Decremental responses to repetitive nerve stimulation in X-linked bulbospinal muscular atrophy

Journal of Clinical Neurology (Korea)

Volumn 9, Issue 1, 2013, Pages 32-35

  Kim, Jee Young ^a   Park, Kee Duk ^b   Kim, Seung Min ^c   Sunwoo, Il Nam ^d  

^a Kwandong University College of Medicine   (South Korea)

^b Ewha Womans University School of Medicine   (South Korea)

^c Yonsei University College of Medicine   (South Korea)

^d Sunwoo and Cho Neurology Clinic   (South Korea)

Author keywords

Bulbospinal; Motor neuron disease; Muscular atrophy; Myasthenia gravis; Neuromuscular junction

Indexed keywords

EID: 84887848096     PISSN: 17386586     EISSN: 20055013     Source Type: Journal    
DOI: 10.3988/jcn.2013.9.1.32     Document Type: Article

References (16)

1. Kim JY, Park KD, Kim SM, Sunwoo IN. Repetitive nerve stimulation test in amyotrophic lateral sclerosis with predominant oropharyngeal manifestations. J Clin Neurol 2011;7:31-33.
2. Denys EH, Norris FHJr. Amyotrophic lateral sclerosis. Impairment of neuromuscular transmission. Arch Neurol 1979;36:202-205.
3. Bernstein LP, Antel JP. Motor neuron disease: Decremental responses to repetitive nerve stimulation. Neurology 1981;31:204-207.
4. Wang FC, De Pasqua V, Gérard P, Delwaide PJ. Prognostic value of decremental responses to repetitive nerve stimulation in ALS patients. Neurology 2001;57:897-899.
5. Killian JM, Wilfong AA, Burnett L, Appel SH, Boland D. Decremental motor responses to repetitive nerve stimulation in ALS. Muscle Nerve 1994;17:747-754.
6. Yamada M, Inaba A, Shiojiri T. X-linked spinal and bulbar muscular atrophy with myasthenic symptoms. J Neurol Sci 1997;146:183-185.
7. Ertekin C, Sirin H. X-linked bulbospinal muscular atrophy (Kenne-dy's syndrome): A report of three cases. Acta Neurol Scand 1993;87: 56-61.
8. Boz C, Kalay E, Sahin N, Velioglu S, Ozmenoglu M, Karagüzel A. Ocular myasthenia gravis associated with x-linked recessive spinal and bulbar muscular atrophy. J Clin Neuromuscul Dis 2004;5:115-118.
9. Lee BJ, Kwon KH, Kim SM, Na HR, Lee BC, Ryu SH, et al. A case of Kennedy syndrome mimicking myasthenia gravis. J Korean Neurol Assoc 2001;19:544-546.
10. Meriggioli MN, Rowin J. Fatigue and abnormal neuromuscular transmission in Kennedy's disease. Muscle Nerve 2003;27:249-251.
11. Inoue K, Hemmi S, Miyaishi M, Kutoku Y, Murakami T, Kurokawa K, et al. Muscular fatigue and decremental response to repetitive nerve stimulation in X-linked spinobulbar muscular atrophy. Eur J Neurol 2009;16:76-80.
12. Oh SJ. Repetitive nerve stimulation test. In: Oh SJ. Principles of Clinical Electromyography: Case Studies. Baltimore: Williams & Wilkins, 1998;59-75.
13. AAEM Quality Assurance Committee. Literature review of the use-fulness of repetitive nerve stimulation and single fiber EMG in the electrodiagnostic evaluation of patients with suspected myasthenia gravis or Lambert-Eaton myasthenic syndrome. Muscle Nerve 2001; 24:1239-1247.
14. Doyu M, Sobue G, Kimata K, Yamamoto K, Mitsuma T. Androgen receptor mRNA with increased size of tandem CAG repeat is widely expressed in the neural and nonneural tissues of X-linked recessive bulbospinal neuronopathy. J Neurol Sci 1994;127:43-47.
15. Sorarù G, D'Ascenzo C, Polo A, Palmieri A, Baggio L, Vergani L, et al. Spinal and bulbar muscular atrophy: Skeletal muscle pathology in male patients and heterozygous females. J Neurol Sci 2008;264:100-105.
16. Monks DA, O'Bryant EL, Jordan CL. Androgen receptor immunore-activity in skeletal muscle: Enrichment at the neuromuscular junction. J Comp Neurol 2004;473:59-72.