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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: [email protected]
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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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