A Case of Late Diagnosed Hiperekplexia

Nonepileptic events are common in children, and may be difficult to distinguish from epileptic events. Hyperekplexia is a rare nonepileptic event characterized by an exaggerated persistent startle reaction to unexpected auditory, somatosensory and visual stimuli, generalized muscular rigidity, and nocturnal myoclonus. In most instances, the clinical history and an age-based approach leads to the diagnosis, and ancillary testing serves as confirmation. A 10-year-old girl was referred to our department with the complaint of seizure and cranial trauma. Her physical examination revealed that tactile stimuli yielded myoclonic jerks. HE was entertained in view of the clinical and laboratory findings. The symptoms almost fully resolved after clonazepam in the follow-up. This case emphasizes that nonepileptic events should be considered in the differential diagnosis of convulsions.