Apply Awaji-shima Consensus Conference Criteria Before Diagnosing Amyotrophic Lateral Sclerosis

Josef Finsterer*, 1, Claudia Stöllberger2
1 Danube University Krems, Krems, Krankenanstalt Rudolfstiftung, Vienna, Austria
2 Medical Department, KrankenanstaltRudolfstiftung, Vienna, Austria

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© Finsterer and Stöllberger; Licensee Bentham Open.

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

* Address correspondence to this author at the Postfach 20 1180 Vienna, Austria, Europe; Tel: +43-1-71165-92085; Fax: +43-1-4781711; E-mail:



ALS may be diagnosed although affection of other organs suggests another pathogenetic back-ground.

Case report:

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 Though the diagnosis of ALS was withdrawn, he was put on comfort care and opiates were given when dyspnea acutely deteriorated to death without further cardiac or pulmonary investigations or specific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.

Keywords: Motor neuron disease, anterior horn cell disease, amyotrophic lateral sclerosis, mimicry, polyneuropathy, cardiomyopathy, cardiac involvement.