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Cervical spondylotic amyotrophy: a systematic review

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Abstract

Purpose

Cervical spondylotic amyotrophy (CSA) is characterized by upper limb muscle weakness and atrophy, without sensory deficits. The pathophysiology of CSA has been attributed to selective injury to the ventral nerve root and/or anterior horn of the spinal cord. This review aimed to delineate the history of CSA and to describe the epidemiology, etiology, pathophysiology, classification, clinical features, radiological and electrophysiological assessment, diagnosis, differential diagnosis, natural history and treatment of CSA.

Methods

A comprehensive search of PubMed, EMBASE, Cochrane library and Web of Science databases was conducted, from their inception to April 3, 2018.

Results

Clinically, CSA is classified into three types: a proximal-type (involving the scapular muscles, deltoid and biceps), a distal-type (involving the triceps and muscles of the forearm and hand) and a diffuse-type (involving features of both the distal- and proximal-type). Diagnosis requires documentation of muscle atrophy, without significant sensory deficits, supported by careful neurological, radiological and neurophysiological assessments, with amyotrophic lateral sclerosis, Parsonage–Turner syndrome, rotator cuff tear and Hirayama disease being the principle differential diagnoses. Conservative management of CSA includes cervical traction, neck immobilization and physical therapy, with vitamin B12 or E administration being useful in some patients. Surgical treatment, including anterior decompression and fusion or laminoplasty, with or without foraminotomy, is indicated after conservative treatment failure. Factors associated with a poor outcome include the distal-type CSA, long symptom duration, older age and greater preoperative muscle weakness.

Conclusion

Although the disease process of CSA is self-limited, treatment remains challenging, leaving scope for future studies.

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References

  1. Kaneko K, Taguchi T, Toyoda K, Kato Y, Azuma Y, Kawai S (2004) Distal-type cervical spondylotic amyotrophy: assessment of pathophysiology from radiological findings on magnetic resonance imaging and epidurally recorded spinal cord responses. Spine (Phila Pa 1976) 29(9):E185–E188

    Article  Google Scholar 

  2. Yanagi T, Kato H, Sobue I (1976) Clinical characteristics of cervical spondylotic amyotrophy. Rinsho shinkeigaku = Clinical neurology 16(7):520–528

    CAS  PubMed  Google Scholar 

  3. Tofuku K, Koga H, Yone K, Komiya S (2011) Conservative treatment with hyperbaric oxygen therapy for cervical spondylotic amyotrophy. Spinal Cord 49(6):749–753

    Article  CAS  PubMed  Google Scholar 

  4. Brain WR, Northfield D, Wilkinson M (1952) The neurological manifestations of cervical spondylosis. Brain 75(2):187–225

    Article  CAS  PubMed  Google Scholar 

  5. Keegan JJ (1965) The cause of dissociated motor loss in the upper extremity with cervical spondylosis. J Neurosurg 23(5):528

    Article  CAS  PubMed  Google Scholar 

  6. Sobue I, Kato H, Yanagi T (1975) Clinical characteristics and classification of cervical spondylotic myelopathy. Rinsho Seikeigaku 10:999–1006 (in Japanese)

    Google Scholar 

  7. Imajo Y, Kato Y, Kanchiku T, Suzuki H, Taguchi T (2011) Pathology and prognosis of proximal-type cervical spondylotic amyotrophy: new assessment using compound muscle action potentials of deltoid and biceps brachii muscles. Spine (Phila Pa 1976) 36(7):E476–E481

    Article  Google Scholar 

  8. Sasai K, Umeda M, Saito T, Ohnari H, Wakabayashi E, Iida H (2006) Microsurgical posterior foraminotomy with laminoplasty for cervical spondylotic radiculomyelopathy including cervical spondylotic amyotrophy. J Neurosurg Spine 5(2):126–132

    Article  PubMed  Google Scholar 

  9. Funaba M, Kanchiku T, Imajo Y et al (2017) A novel scoring system associated with surgical outcome of distal-type cervical spondylotic amyotrophy. Clin Spine Surg 30(9):E1182–E1189

    Article  PubMed  Google Scholar 

  10. Fujiwara K (2001) Cervical spondylotic amyotrophy with intramedullary cavity formation. Spine (Phila Pa 1976) 26(10):E220–E222

