Neuropsychiatric symptoms in idiopathic brain calcification

Abstract

INTRODUCTION: Idiopathic basal ganglia calcification (IBGC) is a rare neurological disease characterized by the deposition of calcium in the brain, without calcium metabolism abnormalities. IBGC may be asymptomatic or clinically evident, causing predominantly movement disorders (parkinsonism, chorea, tremor, dystonia, dyskinesia) and a wide range of neuropsychiatric symptoms (dementia, psychosis, mood disorders, anxiety), which are currently poorly defined in literature. The aim of this study is to deepen the knowledge on psychiatric disorders associated with IBGC, providing an accurate description of eleven cases and summarizing information from the review of literature.EVIDENCE ACQUISITION: From our clinical database, we selected patients meeting the diagnostic criteria for IBGC and collected demographic, clinical, genetic, and neuroradiological data. Psychiatric assessment was performed using the 18-item Brief Psychiatric Rating Scale, the Positive and Negative Syndrome Scale, the Hamilton Anxiety Rating Scale and the Hamilton Depression Rating Scale. We also searched the PubMed database for papers dealing with psychiatric features in IBGC, and the related treatments.EVIDENCE SYNTHESIS: Eight of the eleven patients included in the study reported at least one psychiatric symptom during the course of the disease. The assessment through the HDRS21 and HAM-A Scale confirmed the presence of mild depression and anxiety as the main psychiatric disturbs associated with IBGC. Mean total PANSS and BPRS scores were 40.3±12.4 and 27.6±8.6 respectively, with a major involvement in specific items such as depressive mood, anxiety, tension, motor retardation and disorientation. Data from literature confirmed a significant psychiatric involvement in IBGC. Available case reports mainly concern atypical presentations of IBGC with abrupt onset psychotic symptoms. An in-depth analysis of psychiatric features associated with IBGC is lacking.CONCLUSIONS: Neuropsychiatric involvement is frequent in IBGC. A neuropsychiatric evaluation is highly recommended in patients with evidence of brain calcifications. Conversely, IBGC should be considered in patients presenting with psychiatric symptoms, especially if movement disorders and neurocognitive impairment are co-existing features.