TY - JOUR
T1 - Scintigraphic, neuroradiological and clinical comparison in two patients with primary sporadic and two with secondary Fahr's disease
AU - Caranci, Giovanni
AU - Grillea, Giovanni
AU - Barbato, Francesco
AU - Brunetti, Alexandra
AU - Tassinari, Tiziana
AU - Di Ruzza, Mariarita
AU - Lena, Francesco
AU - Gandolfi, Barbara
AU - Castellano, Anna Elisa
AU - Bartolo, Marcello
AU - Colonnese, Claudio
AU - Modugno, Nicola
AU - Ruggieri, Stefano
AU - Manfredi, Mario
PY - 2011/4
Y1 - 2011/4
N2 - Bilateral striopallidodentate calcification, usually termed Fahr's disease, can give rise to various clinical manifestations including hyperkinetic movement disorders or a hypokinetic Parkinsonian syndrome, behavioural and mood changes, cognitive deficits and even frank dementia. We describe four patients all of whom underwent a detailed scintigraphic, neuroradiological and clinical work-up: two had primary, sporadic Fahr's disease and two had Fahr's disease secondary to hypoparathyroidism. The neuroradiological and clinical studies disclosed similar anatomical and pathological changes in the four patients but variable and sometimes unexpected clinical manifestations. Both patients with primary forms had hypokinetic Parkinsonian syndrome, both patients with secondary forms had hyperkinetic movements. Dopamine autotransporter scan brain scintigraphy disclosed an unexpected unilateral putamen involvement despite substantially symmetric calcifications.
AB - Bilateral striopallidodentate calcification, usually termed Fahr's disease, can give rise to various clinical manifestations including hyperkinetic movement disorders or a hypokinetic Parkinsonian syndrome, behavioural and mood changes, cognitive deficits and even frank dementia. We describe four patients all of whom underwent a detailed scintigraphic, neuroradiological and clinical work-up: two had primary, sporadic Fahr's disease and two had Fahr's disease secondary to hypoparathyroidism. The neuroradiological and clinical studies disclosed similar anatomical and pathological changes in the four patients but variable and sometimes unexpected clinical manifestations. Both patients with primary forms had hypokinetic Parkinsonian syndrome, both patients with secondary forms had hyperkinetic movements. Dopamine autotransporter scan brain scintigraphy disclosed an unexpected unilateral putamen involvement despite substantially symmetric calcifications.
KW - Fahr's disease
KW - Functional imaging
KW - Hyperkinetic movements
KW - Hypoparathyroidism
UR - http://www.scopus.com/inward/record.url?scp=79952897884&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=79952897884&partnerID=8YFLogxK
U2 - 10.1007/s10072-010-0444-9
DO - 10.1007/s10072-010-0444-9
M3 - Article
C2 - 21057841
AN - SCOPUS:79952897884
VL - 32
SP - 337
EP - 341
JO - Neurological Sciences
JF - Neurological Sciences
SN - 1590-1874
IS - 2
ER -