    Article  CAS  Google Scholar 

  11. Iizuka Y, Iizuka H, Mieda T et al (2014) Prognostic factors for cervical spondylotic amyotrophy: are signs of spinal cord involvement associated with the neurological prognosis? Spinal Cord 52(5):364–367

    Article  CAS  PubMed  Google Scholar 

  12. Zhang JT, Yang DL, Shen Y, Zhang YZ, Wang LF, Ding WY (2012) Anterior decompression in the management of unilateral cervical spondylotic amyotrophy. Orthopedics 35(12):e1792–e1797

    Article  PubMed  Google Scholar 

  13. Hashiguchi S, Ogasawara N, Watanabe A, Kawachi Y, Miki N (1997) Cervical spondylotic amyotrophy associated with Hirayama’s disease. Intern Med (Tokyo, Japan) 36(9):647–650

    Article  CAS  Google Scholar 

  14. Kameyama T, Ando T, Yanagi T, Yasui K, Sobue G (1998) Cervical spondylotic amyotrophy. Magnetic resonance imaging demonstration of intrinsic cord pathology. Spine (Phila Pa 1976) 23(4):448–452

    Article  CAS  Google Scholar 

  15. Asaka T, Satake R, Takamori M, Matsushima A (1995) Dissociated motor loss syndrome with cavities in the anterior horns. Intern Med 34(10):1027–1029

    Article  CAS  PubMed  Google Scholar 

  16. Srinivasa Rao NV, Rajshekhar V (2009) Distal-type cervical spondylotic amyotrophy: incidence and outcome after central corpectomy. J Neurosurg Spine 10(4):374–379

    Article  PubMed  Google Scholar 

  17. Zhang J, Cui C, Liu Z, Tong T, Niu R, Shen Y (2016) Predisposing factors for poor outcome of surgery for cervical spondylotic amyotrophy: a multivariate analysis. Sci Rep 6:39512

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Mori K, Yamamoto T, Nakao Y, Maeda M (2006) Cervical spondylotic amyotrophy treated by anterior decompression. Three case reports. Neurol Med Chir (Tokyo) 46(7):366–370

    Article  Google Scholar 

  19. Shinomiya K, Komori H, Matsuoka T, Mutoh N, Furuya K (1994) Neuroradiologic and electrophysiologic assessment of cervical spondylotic amyotrophy. Spine (Phila Pa 1976) 19(1):21–25

    Article  CAS  Google Scholar 

  20. Takebayashi T, Yoshimoto M, Ida K, Tsuda H, Terashima Y, Yamashita T (2013) Minimum invasive posterior decompression for cervical spondylotic amyotrophy. J Orthop Sci 18(2):205–207

    Article  PubMed  Google Scholar 

  21. Tauchi R, Imagama S, Inoh H et al (2015) Appropriate timing of surgical intervention for the proximal type of cervical spondylotic amyotrophy. Eur J Orthop Surg Traumatol 25(Suppl 1):S107–S113

    Article  PubMed  Google Scholar 

  22. Fujiwara Y, Tanaka N, Fujimoto Y, Nakanishi K, Kamei N, Ochi M (2006) Surgical outcome of posterior decompression for cervical spondylosis with unilateral upper extremity amyotrophy. Spine (Phila Pa 1976) 31(20):E728–E732

    Article  Google Scholar 

  23. Wang HL, Li HC, Jiang JY, Lu FZ, Chen WJ, Ma XS (2014) Evaluation of characteristics and surgical outcomes in cervical spondylotic amyotrophy. Indian J Orthop 48(5):511–517

    Article  PubMed  PubMed Central  Google Scholar 

  24. Tauchi R, Imagama S, Inoh H et al (2014) Characteristics and surgical results of the distal type of cervical spondylotic amyotrophy. J Neurosurg Spine 21(3):411–416

    Article  PubMed  Google Scholar 

  25. Jin X, Jiang JY, Lu FZ, Xia XL, Wang LX, Zheng CJ (2014) Electrophysiological differences between Hirayama disease, amyotrophic lateral sclerosis and cervical spondylotic amyotrophy. BMC Musculoskelet Disord 15:349

