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Neurological deficits are associated with increased brain calcinosis, hypoperfusion, and hypometabolism in idiopathic basal ganglia calcification
Author(s) -
Saiki Misuzu,
Saiki Shinji,
Sakai Koichiro,
Matsunari Ichiro,
Higashi Kotaro,
Murata Kenya,
Hattori Nobutaka,
Hirose Genjiro
Publication year - 2007
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.21438
Subject(s) - basal ganglia , asymptomatic , calcinosis , medicine , proband , dentate nucleus , basal ganglia disease , calcification , pathology , central nervous system disease , cardiology , central nervous system , biology , cerebellum , mutation , biochemistry , gene
We report two familial cases of idiopathic basal ganglia calcification. A 60‐year‐old proband with choreoathetosis, dysarthria, and cognitive decline showed more extensive brain calcinosis, hypoperfusion, and hypometabolism than did his asymptomatic 82‐year‐old mother. The mother had no frontal lobe calcinosis but basal ganglia and dentate nucleus depositions were detectable. Perfusion neuroimaging, however, was normal in the asymptomatic mother and abnormal in the clinically impaired proband. The presence of calcinosis cannot be used as an index of neurological impairment but the extent of calcinosis and reduction in perfusion and metabolism may be useful for separating symptomatic from asymptomatic subjects with IBGC. These findings suggest that an interruption of neuronal circuitry may cause neurological deficits. The degree of neurological deficits may correlate with the severity of calcinosis and the reduction of perfusion and metabolism. © 2007 Movement Disorder Society