Ultrasonographic detection of fasciculations markedly increases diagnostic sensitivity of ALS

Objectives: To study the utility of muscle ultrasound (US) for detection of fasciculations and its contribution to diagnosis in amyotrophic lateral sclerosis (ALS). Fasciculations are characteristic features of ALS, and US can detect them easily and reliably. New diagnostic criteria for ALS, the Awaji algorithm, reintroduced fasciculations as evidence of acute denervation equivalent to that of fibrillations and positive sharp waves. Methods: In 81 consecutive patients with sporadic ALS, we prospectively performed needle EMG and US in 6 muscles (tongue, biceps brachii, first dorsalis interosseous, paraspinalis, vastus lateralis, and tibialis anterior), and diagnostic category were determined by revised El Escorial criteria and Awaji criteria. Results: Fasciculations were much more frequently detected by US than by EMG in the tongue (60% vs 0%), biceps brachii (88% vs 60%), and tibialis anterior muscles (83% vs 45%). The proportion of the patients with definite or probable ALS was 48% by revised El Escorial criteria and 79% by Awaji criteria using US. Conclusions: Muscle US is a practical and efficient tool to detect fasciculations, particularly in the tongue. A combination of US and EMG substantially increases the diagnostic sensitivity of ALS.

[1]  J. Abbs,et al.  Hypoglossal, trigeminal, and facial motoneuron involvement in amyotrophic lateral sclerosis , 1988, Neurology.

[2]  Machiel J Zwarts,et al.  Muscle ultrasound in neuromuscular disorders , 2008, Muscle & nerve.

[3]  Kazuaki Kanai,et al.  Altered axonal excitability properties in amyotrophic lateral sclerosis: impaired potassium channel function related to disease stage. , 2006, Brain : a journal of neurology.

[4]  Nens van Alfen,et al.  Muscles alive: Ultrasound detects fibrillations , 2009, Clinical Neurophysiology.

[5]  Toshio Shimizu,et al.  Utility of trapezius EMG for diagnosis of amyotrophic lateral sclerosis , 2009, Muscle & nerve.

[6]  O. Hardiman,et al.  Clinical features of amyotrophic lateral sclerosis according to the El Escorial and Airlie House diagnostic criteria: A population-based study. , 2000, Archives of neurology.

[7]  P. Shaw,et al.  An evaluation of neurophysiological criteria used in the diagnosis of motor neuron disease , 2010, Journal of Neurology, Neurosurgery & Psychiatry.

[8]  M. Swash,et al.  Awaji diagnostic algorithm increases sensitivity of El Escorial criteria for ALS diagnosis , 2009, Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases.

[9]  H. Tumani,et al.  Quantitative ultrasonography in focal neuropathies as compared to clinical and EMG findings. , 1999, European journal of ultrasound : official journal of the European Federation of Societies for Ultrasound in Medicine and Biology.

[10]  Mulder Dw Clinical limits of amyotrophic lateral sclerosis. , 1982 .

[11]  F. Walker,et al.  Sonographic imaging of muscle contraction and fasciculations: A correlation with electromyography , 1990, Muscle & nerve.

[12]  M. de Carvalho,et al.  Fibrillation and sharp-waves: do we need them to diagnose ALS? , 1999, Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases.

[13]  K. Mills,et al.  Characteristics of fasciculations in amyotrophic lateral sclerosis and the benign fasciculation syndrome. , 2010, Brain : a journal of neurology.

[14]  U. Ziemann,et al.  Fasciculations: Clinical, electromyographic, and ultrasonographic assessment , 1996, Journal of Neurology.

[15]  M. Benatar,et al.  The electromyographic diagnosis of amyotrophic lateral sclerosis: Does the evidence support the El Escorial criteria? , 2007, Muscle & nerve.

[16]  M. Swash,et al.  El Escorial revisited: Revised criteria for the diagnosis of amyotrophic lateral sclerosis , 2000, Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases.

[17]  Needle electromyography of bulbar muscles in patients with amyotrophic lateral sclerosis , 1998, Neurology.

[18]  J. Takeda,et al.  Sonographic Evaluation of the Peripheral Nerve in Diabetic Patients , 2010, Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine.

[19]  K. Asanuma,et al.  Can Awaji ALS criteria provide earlier diagnosis than the revised El Escorial criteria? , 2011, Journal of the Neurological Sciences.

[20]  S. Pillen,et al.  Quantitative skeletal muscle ultrasound: Diagnostic value in childhood neuromuscular disease , 2007, Neuromuscular Disorders.

[21]  I. Bone,et al.  DIFFERENTIAL DIAGNOSIS OF MOTONEURONE DISEASE FROM OTHER NEUROLOGICAL CONDITIONS , 1986, The Lancet.

[22]  V. Dubowitz,et al.  Real‐time ultrasound imaging of muscles , 1988, Muscle & nerve.

[23]  Jun Kimura,et al.  Electrodiagnosis in Diseases of Nerve and Muscle , 2013 .

[24]  D. Denny-Brown,et al.  FIBRILLATION AND FASCICULATION IN VOLUNTARY MUSCLE , 1938 .

[25]  A. Boon,et al.  Qualifications for practitioners of neuromuscular ultrasound: Position statement of the American association of neuromuscular and electrodiagnostic medicine , 2010, Muscle & nerve.

[26]  H Bostock,et al.  Axonal ion channel dysfunction in amyotrophic lateral sclerosis. , 1995, Brain : a journal of neurology.

[27]  D. Price,et al.  Motor Neuron Diseases , 2012 .

[28]  M. Toepfer,et al.  Ultrasonographic Assessment of the Prevalence of Fasciculations in Lesions of the Peripheral Nervous System , 1997, Journal of neuroimaging : official journal of the American Society of Neuroimaging.

[29]  M. Zwarts,et al.  Prevalence and distribution of fasciculations in healthy adults: Effect of age, caffeine consumption and exercise , 2010, Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases.

[30]  K. Mills,et al.  Detecting fasciculations in amyotrophic lateral sclerosis: duration of observation required , 2010, Journal of Neurology, Neurosurgery & Psychiatry.

[31]  Alexander Scheel,et al.  Surface EMG and Myosonography in the Detection of Fasciculations: A Comparative Study , 1998, Journal of neuroimaging : official journal of the American Society of Neuroimaging.

[32]  S. Pillen,et al.  Quantitative muscle ultrasonography in amyotrophic lateral sclerosis. , 2008, Ultrasound in medicine & biology.

[33]  M. Swash,et al.  Electrodiagnostic criteria for diagnosis of ALS , 2008, Clinical Neurophysiology.

[34]  B. Brooks,et al.  El escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis , 1994, Journal of the Neurological Sciences.