Diagnosis of equine herpesvirus-1 associated myeloencephalopathy (EHM) can be troublesome, but early recognition and knowledge of risk factors are essential for prevention and control. The objectives for this study are to (1) describe EHM in France, (2) improve clinical recognition, (3) identify risk factors. Through epidemiosurveillance of acute neurological cases (all considered to be potentially infectious cases) in France (2008-2011), 26 EHM cases were identified and 29 EHM negative control cases. EHM cases were described and compared to controls with univariate, multivariate and classification and regression tree analysis. EHM cases had a 46% fatality rate and were frequently isolated cases. Most showed ataxia, paresis and a cauda equina syndrome, yet presence of other neurological signs was variable. Statistical analysis identified the following variables to be significantly associated to EHM compared to controls: introduction of a new horse to the herd, cauda equina syndrome, larger herd size, saddle horses and month of occurrence. The presence of many isolated cases, and less typical and variable clinical presentations emphasize the difficulty in diagnosing EHM. Nevertheless, history and clinical examination of acute neurological cases can be valuable in recognizing EHM early as well in order to select those cases that need further laboratory testing and infection control measures. Moreover, with a different study format and geographic location, risk factors were found to be similar to previous studies, therefore strengthening their significance to the spread of EHM.