A descriptive study of the neuropsychological and psychopathological profile in patients with type 1 myotonic dystrophy

Type 1 myotonic dystrophy (MD-1) or Steinert disease is a multisystemic progressive disorder. Studies have shown cognitive deficits, depressive symptoms and a high incidence of anxiety personality traits that compromise both the functionality and the quality of life of these patients. To describe the cognitive and psychopathological profile of a sample of patients with the adult variant of MD-1. A sample of 27 patients diagnosed with MD-1 was selected from those being followed up in the neurology service of the Complejo Hospitalario de Navarra. Eligibility criteria were age under 50 years and the absence of any other pathology or physical condition that prevented them from performing the psychological evaluation. A neuropsychological evaluation battery specifically designed for this kind of pathology was used, together with psychopathological and functionality measures. The neuropsychological evaluation revealed mainly deficits in visual-constructional and visuospatial skills, alternating attention and in other-reported dysexecutive signs and symptoms. The group of patients did not present any clinically significant symptoms of depression or anxiety, but did score high on obsession-compulsion, interpersonal sensitivity, paranoid ideation and psychoticism. The results point towards a deterioration in functionality. In the integral approach to MD-1, the characterisation and developmental monitoring of the cognitive, psychopathological and personality profile, as well as the level of functionality, all contribute to an improvement in the quality of life of these patients.