Neurological Sciences

, Volume 32, Issue 4, pp 537–545 | Cite as

Strio-pallido-dentate calcinosis: a diagnostic approach in adult patients

  • Sara Bonazza
  • Chiara La Morgia
  • Paolo Martinelli
  • Sabina Capellari
Review Article

Abstract

Familial idiopathic bilateral strio-pallido-dentate calcinosis is a rare autosomal dominant disorder characterized by massive symmetric calcification, detectable by CT, into the globus pallidus and striatum, with or without the involvement of the dentate nucleus, thalamus and white matter in the absence of alterations of calcium metabolism. Clinically, it has been associated with movement and/or neuropsychiatric disorders with age at onset typically in the fourth or fifth decade. Other sporadic or familial diseases can be responsible for brain calcifications with a similar anatomic strio-pallidal or strio-pallido-dentate pattern and, a restricted number of them, for neurological symptoms with onset in adulthood. Moreover, physiological age-related basal ganglia calcifications are often incidentally found, although with a far different CT aspect, in elderly patients with movement disorders. Indentifying familial and idiopathic cases may offer the opportunity to study the molecular mechanisms underlying this minerals deposition.

Keywords

Basal ganglia Strio-pallido-dentate Familial-idiopathic-strio-pallido-dentate calcinosis Calcifications Fahr’s disease 

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Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • Sara Bonazza
    • 1
  • Chiara La Morgia
    • 1
  • Paolo Martinelli
    • 1
  • Sabina Capellari
    • 1
  1. 1.Dipartimento di Scienze NeurologicheUniversità di BolognaBolognaItaly

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