Parkinsonism in Gaucher's disease type 1: Ten new cases and a review of the literature

✍ Scribed by Ichraf Kraoua; Jérôme Stirnemann; Maria João Ribeiro; Tiphaine Rouaud; Marc Verin; Agnès Annic; Christian Rose; Luc Defebvre; Liliane Réménieras; Michaël Schüpbach; Nadia Belmatoug; Marie Vidailhet; Frédéric Sedel

• Publisher: John Wiley and Sons
• Year: 2009
• Tongue: English
• Weight: 816 KB
• Volume: 24
• Category: Article
• ISSN: 0885-3185
• DOI: 10.1002/mds.22593

✦ Synopsis

Abstract

Parkinsonism has been described in patients with Gaucher's disease (GD). We reviewed the 10 cases of patients with both parkinsonism and GD recorded in the French national GD registry, as well as 49 previously published cases. Relative to the general population, parkinsonism in GD patients (1) was more frequent, (2) occurred at an earlier age, (3) responded less well to levodopa, and (4) was more frequently associated with signs of cortical dysfunction. Enzyme replacement therapy (ERT) and substrate reduction therapy (SRT) were ineffective on GD‐associated parkinsonism, suggesting that parkinsonism itself is not an indication for ERT or SRT in this setting. © 2009 Movement Disorder Society