Hyperekplexia (HE) is a benign disease characterized by increased startle response to auditory, tactile and visual stimuli. We report two girls with HE. The patients, at 52 and 21 days of age, were referred to our department with the complaint of seizure onset at the first day of life. For the second case, it was stated that seizure had been refractory to treatment and had no evident etiology. Their medical histories were unremarkable. Physical examination revealed that tactile stimuli yielded generalized myoclonus, which sometimes was persistent as tonic spasms. HE was entertained in the view of clinical and laboratory findings. The symptoms almost fully resolved after clonazepam, and the infants' development was normal in the follow-up. These cases emphasize that nonepileptic events should be considered in the differential diagnosis of convulsions. Performing tactile stimuli would be helpful to elucidate HE in patients with spasms that may be considered as convulsion at first sight.

Keywords: Hiperekpleksia, psödoepilepsi, hyperekplexia, pseudoepilepsy