    Article  PubMed  PubMed Central  Google Scholar 

  26. Shibuya R, Yonenobu K, Yamamoto K et al (2005) Acute arm paresis with cervical spondylosis: three case reports. Surg Neurol 63(3):220–228

    Article  PubMed  Google Scholar 

  27. Imajo Y, Kato Y, Kanchiku T et al (2012) Prediction of surgical outcome for proximal-type cervical spondylotic amyotrophy novel mode of assessment using compound action potentials of deltoid and biceps brachii and central motor conduction time. Spine (Phila Pa 1976) 37(23):E1444–E1449

    Article  Google Scholar 

  28. Ahdab R, Creange A, Benaderette S, Lefaucheur JP (2009) Cervical spondylotic amyotrophy presenting as dropped head syndrome. Clin Neurol Neurosurg 111(10):874–876

    Article  PubMed  Google Scholar 

  29. Hatanaka Y, Chiba T, Okuma H, Kanbayashi T, Kuwabara M, Higashihara M (2017) Diagnostic utility of repetitive nerve stimulation test of the trapezius muscle in amyotrophic lateral sclerosis. Clin Neurophysiol 128(5):823–829

    Article  PubMed  Google Scholar 

  30. Nouri A, Tetreault L, Singh A, Karadimas SK, Fehlings MG (2015) Degenerative cervical myelopathy: epidemiology, genetics, and pathogenesis. Spine (Phila Pa 1976) 40(12):E675–E693

    Article  Google Scholar 

  31. Mizuno J, Nakagawa H, Hashizume Y (2002) Cervical amyotrophy caused by hypertrophy of the posterior longitudinal ligament. Spinal Cord 40(9):484–488

    Article  CAS  PubMed  Google Scholar 

  32. Gebere-Michael SG, Johnston JC, Metaferia GZ, Wuhib MZ, Fernandez HH (2010) Bilaterally symmetric cervical spondylotic amyotrophy: a novel presentation and review of the literature. J Neurol Sci 290(1–2):142–145

    Article  PubMed  Google Scholar 

  33. Kumar KK, Doi K, Hattori Y, Sakamoto S, Yonemura H, Montales T (2014) Reconstruction of shoulder and elbow function using multiple muscle transfers for cervical spondylotic amyotrophy. Spine (Phila Pa 1976) 39(21):E1269–E1275

    Article  Google Scholar 

  34. Iwata E, Shigematsu H, Inoue K, Egawa T, Sakamoto Y, Tanaka Y (2017) Muscle weakness in the empty and full can tests cannot differentiate rotator cuff tear from cervical spondylotic amyotrophy: pain provocation is a useful finding. Open Orthop J 11:1081–1086

    Article  PubMed  PubMed Central  Google Scholar 

  35. Sasaki S, Iwata M (1999) Atypical form of amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 66(5):581–585

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  36. Koda M, Furuya T, Rokkaku T et al (2017) Drop finger caused by 8th cervical nerve root impairment: a clinical case series. Eur Spine J 26(4):1096–1100

    Article  PubMed  Google Scholar 

  37. Badar F, Azfar SF, Ahmad I, Kirmai S (2011) MRI feature of cervical spondylotic amyotrophy: snake eye appearance. JK Science 14(2):107

    Google Scholar 

  38. Zhang ZF, Wang HG (2014) Is the “snake-eye” MRI sign correlated to anterior spinal artery occlusion on CT angiography in cervical spondylotic myelopathy and amyotrophy? Eur Spine J 23(7):1541–1547

    Article  PubMed  Google Scholar 

  39. Inui Y, Miyamoto H, Sumi M, Uno K (2011) Clinical outcomes and predictive factors relating to prognosis of conservative and surgical treatments for cervical spondylotic amyotrophy. Spine (Phila Pa 1976) 36(10):794–799

    Article  Google Scholar 

  40. Jiang SD, Jiang LS, Dai LY (2011) Cervical spondylotic amyotrophy. Eur Spine J 20(3):351–357

    Article  PubMed  Google Scholar 

  41. Uchida K, Nakajima H, Yayama T et al (2009) Anterior and posterior decompressive surgery for progressive amyotrophy associated with cervical spondylosis: a retrospective study of 51 patients. J Neurosurg Spine 11(3):330–337

