Cerebellopontine calcification: a new entity of idiopathic intracranial calcification?
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.
Saito
Yoshiaki
Y
Department of Pediatrics and Pediatric Neurology, Yokohama Ryo-iku Medical Center, Asahi-ku, Japan. saitoyo@fb3.so-net.ne.jp
Shibuya
Makoto
M
Hayashi
Masaharu
M
Matsuoka
Shizuko
S
Kaneko
Kaori
K
Chikumaru
Yuri
Y
Saito
Kazuyo
K
Matsui
Akira
A
Kimura
Seiji
S
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
C576498:CALB1 protein, human; D064092:Calbindin 1; D064026:Calbindins; D010320:Parvalbumins; D064030:S100 Calcium Binding Protein G