Assessment and Determinants of Neuropsychiatric Presentation of Strio-Pallido-Dentate Calcification (Fahr’s disease/syndrome)

Abstract

Background: Strio-Pallido-Dentate calcification can be idiopathic (Fahr's disease) or secondary (Fahr's syndrome). Psychiatric disturbances were the major presenting symptoms.
Objectives: determination and assessment of psychiatric presenting symptoms of Fahr's disease/syndrome, and sociodemographic and clinical variables, and study the statistical significant of each variable.
Methods: patients attending psychiatric unit, during period of study, with Fahr's disease/syndrome were assessed by consultant psychiatrist. Sociodemographic and clinical variables were assessed. Structured clinical interview and MMSE was done. Data analysis and statistical significance and was done.
Results: total 53 patients; Idiopathic basal ganglia calcification (Fahr's disease) (FD) 75.5%, secondary calcification (Fahr's syndrome) 24.5%. The age range 35–78 years, mean 56.4±13 years, 64% married, 88% of higher education. Psychiatric presenting symptoms were; schizophrenia 32.1%, depression 18.9%, mania17.0%, bipolar disorder 17.0% and dementia 15.1%. Negative investigation was 3%, cognitive impairment 17%, seizures11.3%, movement disorders.5%, vertigo 26.4%, headache 9.4%, unsteadiness and difficulty of swallowing 56.6%. Psychiatric morbidity was significantly associated with; cognitive impairment (P<0.001), movement disorders (P=0.034), unsteadiness (P=0.019), difficulty of swallowing (P=0.019).
Conclusion: Fahr’s disease/syndrome diagnosis could be challenging, due to discrepancy between clinical presentations and radio-imaging findings. Fahr’s disease/syndrome should be kept in mind in any patients with late onset neuropsychiatric disorders.