STRIOPALLIDODENTATE CALCIFICATIONS - NOSOGRAPHIC, NEUROPATHOLOGICAL AND CLINICAL PROPOSAL - CASE-REPORT

The case of a 52-year-old man with an ataxicspastic syndrome associate d with dystonia, faciobuccolingual dyskinesias and partial epileptic s eizures is reported Neurological imaging showed aspecific brain stern parenchymal alterations. Cerebellar biopsy revealed calcifications and the accumulation of perivascular acid mucopolysaccharides compatible with a diagnosis of ''Calcinosis Nucleorum Cerebri'', with the absence of any Ca/P metabolism disturbances or other diseases predisposing to calcium deposits showing it to be sporadic and idiopathic. We feel th at this case description is interesting because of some of its particu lar aspects and that it may help in providing a better nosographic cla ssification of these diseases.