Awaji diagnostic algorithm increases sensitivity of El Escorial criteria for ALS diagnosis

Hospital of Santa Maria Hospital and Instituto de Medicina Molecular-Molecular Biology, University of Lisbon, Portugal.
Amyotrophic Lateral Sclerosis (Impact Factor: 2.37). 12/2008; 10(1):53-7. DOI: 10.1080/17482960802521126
Source: PubMed


We have tested the sensitivity of a recently published approach to combining clinical and EMG data in the 'research diagnosis' of ALS, in 55 consecutive patients clinically diagnosed with ALS. The application of this 'Awaji algorithm' to the revised El Escorial diagnostic criteria for diagnosis of ALS achieved a diagnostic sensitivity of 95% for definite ALS compared with 18% using the clinical El Escorial criteria and 53% when the EMG criteria as defined in the El Escorial criteria, were applied to the same dataset. This increased sensitivity was particularly relevant for bulbar onset patients (sensitivity improved from 38% to 87%) and for patients with El Escorial clinically possible ALS (from 50% to 86%). We suggest that, in future, investigators and triallists should use the Awaji algorithm superimposed onto the El Escorial criteria, in selecting patients for research studies.

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    • "Fasciculations are similar to PSWs and fibrillations if they occur in muscles showing neurogenic changes (De Carvalho et al., 2008). This modification increases the sensitivity of diagnosis without increasing the false-positive rate (de Carvalho and Swash, 2009; Boekestein et al., 2010). A meta-analysis showed that the use of the Awaji criteria increased the number of patients diagnosed with probable or definitive ALS by up to 23%, without a decrease in specificity, compared with that diagnosed using the revised El- Escorial criteria (Costa et al., 2012) that emphasizes on the importance of refined electrodiagnostic studies. "
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    ABSTRACT: Objective We aimed to determine the utility of muscle ultrasonography (MUS) in addition to electromyography (EMG) in the diagnosis of amyotrophic lateral sclerosis (ALS). Methods In all, 60 patients with ALS and 20 with other neuromuscular disorders underwent MUS and EMG. In addition, 30 healthy controls underwent only MUS. Occurrence of fasciculations and fibrillations was evaluated. Ultrasonic echogenicity was graded semiquantitatively. Results The incidence of fasciculations was significantly higher in patients undergoing MUS than in those undergoing EMG (p < 0.05), even in muscles of full strength (p < 0.001). However, EMG was more sensitive in detecting fibrillations (p < 0.05). MUS had an overall higher sensitivity in detecting spontaneous activity in the tongue (p < 0.05). Patients with ALS showed significantly increased muscle echo intensity (EI) compared to patients who were initially suspected as having ALS and normal controls (p < 0.05), irrespective of the clinical or electrophysiological status. Conclusion Our results showed that the sensitivity and specificity of MUS in diagnosing ALS was almost equivalent to those of EMG, using the Awaji criteria. Combination of MUS and EMG enhances the diagnostic accuracy compared to EMG alone (p < 0.05). Significance The combination of EMG and MUS can be used to evaluate the lower motor neuron affection by reducing the use of the often painful and uncomfortable EMG examinations but without decreasing the diagnostic sensitivity and specificity.
    Full-text · Article · Aug 2014 · Clinical Neurophysiology
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    • "Table 1. Awaji-shima Consensus Recommendations for the Application of Electrophysiological Tests to Diagnose ALS, as Applied to the Revised El Escorial Criteria (Airlie House 1998) [1] "
