title: Neurological deficits are associated with increased brain calcinosis, hypoperfusion, and hypometabolism in idiopathic basal ganglia calcification.
abstract: 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.
authors.affiliation: Department of Neurology, Kanazawa Medical University, Ishikawa, Japan.
authors.forename: Misuzu
authors.initials: M
authors.lastname: Saiki
authors.forename: Shinji
authors.initials: S
authors.lastname: Saiki
authors.forename: Koichiro
authors.initials: K
authors.lastname: Sakai
authors.forename: Ichiro
authors.initials: I
authors.lastname: Matsunari
authors.forename: Kotaro
authors.initials: K
authors.lastname: Higashi
authors.forename: Ken-Ya
authors.initials: KY
authors.lastname: Murata
authors.forename: Nobutaka
authors.initials: N
authors.lastname: Hattori
authors.forename: Genjiro
authors.initials: G
authors.lastname: Hirose
mesh_terms: D000369:Aged, 80 and over; D001480:Basal Ganglia Diseases / Q000473:pathology* / Q000503:physiopathology*; D002114:Calcinosis / Q000503:physiopathology*; D005192:Family Health; D005260:Female; D006801:Humans; D008297:Male; D008661:Metabolism, Inborn Errors / Q000503:physiopathology*; D008875:Middle Aged; D049268:Positron-Emission Tomography / Q000379:methods; D015899:Tomography, Emission-Computed, Single-Photon / Q000379:methods; D014057:Tomography, X-Ray Computed / Q000379:methods