Autosomal-recessive early-onset Parkinsonism (AREP) due to PINK1 mutations is characterized by an early-onset, slowly progressive disease, with a good response to levodopa. Psychiatric and cognitive disturbances associated with AREP have rarely been reported in the literature. We describe 2 brothers from a Jewish-Iraqi consanguineous family with a homozygous PINK1 nonsense mutation. Both patients presented with anxiety and dysphoria accompanied by a gait disturbance that developed subsequently into a clinical depression. During the course of the disease, both developed drug-induced behavioral disturbances of the hedonistic homeostatic dysregulation type and 1 had drug-induced psychosis. The first patient had been diagnosed with mild mental retardation and during the 22 years of disease had further deteriorated; the second developed frontal-type dementia at an early age, 20 years after onset. Their father had a psychiatric disorder but no Parkinsonism. This report expands the phenotypic profile of PINK1-related disease, presenting unique psychiatric and cognitive features as part of the clinical picture.

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In order to evaluate the severity of behavioral complications after deep brain stimulation of the subthalamic nucleus (STN-DBS) for Parkinson's disease and to explore possible predictive factors, the authors evaluated 22 patients for pre- and postoperative symptoms using a neurobehavioral battery. Compared to the time before STN-DBS, several behavioral symptoms had worsened in terms of prevalence and severity and appeared de novo in other patients. Apathy, anxiety, and suicidal ideation increased significantly, while depressive symptoms appeared stable. Compared with patients who improved, patients who had deteriorated behaviorally had similar prevalence and severity of preoperative behavioral symptoms but significantly shorter disease duration.

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