AI Summary & Key Takeaways
AI InsightPatients under levodopa or dopamine agonist therapy due to parkinsonian symptoms responded poorly and a progressive clinical course was observed in all five patients.It is important to consider this rare syndrome in the differential diagnosis of the patients displaying rapidly progressive parkinsonism and/or cerebellar symptoms accompanied by autonomic signs and responding poorly to Parkinson disease therapy.
📌 3 Key Takeaways
Abstract
Multiple system atrophy (MSA) is a rare, sporadic and progressive neurodegenerative syndrome characterized by various combinations of parkinsonian, cerebellar, autonomic or pyramidal signs with an onset in adulthood. It has been classified as MSA-P or MSA-C depending on the type of dominant symptoms, either parkinsonism or cerebellar ataxia, respectively.In this report, we have presented five patients diagnosed as MSA based on the clinical signs varying from parkinsonian symptoms to cerebellar ataxia. While dysarthria, hyperreflexia and urinary dysautonomia were the common findings in all of these patients, other clinical and radiological signs supporting MSA were observed in different combinations. Patients under levodopa or dopamine agonist therapy due to parkinsonian symptoms responded poorly and a progressive clinical course was observed in all five patients.It is important to consider this rare syndrome in the differential diagnosis of the patients displaying rapidly progressive parkinsonism and/or cerebellar symptoms accompanied by autonomic signs and responding poorly to Parkinson disease therapy.
Özet
Multipl sistem atrofi (MSA); otonomik disfonksiyon, parkinsonizm, serebellar ataksi ve piramidal bulguların değiflken bileflimleriyle karakterize; nadir görülen eriflkin bafllangıçlı, sporadik, ilerleyici bir nörodejeneratif sendromdur. Baskın bulguların parkinsonizm veya serebellar ataksi olmasına göre MSA-P ve MSA-C olarak iki alt gruba ayrılır.Bu bildiride klinik bulguları parkinsonizmden serebellar bozukluğa uzanan ve MSA düflündüren özgün klinik belirtilere sahip befl hasta sunulmufltur. Disartri, hiperrefleksi ve üriner disotonomi bulguları tüm hastalarda saptanırken, MSA tanısını destekleyen diğer klinik ve radyolojik bulgular farklı kombinasyonlarda bulunmaktaydı. Parkinsonizm bulguları nedeniyle dopamin agonisti ve L-dopa tedavisi uygulanan hastalarda tedaviye yanıt yetersizdi ve tüm hastalarda klinik seyir ilerleyici özellikteydi. Hızlı ilerleyici parkinsonizm ve/veya serebellar bulguları olan, otonomik belirtilerin efllik ettiği, Parkinson hastalığı tedavisine yetersiz yanıt veren hastaların ayırıcı tanısında bu nadir görülen sendromun düflünülmesi önemlidir.