Can Awaji ALS criteria provide earlier diagnosis than the revised El Escorial criteria?

Okita, Takahiro; Nodera, Hiroyuki; Shibuta, Yoshiko; Nodera, Atsuko; Asanuma, Kotaro; Shimatani, Yoshimitsu; Sato, Kengo; Izumi, Yuishin; Kaji, Ryuji

doi:10.1016/j.jns.2010.12.007

Cited by 61 publications

(31 citation statements)

References 15 publications

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“…15 However, it is often hard to determine whether fasciculations are present or not, especially in muscles in which complete relaxation is rarely achieved, such as the tongue. 15 However, it is often hard to determine whether fasciculations are present or not, especially in muscles in which complete relaxation is rarely achieved, such as the tongue.…”

Section: Discussionmentioning

confidence: 99%

Ultrasonographic detection of fasciculations markedly increases diagnostic sensitivity of ALS

Misawa¹,

Noto²,

Shibuya³

et al. 2011

Neurology

160

111

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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.

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Section: Discussionmentioning

confidence: 99%

Ultrasonographic detection of fasciculations markedly increases diagnostic sensitivity of ALS

Misawa¹,

Noto²,

Shibuya³

et al. 2011

Neurology

160

111

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“…Electrophysiological changes are one of the first measurable alterations to occur and form a large part of the tests used to diagnose ALS in humans; the (modified) El Escorial criteria (De Carvalho et al, 2008) alongside the Awaji criteria (Okita et al, 2011) provide electrophysiological data, and this, combined with the ALS functional-rating scale (Cedarbaum and Stambler, 1997) provides a more definitive diagnosis. Nerve conduction measurements such as Muscle Fiber Conduction Velocity (MFCV) can be 89% effective at predicting the development of ALS in patients who already show denervation in muscle biopsies but do not yet conform to the other ALS diagnostic tests mentioned above (Blijham et al, 2007).…”

Section: Early Clinical Manifestations Of Alsmentioning

confidence: 99%

ALS as a distal axonopathy: molecular mechanisms affecting neuromuscular junction stability in the presymptomatic stages of the disease

2014

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Amyotrophic Lateral Sclerosis (ALS) is being redefined as a distal axonopathy, in that many molecular changes influencing motor neuron degeneration occur at the neuromuscular junction (NMJ) at very early stages of the disease prior to symptom onset. A huge variety of genetic and environmental causes have been associated with ALS, and interestingly, although the cause of the disease can differ, both sporadic and familial forms of ALS show a remarkable similarity in terms of disease progression and clinical manifestation. The NMJ is a highly specialized synapse, allowing for controlled signaling between muscle and nerve necessary for skeletal muscle function. In this review we will evaluate the clinical, animal experimental and cellular/molecular evidence that supports the idea of ALS as a distal axonopathy. We will discuss the early molecular mechanisms that occur at the NMJ, which alter the functional abilities of the NMJ. Specifically, we focus on the role of axon guidance molecules on the stability of the cytoskeleton and how these molecules may directly influence the cells of the NMJ in a way that may initiate or facilitate the dismantling of the neuromuscular synapse in the presymptomatic stages of ALS.

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“…In addition, a careful and extensive EMG and nerve conduction study is essential in shortening the diagnostic delay. In particular, the recent Awaji criteria [7] enable early detection of electrophysiological abnormalities in apparently unaffected limbs and outside the region of clinical onset [29]. Slowly progressing patients have to be followed up carefully and when only lower motoneuron signs and symptoms are apparent, common ALS-mimic disorders (e.g., Kennedy disease and [multifocal] motor neuropathy) have to be ruled out [30].…”

Section: Independent Variablementioning

confidence: 99%

Factors affecting the diagnostic delay in amyotrophic lateral sclerosis

Cellura

Spataro

Taiello

et al. 2012

Clinical Neurology and Neurosurgery

108

101

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a b s t r a c tBackground: Although amyotrophic lateral sclerosis (ALS) is a relentlessly progressive disorder, early diagnosis allows a prompt start with the specific drug riluzole and an accurate palliative care planning. ALS at onset may however mimic several disorders, some of them treatable (e.g., multifocal motor neuropathy) or epidemiologically more frequent (e.g., cervical myelopathy). Objective: To study the delay from onset to diagnosis in a cohort of ALS patients and to the variables that may affect it. Methods: We performed a retrospective analysis of the diagnostic delays in a cohort of 260 patients affected by ALS (M/F = 1.32) followed at our tertiary referral ALS Center between 2000 and 2007.Results: The median time from onset to diagnosis was 11 months (range: 6-21) for the whole ALS cohort, 10 months (range: 6-15) in bulbar-onset (n = 65) and 12 months (range: 7-23) in spinal-onset (n = 195) patients (p = 0.3). 31.1% of patients received other diagnoses before ALS and this led to a significant delay of the correct diagnosis in this group (other diagnoses before ALS, n = 81: median delay, 15 months [9.75-24.25] vs ALS, n = 179, median delay, 9 months [6-15.25], p < 0.001). Conclusions: The diagnostic delay in ALS is about one year, besides the growing number of tertiary centres and the spread of information about the disease through media and internet. Cognitive errors based on an incorrect use of heuristics might represent an important contributing factor. Furthermore, the length of the differential diagnosis from other disorders and delays in referral to the neurologist seems to be positively associated with the delay in diagnosis.

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Can Awaji ALS criteria provide earlier diagnosis than the revised El Escorial criteria?

Cited by 61 publications

References 15 publications

Ultrasonographic detection of fasciculations markedly increases diagnostic sensitivity of ALS

Ultrasonographic detection of fasciculations markedly increases diagnostic sensitivity of ALS

ALS as a distal axonopathy: molecular mechanisms affecting neuromuscular junction stability in the presymptomatic stages of the disease

Factors affecting the diagnostic delay in amyotrophic lateral sclerosis

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