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Dement Neuropsychol
January 2016
MD, University of São Paulo Clinicas Hospital, Behavioral and Cognitive Neurology Unit, Department of Neurology and Cognitive Disorders Reference Centers (CEREDIC), São Paulo, Brazil.
The atypical form of Pantothenate Kinase-Associated Neurodegeneration (PKAN) tends to present at around the age of 14 years, has a heterogeneous presentation with extrapyramidal symptoms, and approximately one third of patients exhibit psychiatric problems. This paper reports the case of a patient with apparent typical symptoms of Tourette syndrome. However, the severity and poor response to treatment led to further investigation and the diagnosis of PKAN as a secondary cause of Tourettism was reached.
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