Abstract
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 feel 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.
Sommario
Descriviamo il caso di un uomo di 52 anni, affetto da una sindrome atasso-spastica associata a distonia, discinesie facio-buccolinguali, crisi epilettiche parziali. Le neuroimmagini mostrano alterazioni parenchimali troncoencefaliche e cerebellari di natura aspecifica. La biopsia cerebellare rileva calcificazioni ed accumulo di mucopolisaccaridi acidi perivascolari, compatibili con una diagnosi di “Calcinosis Nucleorum Cerebri”, sporadica ed idiopatica per l'assenza di disturbi del metabolismo Ca/P e di altre patologie predisponenti i depositi calcifici. Riteniamo interessante la descrizione del caso per alcuni suoi aspetti peculiari e per un miglior inquadramento nosografico di queste forme morbose.
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Francia, A., Calandriello, L., Restante, R. et al. Striopallidodentate calcifications: nosographic, neuropathological and clinical proposal. Case report. Ital J Neuro Sci 17, 169–173 (1996). https://doi.org/10.1007/BF02000851
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DOI: https://doi.org/10.1007/BF02000851