This review identified eleven studies that used a control group to evaluate the diagnostic accuracy of various signs. An additional 61 articles reported on the frequency of clinical signs in a cohort of DCM patients. Based on the results of the eleven studies, the most sensitive clinical tests for diagnosing DCM are the Tromner (94%) and hyperreflexia (79%), while the most specific tests are the Babinski (99%), Tromner (93%), clonus (99%) and inverted supinator sign (93%). Five studies evaluated the association between the presence of various clinical signs and disease severity. Results were inconsistent across studies; no definite relationship was identified between disease severity and Hoffmann, Babinski or hyperreflexia.