Awaji Criteria for the Diagnosis of Amyotrophic Lateral Sclerosis

Abstract

To estimate the potential diagnostic added value of the Awaji criteria for diagnosis of amyotrophic lateral sclerosis (ALS), which have been compared with the previously accepted gold standard the revised El Escorial criteria in several studies. Data Sources: MEDLINE and Web of Science (until October 2011). Study Selection: We searched for studies testing the diagnostic accuracy of the Awaji criteria vs the revised El Escorial criteria in patients referred with suspected ALS. Data Extraction: Evaluation and data extraction of identified studies were done independently. The Quality Assessment of Diagnostic Accuracy Studies list was used to assess study quality. We determined the proportion of patients classified as having probable/ definite ALS and derived indices of diagnostic performance (sensitivity, specificity, and diagnostic odds ratio). Quantitative data synthesis was accomplished through random-effects meta-analysis, and heterogeneity was assessed with the I 2 test. Data Synthesis: Eight studies were included (3 prospective and 5 retrospective) enrolling 1187 patients. Application of Awaji criteria led to a 23% (95% CI, 12% to 33%; I 2 =84%) increase in the proportion of patients classified as having probable/definite ALS. Diagnostic performance of the Awaji criteria was higher than the revised El Escorial criteria (pooled sensitivity: 81.1% [95% CI, 72.2% to 90.0%; I 2 =91%] vs 62.2% [95% CI, 49.4% to 75.1%; I 2 = 93%]; pooled diagnostic odds ratio, 35.8 [95% CI, 15.2 to 84.7; I 2 =3%] vs 8.7 [95% CI, 2.2 to 35.6; I 2 =50%]). Diagnostic accuracy of Awaji criteria was higher in bulbar-than in limb-onset cases. Conclusion: The Awaji criteria have a significant clinical impact allowing earlier diagnosis and clinical trial entry in ALS.

The role of cranial and thoracic electromyography within diagnostic criteria for amyotrophic lateral sclerosis

“…PLS and PMA patients represented 2% and 6% of our MND patient population, respectively, which is similar to previous reports . The percentage of patients with “probable/definite” disease and the improvements in diagnostic sensitivity achieved by adding EMG to clinical data are comparable with previous studies . Most of the increase in diagnostic confidence by the Awaji‐Shima classification was derived from EMG examination of the limbs, perhaps because limb‐onset ALS is the most common form of disease presentation, or because clinical detection of UMN‐only pathology together with EMG‐only detection of LMN pathology occurs most frequently in the limbs (Fig.…”

The role of cranial and thoracic electromyography within diagnostic criteria for amyotrophic lateral sclerosis

“…PLS and PMA patients represented 2% and 6% of our MND patient population, respectively, which is similar to previous reports . The percentage of patients with “probable/definite” disease and the improvements in diagnostic sensitivity achieved by adding EMG to clinical data are comparable with previous studies . Most of the increase in diagnostic confidence by the Awaji‐Shima classification was derived from EMG examination of the limbs, perhaps because limb‐onset ALS is the most common form of disease presentation, or because clinical detection of UMN‐only pathology together with EMG‐only detection of LMN pathology occurs most frequently in the limbs (Fig.…”

Motor Neuron Disease in a Patient With Cervical Spondylotic Myelopathy: Too Much Bad Luck

“…In the present case, the patient predominantly showed clinical and EMG findings of LMN syndrome, suggestive of progressive muscular atrophy, considered to be a rare presentation of MND [4]. The patient met both the clinical criteria as well as the Awaji EMG criteria for MND [5].…”

Severe cervical compressive polydiscopathic myelopathy with features of motor neuron disease: A case report

