Article

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: 3.4). 12/2008; 10(1):53-7. DOI: 10.1080/17482960802521126
Source: PubMed

ABSTRACT 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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    ABSTRACT: Background and purposeThe diagnosis of amyotrophic lateral sclerosis (ALS) relies on identification of a combination of upper and lower motor neuron signs. In order to improve the diagnostic sensitivity for ALS, Awaji criteria were developed, in part to better incorporate neurophysiological measures, although assessment of upper motor neuron dysfunction remained clinically based. Given that cortical hyperexcitability appears to be an early feature in ALS, the present study assessed the diagnostic utility of a threshold tracking transcranial magnetic stimulation technique as an aid to the research-based Awaji criteria in establishing an earlier diagnosis of ALS.Methods Prospective studies were undertaken on a cohort of 82 patients with suspected ALS and results were compared with 34 healthy controls.ResultsShort-interval intracortical inhibition (SICI) was significantly reduced in ALS patients (P < 0.0001), with a comparable reduction evident in the Awaji groups (SICIAWAJI POSSIBLE 1.3% ± 1.3%; SICIAWAJI PROBABLE/DEFINITE 1.4% ±1.7%). Central motor conduction time was significantly prolonged (P < 0.001), whereas the motor evoked potential amplitude (P < 0.05) and intracortical facilitation (P < 0.05) were increased. The frequency of transcranial magnetic stimulation abnormalities was similar across Awaji subgroups, and addition of transcranial magnetic stimulation abnormalities as a diagnostic category enabled reclassification of 88% of Awaji possible patients to Awaji probable/definite.Conclusions Cortical excitability studies potentially facilitate an earlier diagnosis of ALS when combined with clinical and conventional neurophysiological findings, albeit in a research setting.
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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.
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    ABSTRACT: Amyotrophic lateral sclerosis is a neurodegenerative disease with a survival rate up to 5 years of 20%. It is characterized by the damage of the first and second motor neurons in a progressive course. There is no cure for this disease at the moment. Nevertheless, new treatments are being developed with the haunting shadow of not few drugs that were proven to be ineffective in stopping or slowing the disease progression. As a result, the main treatment objective at the moment is to soothe the complications the disease inexorably generates in its course. In this review we describe the signs and symptoms of the disease progression and its therapeutic options, for which we have reviewed national and international publications, as well as shearing the experience in our centre. The main topics developed are: weakness, sialorrhea, bronchial secretions, pseudobulbar affect, cramps, spasticity, respiratory insufficiency, limb edema, depression and dysphagia. We would like to point out that in spite of the general belief that «there is nothing left to do» we are summarizing a few, and only a few, of the therapeutic tools available for aiding the patients affected with ALS.
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