title: Cerebellopontine calcification: a new entity of idiopathic intracranial calcification?
abstract: We report the autopsy case of a 40-year-old woman with severe intellectual and motor disabilities, who showed calcification in the cerebellum and pons but not in the basal ganglia on CT scan, and died of intracranial hemorrhage due to intractable hypertension. At autopsy, numerous calcium deposits were noted in the cerebellar cortex, the dentate nucleus, the cerebellar white matter and the ventral pons. These deposits were distributed both in the neuropil and the white matter, but rarely within the arterial walls or in contact with capillaries. This weak relationship between calcification and the blood vessels, in addition to the paucity of basal ganglia calcification, is in contrast to the findings with other disorders involving intracranial calcification, including Fahr's disease and calcium metabolism disorders. Immunohistochemistry revealed intense staining of calbindin-D28K and parvalbumin at sites of calcium deposits both in the present case and in a case of pseudohypoparathyroidism, whereas these proteins were not localized to calcium deposits in the cerebellum of a Fahr's disease brain. We propose that the present case may represent a distinct entity among diseases characterized by idiopathic intracranial calcification. In addition, calcium-binding proteins may be involved in the calcification process in some cases with intracranial calcification.
authors.affiliation: Department of Pediatrics and Pediatric Neurology, Yokohama Ryo-iku Medical Center, Asahi-ku, Japan. saitoyo@fb3.so-net.ne.jp
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Saito
authors.forename: Makoto
authors.initials: M
authors.lastname: Shibuya
authors.forename: Masaharu
authors.initials: M
authors.lastname: Hayashi
authors.forename: Shizuko
authors.initials: S
authors.lastname: Matsuoka
authors.forename: Kaori
authors.initials: K
authors.lastname: Kaneko
authors.forename: Yuri
authors.initials: Y
authors.lastname: Chikumaru
authors.forename: Kazuyo
authors.initials: K
authors.lastname: Saito
authors.forename: Akira
authors.initials: A
authors.lastname: Matsui
authors.forename: Seiji
authors.initials: S
authors.lastname: Kimura
mesh_terms: D000328:Adult; D064092:Calbindin 1; D064026:Calbindins; D002114:Calcinosis / Q000378:metabolism / Q000473:pathology*; D002530:Cerebellopontine Angle / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006801:Humans; D006973:Hypertension / Q000503:physiopathology; D007150:Immunohistochemistry; D010320:Parvalbumins / Q000378:metabolism; D064030:S100 Calcium Binding Protein G / Q000378:metabolism; D014057:Tomography, X-Ray Computed
chemical_list: C576498:CALB1 protein, human; D064092:Calbindin 1; D064026:Calbindins; D010320:Parvalbumins; D064030:S100 Calcium Binding Protein G