“…14,15 Moreover, the Awaji criteria for the diagnosis of ALS have proved to be of a significant importance in earlier diagnosis and clinical trial entry for this disease, in comparison with the previously accepted gold standard-the revised El Escorial criteria. 16 In our case, the neuroimaging suggests cervical myelopathy as a possible cause for the clinical presentation. The presence of sensory symptoms and signs, lower motor neuron signs at the level of compression, upper motor neuron signs in lower limbs, sphincter dysfunction, the aspect of the cervical spinal cord on MRI, the electrodiagnostic studies, and absence of cases of neurodegenerative disorders in family support our diagnosis and exclude ALS.…”

The role of cranial and thoracic electromyography within diagnostic criteria for amyotrophic lateral sclerosis

“…PLS and PMA patients represented 2% and 6% of our MND patient population, respectively, which is similar to previous reports . The percentage of patients with “probable/definite” disease and the improvements in diagnostic sensitivity achieved by adding EMG to clinical data are comparable with previous studies . Most of the increase in diagnostic confidence by the Awaji‐Shima classification was derived from EMG examination of the limbs, perhaps because limb‐onset ALS is the most common form of disease presentation, or because clinical detection of UMN‐only pathology together with EMG‐only detection of LMN pathology occurs most frequently in the limbs (Fig.…”

Motor Neuron Disease in a Patient With Cervical Spondylotic Myelopathy: Too Much Bad Luck

“…In the present case, the patient predominantly showed clinical and EMG findings of LMN syndrome, suggestive of progressive muscular atrophy, considered to be a rare presentation of MND [4]. The patient met both the clinical criteria as well as the Awaji EMG criteria for MND [5].…”

Severe cervical compressive polydiscopathic myelopathy with features of motor neuron disease: A case report

“…14,15 Moreover, the Awaji criteria for the diagnosis of ALS have proved to be of a significant importance in earlier diagnosis and clinical trial entry for this disease, in comparison with the previously accepted gold standard-the revised El Escorial criteria. 16 In our case, the neuroimaging suggests cervical myelopathy as a possible cause for the clinical presentation. The presence of sensory symptoms and signs, lower motor neuron signs at the level of compression, upper motor neuron signs in lower limbs, sphincter dysfunction, the aspect of the cervical spinal cord on MRI, the electrodiagnostic studies, and absence of cases of neurodegenerative disorders in family support our diagnosis and exclude ALS.…”

The role of cranial and thoracic electromyography within diagnostic criteria for amyotrophic lateral sclerosis

“…PLS and PMA patients represented 2% and 6% of our MND patient population, respectively, which is similar to previous reports . The percentage of patients with “probable/definite” disease and the improvements in diagnostic sensitivity achieved by adding EMG to clinical data are comparable with previous studies . Most of the increase in diagnostic confidence by the Awaji‐Shima classification was derived from EMG examination of the limbs, perhaps because limb‐onset ALS is the most common form of disease presentation, or because clinical detection of UMN‐only pathology together with EMG‐only detection of LMN pathology occurs most frequently in the limbs (Fig.…”

Motor Neuron Disease in a Patient With Cervical Spondylotic Myelopathy: Too Much Bad Luck

“…In the present case, the patient predominantly showed clinical and EMG findings of LMN syndrome, suggestive of progressive muscular atrophy, considered to be a rare presentation of MND [4]. The patient met both the clinical criteria as well as the Awaji EMG criteria for MND [5].…”

Severe cervical compressive polydiscopathic myelopathy with features of motor neuron disease: A case report

“…14,15 Moreover, the Awaji criteria for the diagnosis of ALS have proved to be of a significant importance in earlier diagnosis and clinical trial entry for this disease, in comparison with the previously accepted gold standard-the revised El Escorial criteria. 16 In our case, the neuroimaging suggests cervical myelopathy as a possible cause for the clinical presentation. The presence of sensory symptoms and signs, lower motor neuron signs at the level of compression, upper motor neuron signs in lower limbs, sphincter dysfunction, the aspect of the cervical spinal cord on MRI, the electrodiagnostic studies, and absence of cases of neurodegenerative disorders in family support our diagnosis and exclude ALS.…”