Levodopa therapy in a Lesch-Nyhan disease patient: Pathological, biochemical, neuroimaging, and therapeutic remarks
✍ Scribed by Mercedes Serrano; Belen Pérez-Dueñas; Aida Ormazábal; Rafael Artuch; Jaume Campistol; Rosa J. Torres; Angels García-Cazorla
- Publisher
- John Wiley and Sons
- Year
- 2008
- Tongue
- English
- Weight
- 749 KB
- Volume
- 23
- Category
- Article
- ISSN
- 0885-3185
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✦ Synopsis
Abstract
Lesch‐Nyhan disease (LND) is a hereditary disorder of purine metabolism causing severe neurobehavioral disturbances in which an abnormal central nervous system dopaminergic function has been implied. However, levodopa treatment has rarely been used, and reports describe heterogeneous responses. We report an LND patient with low dopamine metabolite values in cerebrospinal fluid for whom early levodopa/carbidopa therapy was begun with a notable clinical improvement. We propose that very early treatment of LND patients with levodopa may improve their neurological symptoms and may contribute to a better outcome. © 2008 Movement Disorder Society