Acta Neuropathologica

, Volume 110, Issue 1, pp 77–83

Cerebellopontine calcification: a new entity of idiopathic intracranial calcification?

Authors

    • Department of Pediatrics and Pediatric NeurologyYokohama Ryo-iku Medical Center
    • Department of PediatricsTokyo Women’s Medical University
  • Makoto Shibuya
    • Department of Laboratory MedicineTokai University
  • Masaharu Hayashi
    • Department of Clinical NeuropathologyTokyo Metropolitan Institute for Neuroscience
  • Shizuko Matsuoka
    • Department of NeurologyYokohama Stroke and Brain Center
  • Kaori Kaneko
    • Department of Pediatrics and Pediatric NeurologyYokohama Ryo-iku Medical Center
    • Department of PediatricsTokyo Women’s Medical University
  • Yuri Chikumaru
    • Department of Pediatrics and Pediatric NeurologyYokohama Ryo-iku Medical Center
  • Kazuyo Saito
    • Department of Pediatrics and Pediatric NeurologyYokohama Ryo-iku Medical Center
  • Akira Matsui
    • Department of Pediatrics and Pediatric NeurologyYokohama Ryo-iku Medical Center
  • Seiji Kimura
    • Department of Pediatrics and Pediatric NeurologyYokohama Ryo-iku Medical Center
Case Report

DOI: 10.1007/s00401-005-1011-y

Cite this article as:
Saito, Y., Shibuya, M., Hayashi, M. et al. Acta Neuropathol (2005) 110: 77. doi:10.1007/s00401-005-1011-y

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.

Keywords

CalcificationCalcium-binding proteinCerebellumImmunohistochemistryPons

Copyright information

© Springer-Verlag 2005