Chiari malformation type I with cervicothoracic syringomyelia masquerading as bibrachial amyotrophy: a case report

Journal of Medical Case Reports

Said R Beydoun²  Richard A Rison³  Jeffrey R Mora¹ 

[1] Keck School of Medicine, Neuromuscular Division, University of Southern California, 1520 San Pablo Street, Suite 3000, Los Angeles, CA 90033, USA;Keck School of Medicine, Los Angeles County Medical Center, University of Southern California, 1520 San Pablo Street, Suite 3000, Los Angeles, CA 90033, USA;Department of Neurology, PIH Health Hospital-Whittier, 12401 Washington Boulevard, Whittier, California 90602, USA

关键词: Syringomyelia;    Misdiagnosis;    Distal muscle;    Chiari type 1 malformation;    Cervicothoracic;    Bulbar dysfunction;    Bibrachial amyotrophy;    Atrophy;   

Others  :  1145568
DOI  :  10.1186/1752-1947-9-11

 received in 2014-07-21, accepted in 2014-12-04,  发布年份 2015

【 摘 要 】

Introduction

Clinical presentation of syringomyelia can mimic a variety of neuromuscular disorders. A misdiagnosis can result in progressive pressure on the spinal cord, causing the development of severe irreversible neurologic deficits.

Case presentation

We report the very unusual case of a 50-year-old Latino man who developed severe distal muscle atrophy and bulbar dysfunction as a result of Chiari malformation type I with chronic cervicothoracic syringomyelia.

Conclusion

Syringomyelia is a potentially serious neurologic condition with symptoms that can mimic other neuromuscular disorders. Severe untreated cases can result in irreversible spinal cord injury. Prompt diagnosis with magnetic resonance imaging is important in both establishing diagnosis and directing further surgical management.

【 授权许可】

   
2015 Mora et al.; licensee BioMed Central.

【 图 表 】

Figure 1.

Figure 2.

Figure 3.

Figure 4.

Figure 5.

【 参考文献 】

• [1]Milhorat TH: Classification of syringomyelia. Neurosurg Focus 2000, 8(3):E1.
• [2]Stoodley MA, Jones NR, Yang L, Brown CJ: Mechanisms underlying the formation and enlargement of noncommunicating syringomyelia: experimental studies. Neurosurg Focus 2000, 8(3):E2.
• [3]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(8):989-991.
• [4]Koyangi I, Houkin K: Pathogenesis of syringomyelia associated with Chiari type 1 malformation: review of evidences and proposal of a new hypothesis. Neurosurg Rev 2000, 33:271-285.
• [5]Todor DR, Mu HTM, Milhorat TH: Pain and syringomyelia: a review. Neurosurg Focus 2000, 8(3):E11.
• [6]Paul KS, Lye RH, Strang FA, Dutton J: Arnold-Chiari malformation. Review of 71 cases. J Neurosurg 1983, 58(2):183-187.
• [7]Alden TD, Ojemann JG, Park TS: Surgical treatment of Chiari I malformation: indications and approaches. Neurosurg Focus 2001, 11(1):E2.
• [8]Verms S, Bradley WG: Atypical motor neuron disease and related motor syndromes. Semin Neurol 2001, 21(2):177-188.
• [9]Couratier P, Truong CT, Khalil M, Deviere F, Vallat JM: Clinical features of flail arm syndrome. Muscle Nerve 2000, 23:646-648.
• [10]Roser F, Ebner FH, Sixt C, Hagen JM, Tatagiba MS: Defining the line between hydromyelia and syringomyelia. A differentiation is possible based on electrophysiological and magnetic resonance imaging studies. Acta Neurochir 2010, 152(2):213-219.
• [11]Veilleux M, Stevens JC: Syringomyelia: electrophysiologic aspects. Muscle Nerve 1987, 10(5):449-458.
• [12]Navarro R, Olavarria G, Seshadri R, Gonzales-Portillo G, McLone DG, Tomita T: Surgical results of posterior fossa decompression for patients with Chiari I malformation. Childs Nerv Syst 2004, 20(5):349-356.
• [13]Novegno F, Caldarelli M, Massa A, Chieffo D, Massimi L, Pettorini B, Tamburrini G, Di Rocco C: The natural history of the Chiari Type I anomaly. J Neurosurg Pediatr 2009, 2(3):179-187.
• [14]Schuster JM, Zhang F, Norvell DC, Hermsmeyer JT: Persistent/recurrent syringomyelia after Chiari decompression – natural history and management strategies: a systematic review. Evid Based Spine Care J 2013, 4(2):116-125.