Striopallidodentate calcifications: nosographic, neuropathological and clinical proposal. Case report

The case of a 52-year-old man with an ataxicspastic syndrome associated with dystonia, faciobuccolingual dyskinesias and partial epileptic seizures is reported. Neurological imaging showed aspecific brain stem 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 fell that this case description is interesting because of some of its particular aspects and that it may help in providing a better nosographic classification of these diseases.