan MC, Vucic S. Cortical dysfunction underlies the development of the split-hand in amyotrophic lateral sclerosis PLoS One 2014; 24: e87124
- Wilbourn AJ . The "split hand syndrome". Muscle Nerve 2000; 23:138-42
- Corcia P, Bede P, Pradat PF, et al. Split-hand and split-limb phenomena in amyotrophic lateral sclerosis: pathophysiology, electrophysiology and clinical manifestations. J Neurol Neurosurg Psychiatry 2021; 92:1126–30
- Shibuya K, Misawa S, Nasu S, et al. Split hand syndrome in amyotrophic lateral sclerosis: different excitability changes in the thenar and hypothenar motor axons. Neurol Neurosurg Psychiatry 2013; 84: 969-72.
- Hu N, Wang J, Liu M. Split hand in amyotrophic lateral sclerosis: A systematic review and meta-analysis. J Clin Neurosci 2021; 90: 293-301
- Park D, Park JS. Terminal latency abnormality in amyotrophic lateral sclerosis without split hand syndrome. Neurol Sci 2017; 38: 775-81
- Susanna B. Park, Matthew C. Kiernan, Steve Vucic . Axonal Excitability in Amyotrophic Lateral Sclerosis: Axonal Excitability in ALS. Neurotherapeutics 2017; 14: 78–90.
- Fang J, Cui L, Liu M, et al. Differences in Dysfunction of Thenar and Hypothenar Motoneurons in Amyotrophic Lateral Sclerosis. Front Hum Neurosci 2016; 10: e99
- Chi`o A, Calvo A, Moglia C, et al. Phenotypic heterogeneity of amyotrophic lateral sclerosis: a population based study. J Neurol Neurosurg Psychiatry 2011; 82: 740–6.
- Jin X, Jiang JY, Lu FZ, Xia XL, Wang LX, Zheng CJ. Electrophysiological differences between Hirayama disease, amyotrophic lateral sclerosis and cervical spondylotic amyotrophy. BMC Musculoskelet Disord 2014; 15: e349
- Bedlack RS. Amyotrophic lateral sclerosis: current practice and future treatments. Curr Opin Neurol 2010; 23: 524–9
- Aksoy D, Cevik B, Solmaz V, Kurt SG. Clinical, demographic and prognostic features of sporadic amyotrophic lateral sclerosis in Northern Turkey. Int J Neurosci 2014; 124: 68-73
- Richards D, Morren J A, Pioro EP. Time to diagnosis and factors affecting diagnostic delay in amyotrophic lateral sclerosis. J Neurol Sci 2020; 417: e117054
Details
| Primary Language | English |
|---|---|
| Subjects | Clinical Sciences |
| Journal Section | Original Article |
| Authors | |
| Publication Date | August 31, 2022 |
| Submission Date | June 14, 2022 |
| Published in Issue | Year 2022 Volume: 32 Issue: 4 |
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