Fahr’s Syndrome: A Typical Entity Presenting as Psychosis
Abstract
Fahr’s syndrome is a rare neurological entity characterized by bilateral and symmetrical intracranial deposition of calcium mainly in the basal ganglia. Motor and Psychiatric symptoms are clinically relevant features in this syndrome. We report a case where psychiatric symptoms and features of delirium were more prominent as compared to motor symptoms. Radiological findings depicted bilateral basal ganglia calcification and oedema in parietal region mainly on right side. Parathyroid hormone and calcium levels were mildly low with no significant past or family history. Most behavioural symptoms showed significant improvement on treatment with Risperidone, Amisulpride, Lorazepam, Sodium Valproate, Calcium, Folic acid and Trihexyphenidyl but some symptoms like auditory hallucinations remained unresponsive.
How to cite this article:
Kumar P, Kukreti P, Mathur H, Saurabh, Kohat K, Kataria D. Fahr’s Syndrome: A Typical Entity Presenting as Psychosis. J Adv Res Psychol Psychother 2020; 3(1): 29-31.
DOI: https://doi.org/10.24321/2581.5822.202006
References
Srivastava S, Bhatia MS, Sharma V, Mahajan S, Rajender G. Fahr’s disease: An incidental finding in a case
presenting with psychosis. Ger J Psychiatry 2010; 13: 86-90.
Bonazza S, La Morgia C, Martinelli P, Capellari S. Striopallido-dentate calcinosis: A diagnostic approach in
adult patients. Neurol Sci 2011; 32: 537-545.
Uygur GA, Liu Y, Hellman RS, Tikofsky RS, Collier BD. Evaluation of regional cerebral blood flow in massive
intracerebral calcifications. J Nucl Med 1995; 36: 61-02.
Sobrido MJ, Hopfer S, Geschwind DH. Familial idiopathic basal ganglia calcification. In: Pagon RA, Bird TD, Dolan CR, Stephens K, editors. SourceGeneReviews. Seattle (WA): University of Washington; 2007; 1993-2004.
Chiu HF, Lam LC, Shum PP, Li KW. Idiopathic calcification of the basal ganglia. Postgrad Med J 1993; 69: 68-70.
König P. Psychopathological alterations in cases of symmetrical basal ganglia sclerosis. Biol Psychiatry
; 25: 459-468.
Nicolau Ramis J, Espino Ibáñez A, Rivera IrigoÃn R, Artigas CF, Masmiquel Comas L. Extrapyramidal symptoms dueto calcinosis cerebri in a patient with unknown primary hypoparathyroidism. Endocrinol Nutr 2012; 59: 69-71.
Goswami R, Sharma R, Sreenivas V, Gupta N, Ganapathy A, Das S. Prevalence and progression of basal ganglia
calcification and its pathogenic mechanism in patients with idiopathic hypoparathyroidism. Clin Endocrinol
(Oxf) 2012; 77: 200-206.
Swami A, Kar G. Intracranial hemorrhage revealing pseudohypoparathyroidism as a cause of Fahr
syndrome. Case Rep Neurol Med 2011; 407567.
Kazis AD. Contribution of CT scan to the diagnosis of Fahr’s syndrome. Acta Neurol Scand 1985; 71: 206-211.
Fénelon G, Gray F, Paillard F, Thibierge M, Mahieux F, Guillani A. A prospective study of patients with CT
detected pallidal calcifications. J Neurol Neurosurg Psychiatry 1993; 56: 622-625.
Modrego PJ, Mojonero J, Serrano M, Fayed N. Fahr’s syndrome presenting with pure and progressive
presenile dementia. Neurol Sci 2005; 26: 367-369.
Cummings JL, Gosenfeld LF, Houlihan JP, McCaffrey T. Neuropsychiatric disturbances associated with
idiopathic calcification of the basal ganglia. Biol Psychiatry 1983; 18: 591-601.