    Article  PubMed  Google Scholar 

  42. Shibuya K, Misawa S, Nasu S et al (2013) Split hand syndrome in amyotrophic lateral sclerosis: different excitability changes in the thenar and hypothenar motor axons. J Neurol Neurosurg Psychiatry 84(9):969–972

    Article  PubMed  Google Scholar 

  43. Park D, Park JS (2017) Terminal latency abnormality in amyotrophic lateral sclerosis without split hand syndrome. Neurol Sci 38(5):775–781

    Article  PubMed  Google Scholar 

  44. Stark RJ, Kennard C, Swash M (1981) Hand wasting in spondylotic high cord compression: an electromyographic study. Ann Neurol 9(1):58–62

    Article  CAS  PubMed  Google Scholar 

  45. Fang J, Liu MS, Guan YZ et al (2016) Pattern differences of small hand muscle atrophy in amyotrophic lateral sclerosis and mimic disorders. Chin Med J (Engl) 129(7):792–798

    Article  Google Scholar 

  46. Iwata E, Shigematsu H, Inoue K et al (2018) Biceps-related physical findings are useful to prevent misdiagnosis of cervical spondylotic amyotrophy as a rotator cuff tear. Asian Spine J12(1):69–73

    Article  Google Scholar 

  47. Miller JD, Pruitt S, McDonald TJ (2000) Acute brachial plexus neuritis: an uncommon cause of shoulder pain. Am Fam Physician 62(9):2067–2072

    CAS  PubMed  Google Scholar 

  48. Rowland LP (1998) Diagnosis of amyotrophic lateral sclerosis. J Neurol Sci 160(Suppl 1):S6–S24

    Article  PubMed  Google Scholar 

  49. Yamada M, Furukawa Y, Hirohata M (2003) Amyotrophic lateral sclerosis: frequent complications by cervical spondylosis. J Orthop Sci 8(6):878–881

    Article  PubMed  Google Scholar 

  50. Ghasemi M (2016) Amyotrophic lateral sclerosis mimic syndromes. Iran J Neurol 15(2):85–91

    PubMed  PubMed Central  Google Scholar 

  51. Kuwabara S, Mizobuchi K, Ogawara K, Hattori T (1999) Dissociated small hand muscle involvement in amyotrophic lateral sclerosis detected by motor unit number estimates. Muscle Nerve 22(7):870–873

    Article  CAS  PubMed  Google Scholar 

  52. Kuncl RW, Cornblath DR, Griffin JW (1988) Assessment of thoracic paraspinal muscles in the diagnosis of ALS. Muscle Nerve 11(5):484–492

    Article  CAS  PubMed  Google Scholar 

  53. Ellenberg MR, Honet JC, Treanor WJ (1994) Cervical radiculopathy. Arch Phys Med Rehabil 75(3):342–352

    Article  CAS  PubMed  Google Scholar 

  54. Tauchi R, Imagama S, Inoh H et al (2013) Risk factors for a poor outcome following surgical treatment of cervical spondylotic amyotrophy: a multicenter study. Eur Spine J 22(1):156–161

    Article  PubMed  Google Scholar 

  55. Sonoo M, Kuwabara S, Shimizu T et al (2009) Utility of trapezius EMG gor diagnosis of amyotrophic lateral sclerosis. Muscle Nerve 39(1):63–70

    Article  PubMed  Google Scholar 

  56. Shindo K, Watanabe H, Tanaka H, Nagasaka T, Tsunoda S, Shiozawa Z (2002) A comparison of sympathetic outflow to muscles between cervical spondylotic amyotrophy and ALS. Amyotroph Lateral Scler Other Motor Neuron Disord 3(4):233–238

    Article  PubMed  Google Scholar 

  57. Jiang M, Yan X, Yan LR, Zhan YB, Hu HT (2017) Value of split hand in the differential diagnosis of amyotrophic lateral sclerosis and cervical spondylotic amyotrophy. Zhonghua Yi Xue Za Zhi 97(47):3729–3732

    CAS  PubMed  Google Scholar 

  58. Hatanaka Y, Higashihara M, Chiba T, Miyaji Y, Kawamura Y, Sonoo M (2017) Utility of repetitive nerve stimulation test for ALS diagnosis. Clin Neurophysiol 128(5):823–829