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    ABSTRACT: ALS may be diagnosed although affection of other organs suggests another pathogenetic back-ground. In a 72yo non-smoking male progressive gait disturbance with recurrent falls since 2y was initially attributed to axonal polyneuropathy. Additionally, he had arterial hypertension, diabetes, hyperlipidemia, hyperuricemia, hyper-CK-emia, hepatopathy, atrial fibrillation, recurrent heart-failure, pulmonary hypertension, mitral insufficiency, and restrictive cardiomyopathy. Possible causes of polyneuropathy were diabetes, long-standing alcoholism, folate-deficiency, or hereditary disease. Later the patient was re-diagnosed as ALS despite absence of upper motor-neuron or bulbar signs, the presence of multiple risk factors for polyneuropathy, of stocking-type sensory disturbances, and of cardiac abnormalities, which could explain dyspnea. Misdiagnosing polyneuropathy as ALS stigmatized the patient and prevented him from further diagnostic work-up for cardiac disease and adequate treatment for heart-failure. Though the diagnosis of ALS was withdrawn, he was put on comfort care and opiates were given when dyspnea acutely deteriorated to death without further cardiac or pulmonary investigations or specific cardiac treatment. ALS should be diagnosed only if the Awaji-shima criteria are fulfilled and if all differential diagnoses were profoundly excluded. Respiratory insufficiency should not be attributed to bulbar involvement in ALS as long as cardiac, pulmonary, or myopathic causes were excluded.
    Full-text · Article · Jan 2013 · The Open Neurology Journal
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    • "According to the El Escorial Criteria [2] a ‘clinically probable ALS’ was suggested (evidence of upper motor neuron involvement was demonstrated in two regions). "
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    ABSTRACT: Introduction Amyotrophic lateral sclerosis is a progressive neurodegenerative disorder characterized by degeneration of motoneuron cells in anterior spinal horns. There is a need for early and accurate diagnosis with this condition. In this case report we used two complementary methods: scanning electron microscopy and fluorescence-activated cell sorting. This is the first report to our knowledge of microparticles in the cerebrospinal fluid of a patient with amyotrophic lateral sclerosis. Case presentation An 80-year-old Swedish man of Caucasian ethnicity presented to our facility with symptoms of amyotrophic lateral sclerosis starting a year before his first hospital examination, such as muscle weakness and twitching in his right hand progressing to arms, body and leg muscles. Electromyography showed classical neurophysiological findings of amyotrophic lateral sclerosis. Routine blood sample results were normal. A lumbar puncture was performed as a routine investigation and his cerebrospinal fluid was normal with regard to cell count and protein levels, and there were no signs of inflammation. However, scanning electron microscopy and fluorescence-activated cell sorting showed pronounced abnormalities compared to healthy controls. Flow cytometry analysis of two fractions of cerebrospinal fluid from our patient with amyotrophic lateral sclerosis was used to measure the specific binding of antibodies to CD42a, CD144 and CD45, and of phosphatidylserine to lactadherin. Our patient displayed over 100 times more phosphatidylserine-positive microparticles and over 400 times more cell-derived microparticles of leukocyte origin in his cerebrospinal fluid compared to healthy control subjects. The first cerebrospinal fluid fraction contained about 50% more microparticles than the second fraction. The scanning electron microscopy filters used with cerebrospinal fluid from our patient were filled with compact aggregates of spherical particles of lipid appearance, sticking together in a viscous batter. The quantitative increase in scanning electron microscopy findings corresponded to the flow cytometry result of an increase in leukocyte-derived microparticles. Conclusions Microparticles represent subcellular arrangements that can influence the pathogenesis of amyotrophic lateral sclerosis and may serve as biomarkers for underlying cellular disturbances. The increased number of leukocyte-derived microparticles with normal cell counts in cerebrospinal fluid may contribute to the amyotrophic lateral sclerosis inflammatory process by formation of immune complexes of prion-like propagation, possibly due to misfolded proteins. The two complementary methods used in this report may be additional tools for revealing the etiology of amyotrophic lateral sclerosis, for early diagnostic purposes and for evaluation of clinical trials, long-term follow-up studies and elucidating the pathophysiology in amyotrophic lateral sclerosis.
    Full-text · Article · Sep 2012 · Journal of Medical Case Reports
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