The neuropsychological pattern of Unverricht–Lundborg disease

SummaryThis study determined the neuropsychological pattern of Unverricht–Lundborg disease (ULD) and its relationship to disease-related variables. Twenty-one ULD patients were evaluated using neuropsychological tests for abstract reasoning, attention, planning, set-shifting, theory of mind, visual perception, visuomotor coordination, ideomotor, orofacial and constructive praxis, language, learning, and memory. The control groups consisted of 21 healthy subjects and 21 patients with cryptogenic temporal lobe epilepsy (TLE). Multivariate analysis of variance showed that, in comparison with both control groups, the ULD patients showed significantly impaired abstract reasoning, attention, planning, word fluency, constructive praxis, and visuospatial memory and learning. Factor analysis of the test scores obtained by the ULD and TLE groups identified four factors (processing and execution, praxis, memory, theory of mind), and separate ANOVAs using epilepsy-related and demographic variables as covariates showed that the ULD patients had significantly impaired processing and execution. Correlation and regression analyses showed that processing and execution was significantly associated with diagnosis, disease duration and education, and logistic regression analyses indicated that it significantly predicted a diagnosis of ULD. To conclude, ULD is characterised by impaired processing and execution functions. This impairment is a strong independent predictor of ULD and may contribute to the characterisation of the diagnosis.