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Bilateral striopallidodentate calcinosis presenting with psychiatric symptoms and speech disorders

Published online by Cambridge University Press:  24 June 2014

Betul Ozdilek ,
Kayıhan Uluc  and
Dilek I. Gunal
Betul Ozdilek*
Affiliation:
Department of Neurology, Marmara University Hospital, Istanbul, Turkey
Kayıhan Uluc
Affiliation:
Department of Neurology, Marmara University Hospital, Istanbul, Turkey
Dilek I. Gunal
Affiliation:
Department of Neurology, Marmara University Hospital, Istanbul, Turkey
*
Betul Ozdilek, Department of Neurology, Erenkoy Research and Training Hospital for Neurology and Psychiatry,Sinan Ercan Caddesi No: 29, 34736 Erenkoy, Kadikoy, Istanbul, Turkey Tel: +90 216 302 59 59; Fax: +90 216 356 04 96; E-mail: betulozdilek@yahoo.com
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Extract

Ozdilek B, Uluc K, Gunal DI. Bilateral striopallidodentate calcinosis presenting with psychiatric symptoms and speech disorders.

Background: Bilateral striopallidodentate calcinosis (BSPDC), also known as Fahr's disease, is a rare neurodegenerative disorder characterised by the deposition of calcium and other minerals in the basal ganglia, centrum semiovale and cerebellum. It is usually idiopathic. Its clinical manifestations vary from asymptomatic individuals to neuropsychiatric abnormalities, movement disorders, cerebellar symptoms and cognitive impairments.

Methods: Five cases of BSPDC – all of which include psychiatric symptoms and speech problems – from two families are documented in this article.

Conclusion: The most important diagnostic marker is the demonstration of symmetrical intracranial calcifications. Computerised tomography of the brain is the most frequently used radiologic method to diagnose BSPDC.

Keywords

bilateral striopallidodentate calcinosiscranial computed tomographypsychiatric symptomsspeech disorders
Type
Case Reports
Information
Acta Neuropsychiatrica , Volume 24 , Issue 5 , October 2012 , pp. 310 - 313
Copyright
Copyright © Cambridge University Press 2012

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References

1.Manyam, BV.What is and what is not ‘Fahr's disease'. Parkinsonism Relat Disord 2005;11:73–80.CrossRefGoogle ScholarPubMed
2.Klein, C, Vieregge, P.Fahr's disease: far from a disease. Mov Disord 1998;13:620–621.CrossRefGoogle ScholarPubMed
3.Kobari, M, Nogawa, S, Sugimoto, Y, Fukuuchi, Y.Familial idiopathic brain calcification with autosomal dominant inheritance. Neurology 1997;48:645–649.CrossRefGoogle ScholarPubMed
4.Geschwind, DH, Loginov, M, Stern, JM.Identfication of a locus on chromosome 14q for idiopathic basal ganglia calcification (Fahr disease). Am J Hum Genet 1999;65:764–772.CrossRefGoogle Scholar
5.Harrington, MG, Macpherson, P, McIntosh, WB, Allam, BF, Bone, I.The significance of the incidental finding of basal ganglia calcification on computed tomography. J Neurol Neurosurg Psychiatry 1981;44:1168–1170.CrossRefGoogle ScholarPubMed
6.Trautner, RJ, Cummings, JL, Read, SL, Benson, DF.Idiopathic basal ganglia calcification and organic mood disorder. Am J Psychiatry 1988;145:350–353.Google ScholarPubMed
7.Cartier, L, Passig, C, Gormaz, A, Lopez, J.Neuropsychological and neurophysiological features of Fahr's disease. Rev Med Chil 2002;130:1383–1390.Google ScholarPubMed
8.Malik, R, Pandya, VK, Naik, D., Fahr disease. A rare neurodegenerative disorder. Ind J Radiol Imag 2004;14:383–384.Google Scholar
9.Modrego, PJ, Mojonero, J, Serrano, M, Fayed, N.Fahr's syndrome presenting with pure and progressive presenile dementia. Neurol Sci 2005;26:367–369.CrossRefGoogle ScholarPubMed
10.Osborn, AG.Diagnostic Neuroradiology. 2nd edn. St. Louis: Mosby, 1994:744–745.Google Scholar
11.Ogi, S, Fukumitsu, N, Tsuchida, D, Uchiyama, M, Mori, Y, Matsui, K.Imaging of bilateral striopallidodentate calcinosis. Clin Nucl Med 2002;27:721–724.CrossRefGoogle ScholarPubMed
12.Mega, MS, Cummings, JL.Frontal-subcortikal circuits and neuropsychiatric disorders. J Neuropsychiatry Clin Neurısci 1994;6:358–370.Google ScholarPubMed
13.Lopez-Villegas, D, Kulisevsky, J, Deus, J et al. Neuropsychological alterations in patients with computed tomography-detected basal ganglia calcification. Arch Neurol 1996;53:251–256.CrossRefGoogle ScholarPubMed
14.Ring, HA, Serra-Mestres, J.Neuropsychiatry of the basal ganglia. J Neurol Neurosurg Psychiatry 2002;72:12–21.CrossRefGoogle ScholarPubMed
15.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. J Neuropsychiatry Clin Neurosci 1998;10:249–266.Google Scholar
16.Cummings, JL, Gosenfeld, LF, Houlihan, JP, McCaffrey, T.Neuropsychiatric disturbances associated with idiopathic calcification of the basal ganglia. Biol Psychiatry 1983;18: 591–601.Google ScholarPubMed
17.Shibayama, H, Kobayashi, H, Nakagawa, M et al. Non Alzheimer, non-Pick dementia with Fahr's syndrome. Clin Neuropathol 1992;11:237–245.Google ScholarPubMed
18.Kazis, AD.Contribution of CT scan to the diagnosis of Fahr's syndrome. Acta Neurol Scand 1985;71:206–211.CrossRefGoogle Scholar