Case Report 10.4172/Neuropsychiatry.1000207 © 2017 p- ISSN 1758-2008 e- ISSN 1758-2016 Neuropsychiatry (London) (2017) 7(3), 254–257 254 1 New York Medical College, 40 Sunshine Cottage Road, Valhalla, New York 10595, USA 2 Westchester Medical Center Behavioral Health Center, 100 Woods Road, Valhalla, New York, 10595, USA † Author for correspondence: Ian Kane, BS, Medical Student, New York Medical College, 40 Sunshine Cottage Road, Valhalla, NY 10595, USA. Phone: 609-513-7466, email: Ian_kane@nymc.edu Acute psychosis with manic features in patient with Fahr’s syndrome: A Case report and Clinical review Ian Kane 1,2,† , Michael Light 1,2 , Maria Khan 2 , Ifeoluwa Osewa 2 , Mitchell Nobler 2 , Nabil Siddiqi 2 ABSTRACT Fahr’s syndrome, or Idiopathic Basal Ganglia Calcifcation syndrome, is a rare neurologic condition with a prevalence of <1/1,000,000 individuals. Calcifcations can be seen primarily in the basal ganglia, more specifcally in the internal globus pallidus, as well as other regions such as the caudate, putamen, dentate nuclei, and thalamus. Afected individuals can have an unsteady gait, slow or slurred speech, clumsiness, involuntary muscle movements, muscle cramps, and seizures. Neuropsychiatric manifestations are also common presenting symptoms and can range in severity from mild memory impairments to dementia, psychosis, and personality or behavior changes. The diagnosis can be made once several criteria are met: bilateral basal ganglia calcifcations, progressive neurologic dysfunction, family history of basal ganglia calcifcations with an autosomal dominant pattern of inheritance, and the absence of another condition that could explain the calcifcations such as metabolic, mitochondrial, infectious, toxic, or traumatic etiologies. There are a handful of case reports in the literature concerning patients with Fahr’s Syndrome that experienced their frst psychotic break at an advanced age. The case presented in this paper outlines the successful management of a patient with Fahr’s Syndrome, and the corresponding literature review serves to highlight an important but little known condition that should be considered in the diferential diagnosis for patients presenting with a frst psychotic break at an atypical age. Keywords Fahr’s syndrome, Neuropsychiatry, Genetic, Degenerative, Psychosis, Mania Introduction Fahr’s syndrome, or Idiopathic Basal Ganglia Calcifcation syndrome, is a rare neurologic condition with a prevalence of <1/1,000,000 individuals [1-3]. It is most commonly inherited in an autosomal dominant fashion, but can also occur sporadically. Several possible chromosomal loci have been proposed as the source of the defect [4]. Calcifcations can be seen primarily in the basal ganglia, more specifcally in the internal globus pallidus, as well as other regions such as the caudate, putamen, dentate nuclei, and thalamus. Afected individuals can have an unsteady gait, slow or slurred speech, clumsiness, involuntary muscle movements, muscle cramps, and seizures. Neuropsychiatric manifestations are also common presenting symptoms and can range in severity from mild memory impairments to dementia, psychosis, and personality or behavior changes. Calcifcations can be seen on CT or MRI of the head as well as with plain flm X-rays. Te diagnosis can be made once several criteria are met: bilateral basal ganglia calcifcations, progressive neurologic dysfunction, family history of basal ganglia calcifcations with an autosomal dominant pattern of inheritance, and the absence of another condition that could explain the calcifcations such as metabolic, mitochondrial, infectious, toxic, or traumatic etiologies [4,5]. Tere are a handful of case