    Article  PubMed  Google Scholar 

  59. Zheng C, Jin X, Zhu Y, Lu F, Jiang J, Xia X (2017) Repetitive nerve stimulation as a diagnostic aid for distinguishing cervical spondylotic amyotrophy from amyotrophic lateral sclerosis. Eur Spine J 26(7):1929–1936

    Article  PubMed  Google Scholar 

  60. Hirayama K, Tsubaki T, Toyokura Y, Okinaka S (1965) Juvenile muscular atrophy of unilateral upper extremity. Wakayama Med Rep 9(3):161–167

    Google Scholar 

  61. Okumura H, Homma TT (1994) Juvenile compression myelopathy in the cervical spine. Spine (Phila Pa 1976) 19(1):72–76

    Article  CAS  Google Scholar 

  62. Baxter C, Miller TA, Ross DC, Doherty C (2018) Treatment of cervical spondylotic amyotrophy with nerve transfers. J Hand Surg 43(7):e1–e4

    Article  Google Scholar 

  63. Meng YK, Zhang J, Yang Y et al (2017) Clinical diagnosis and management of cervical spondylotic amyotrophy. Zhonghua Yi Xue Za Zhi 97(17):1320–1323

    CAS  PubMed  Google Scholar 

  64. Kong LD, Wang LF, Zhang JT, Zhang YZ, Ding WY, Shen Y (2015) Predictive factors relating to prognosis of anterior decompressive surgery for proximal-type cervical spondylotic amyotrophy. J Back Musculoskelet Rehabil 28(2):261–266

    Article  PubMed  Google Scholar 

  65. Liu XY, Li CD, Yi XD, Li H, Yu ZR (2007) Surgical treatment of cervical spondylotic amyotrophy. Zhonghua Yi Xue Za Zhi 87(47):3339–3342

    PubMed  Google Scholar 

  66. Terai H, Suzuki A, Toyoda H et al (2014) Tandem keyhole foraminotomy in the treatment of cervical radiculopathy: retrospective review of 35 cases. J Orthop Surg Res 16(9):38

    Article  Google Scholar 

  67. Dorsen M, Ehni G (1979) Cervical spondylotic radiculopathy producing motor manifestations mimicking primary muscular atrophy. Neurosurgery 5(4):427–431

    Article  CAS  PubMed  Google Scholar 

  68. Ebara S, Yonenobu K, Fujiwara K, Yamashita K, Ono K (1988) Myelopathy hand characterized by muscle wasting. A different type of myelopathy hand in patients with cervical spondylosis. Spine (Phila Pa 1976) 13(7):785–791

    Article  CAS  Google Scholar 

  69. Querin G, Mendili MM, Lenglet T et al (2018) The spinal and cerebral profile of adult spinal-muscular atrophy: a multimodal imaging study. Neuroimage Clin. https://doi.org/10.1016/j.nicl.2018.101618

    Article  PubMed  PubMed Central  Google Scholar 

  70. Visocchi M, Conforti G, Roselli R et al (2015) From less to maximally invasiveness in cervical spine surgery. Int J Surg Case Rep 9:85–88

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  71. Spallone A, Marchione P, Li Voti P et al (2014) Anterior cervical discectomy and fusion with “mini-invasive” harvesting of iliac crest graft versus polyetheretherketone (PEEK) cages: a retrospective outcome analysis. Int J Surg 12(12):1328–1332

    Article  CAS  PubMed  Google Scholar 

  72. Barbagallo GM, Certo F, Visocchi M et al (2014) Double-level cervical total disc replacement for adjacent segment disease: is it a useful treatment? Description of late onset heterotopic ossification and review of the literature. Eur Rev Med Pharmacol Sci 18(1 Suppl):15–23

    CAS  PubMed  Google Scholar 

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Funding

This study was funded by Jilin Province Department of Finance (SCZSY201709 to Rui Gu).

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Correspondence to Rui Gu or Jianhui Zhao.

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Luo, W., Li, Y., Xu, Q. et al. Cervical spondylotic amyotrophy: a systematic review. Eur Spine J 28, 2293–2301 (2019). https://doi.org/10.1007/s00586-019-05990-7

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  • DOI: https://doi.org/10.1007/s00586-019-05990-7

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