Lafora´s disease is a strange type of myoclonic disorder (there have been described no more than 200 cases in the literature), with genetic basis. Some mutations provoke a beginning in the adolescence and a quickly development. At present it doesn’t exist a treatment, none to the disease, but also to the different neurologic associated disorders (myoclonus, seizures, dementia) and fatal outcome. Along the evolution, it´s usual to find psychotic symptoms, especially auditory hallucinations. We present a case with atypical evolution: The subject presents auditory hallucinations, with a good response to psychopharmacological treatment. The presence of these symptoms could be observed in the first years of the diseases. We describe the status of the patient at the admission, the evolution during the stay, with the increase of symptoms in several areas (cognitive, motor, neurologic), and the current status, after hospitalization, where the patient begun receiving enteral nutrition by gastrostomie, due to the limitations to receive the treatment and /or nutrition by oral way. This case is also interesting due to the use of Metformine, an antidiabetic agent, whose use has been suggested as therapeutic alternative for this syndrome.

Lafora Disease, Myoclonus, Treatment, Dementia, Psychosis, Visual Hallucinations