dc.contributor.author |
Fàbregues-Boixar i Nebot, Oriol de |
dc.contributor.author |
Viñas, Jaume |
dc.contributor.author |
Palasí Franco, Antoni |
dc.contributor.author |
Quintana, Manuel |
dc.contributor.author |
Cardona, Ignasi |
dc.contributor.author |
Auger, Cristina |
dc.contributor.author |
Vargas, Víctor |
dc.contributor.author |
Universitat Autònoma de Barcelona |
dc.date |
2020 |
dc.identifier |
https://ddd.uab.cat/record/227680 |
dc.identifier |
urn:10.1002/brb3.1596 |
dc.identifier |
urn:oai:ddd.uab.cat:227680 |
dc.identifier |
urn:pmid:32202078 |
dc.identifier |
urn:pmcid:PMC7218247 |
dc.identifier |
urn:pmc-uid:7218247 |
dc.identifier |
urn:oai:pubmedcentral.nih.gov:7218247 |
dc.format |
application/pdf |
dc.language |
eng |
dc.publisher |
|
dc.relation |
Brain and behavior ; Vol. 10 (march 2020) |
dc.rights |
open access |
dc.rights |
Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original. |
dc.rights |
https://creativecommons.org/licenses/by/4.0/ |
dc.subject |
Ammonium tetrathiomolybdate |
dc.subject |
Efficacy |
dc.subject |
Neurological symptoms |
dc.subject |
Safety |
dc.subject |
Wilson's disease |
dc.title |
Ammonium tetrathiomolybdate in the decoppering phase treatment of Wilson's disease with neurological symptoms : A case series |
dc.type |
Article |
dc.description.abstract |
To present our experience with ammonium tetrathiomolybdate (ATTM) in the decoppering phase treatment of Wilson's disease (WD) with neurological symptoms. An uncontrolled longitudinal study was carried out to describe a case series of five patients diagnosed of WD with neurological symptoms in our hospital over the last 5 years and receiving ATTM for 8 (or 16) weeks. Unified Wilson's Disease Rating Scale (UWDRS), Global Assessment Scale (GAS) for WD and the Brewer-adapted Unified Huntington's Disease Rating Scale (UHDRS) for WD, magnetic resonance imaging, and monitoring for potential adverse effects were carried out in all patients before starting ATTM and 3 months later when ATTM was stopped and zinc treatment was initiated. All five patients presented neurological clinical improvement in UWDRS, GAS, and Brewer-adapted UHDRS for WD. Neuroimaging improvement was present in 2/5 patients with brain edema reduction. Mild anemia, leukopenia, and elevation of transaminases were detected in 1 patient, with complete remission after stopping ATTM for 1 week and then restarting at a half dose. ATTM could be a good treatment for the initial treatment of WD with neurological symptoms due to its high efficacy, with a lower rate of neurological deterioration than the drugs currently available, despite the potential adverse effects. Effectiveness and safety of ammonium tetrathiomolybdate in decoppering therapy for Wilson's disease with neurological symptoms, a pilot study in routine care. |