FAHR SYNDROME: Three cases presenting with psychiatric signs

Year 2006, Volume: 3 Issue: 1, 35 - 40, 01.03.2006

Murat Gülsün Ali Fuat Baykız Serdar Kabataş Hasan Belli

Abstract

-

Keywords

-

FAHR SYNDROME: Three cases presenting with psychiatric signs

Abstract

Fahr syndrome, a clinical entity that manifests with various signs and symptoms and has a familial predisposition is characterized by symmetric calcification of basal ganglia. It may present with neuropsychiatric, extrapyramidal and cerebellar symptoms. The etiology has not been defined yet. Three cases of Fahr syndrome; presenting with various clinical signs and psychiatric symptoms are reported in this article. The first case that occured following Brucella treatment and the other two occuring idiopathically are evaluated with a neuropsychiatric approach. Key words: Fahr syndrome, basal ganglia, calcification, Brucella, anxiety, psychosis

Keywords

Fahr syndrome , basal ganglia , calcification , Brucella , anxiety , psychosis

References

• Foley J, Calcification of the corpus stiatum and dentate nuclei occurring in a family. J Neurochem 1951;253-61
• Gomille T, Meyer RA , Falkai P, Gaebel W, Konigshausen T, Christ F. Prevalence and clinical significance of computerized tomography verified idiopathic calcinosis of the basal ganglia. Radiology 2001; 41;205-10
• Bruyn GW, Bots GT, Staal A. Familial bilateral vascular calcification in the central nervous system , Psychiatr Neurol Neurochir 1964; 342-76
• Konig P, Haller R. Results of a study of a family with aggregated occurrence of bilateral symmetrical basal ganglia calcinosis. Eur Arch Psychiatry Neurol Sci 1985;234;325-34
• Larsen TA, Dunn HG, Jan JE, Calne DB, Dystonia and calcification of the basal ganglia. Neurology 1985;35;533-7
• Ellie E, Julie J, Ferrer X. Familial idiopathic calcifications. Neurology 1989;39;381-5
• Cummings JL, Gosenfeld LF, Houlihan JP, McCaffrey T. Neuropsychiatric disturbances associated with idiopathic calcification of the basal ganglia. Biol Psychiatry 1983;18;591-601
• Lauterbach EC, Cummings JL, Duffy J et al. Neuropsychiatric correlates and treatment of lenticulostriatal diseases: a review of the literature and overview of research opportunities in Huntington’s, Wilson’s, and Fahr’s diseases. A J 1998;10;249-66 Clin Neurosci
• Hamilton M. The assessment of anxiety states by rating. Br J Med Psychol 1959; 55
• Anonymous, Diagnostic and Statistical Manual of Mental Disorders - Fourth Edition (DSM-IV), published by the. American Washington D C, 1994 Association,
• Williams JB. Standardizing the Hamilton Depression Rating Scale: past, present, and future, Eur Arch Psychiatry Clin Neurosci 2001;251;(Suppl 2):116-12
• Overall JE, Gorham DR. The brief psychiatric rating scale. Psychological Rep 1962;799
• Cummings JL, Gosenfeld LF, Houlihan JP, McCaffrey T. Neuropsychiatric disturbances associated with idiopathic calcification of the basal ganglia. Biol Psychiatry 1983;18:591-601
• Morita M, Tsuge I, Matsuoka H et al. Calcification in the basal ganglia with chronic active Epstein-Barr virus infection, Neurology 1998:50;1485-8
• Gastellu J, Garin-Bastuji B, Oudar J. Pathologic features of a case of systemic brucellosis due to Brucella melitensis biovar 3 in a chamois (Rupicapra rupicapra) Bull Acad Natl Med 1993;177(4):575-83
• Gerfen CR. Molecular effects of dopamine on striatal-projection pathways. Trends Neurosci 2000; 23; S64-S70
• Canales JJ, Graybiel AM. A measure of striatal function predicts motor stereotypy. Nat Neurosci 2000;3;377-83
• Lopez-Villegas D, Kulisevsky J, Deus J et al. Neuropsychological alterations in patients with computed tomography- detected basal ganglia calcification. Arch Neurol 1996;53;251-6
• Chiu HF, Lam LC, Shum PP, Li KW. Idiopathic calcification of the basal ganglia. Postgrad Med J 1993;69:68-70