| Full text | |
| Author(s): Show less - |
Faber, Ingrid
[1]
;
Muro Martinez, Alberto Rolim
[1]
;
Martins, Jr., Carlos Roberto
[1]
;
Maia, Maidane Luise
[2]
;
Souza, Juliana Pasquotto
[2]
;
Lourenco, Charles Marques
[3]
;
Marques, Jr., Wilson
[3]
;
Montecchiani, Celeste
[4]
;
Orlacchio, Antonio
[5, 4]
;
Pedroso, Jose Luiz
[6]
;
Povoas Barsottini, Orlando Graziani
[6]
;
Ramos, Celso Dario
[2]
;
Lopes-Cendes, Iscia
[7]
;
Friedman, Joseph H.
[8, 9]
;
Amorim, Barbara Juarez
[2]
;
Franca, Jr., Marcondes Cavalcante
[1]
Total Authors: 16
|
| Affiliation: | [1] Univ Estadual Campinas, Dept Neurol, R Tessalia Vieira de Camargo 126, BR-13083887 Campinas, SP - Brazil
[2] Univ Estadual Campinas, Div Nucl Med, Dept Radiol, Campinas, SP - Brazil
[3] Univ Sao Paulo USP RP, Dept Neurol, Ribeirao Preto - Brazil
[4] Ist Ricovero & Cura Carattere Sci IRCCS Santa Luc, Lab Neurogenet, CERC, Rome - Italy
[5] Univ Perugia, Dipartimento Sci Chirurg & Biomed, Perugia - Italy
[6] Fed Univ Sao Paulo UNIFESP, Dept Neurol, Sao Paulo - Brazil
[7] Univ Estadual Campinas, Dept Med Genet, Campinas, SP - Brazil
[8] Brown Univ, Butler Hosp, Dept Neurol, Providence, RI 02912 - USA
[9] Brown Univ, Warren Alpert Med Sch, Providence, RI 02912 - USA
Total Affiliations: 9
|
| Document type: | Journal article |
| Source: | MOVEMENT DISORDERS; v. 33, n. 10, p. 1650-1656, OCT 2018. |
| Web of Science Citations: | 2 |
| Abstract | |
Background: Molecular imaging has proven to be a powerful tool to elucidate degenerated paths in a wide variety of neurological diseases and has not been systematically studied in hereditary spastic paraplegias. Objectives: To investigate dopaminergic degeneration in a cohort of 22 patients with hereditary spastic paraplegia attributed to SPG11 mutations and evaluate treatment response to l-dopa. Methods: Patients and controls underwent single-photon emission computed tomography imaging utilizing Tc-99m-TRODAT-1 tracer. A single-blind trial with 600mg of l-dopa was performed comparing UPDRS scores. Results: Reduced dopamine transporter density was universal among patients. Nigral degeneration was symmetrical and correlated with disease duration and motor and cognitive handicap. No statistically significant benefit could be demonstrated with l-dopa intake during the trial. Conclusion: Disruption of presynaptic dopaminergic pathways is a widespread phenomenon in patients with SPG11 mutations, even in the absence of parkinsonism. Unresponsiveness to treatment could be related to postsynaptic damage that needs to be further investigated. (AU) | |
| FAPESP's process: | 13/01766-7 - Sensory neuronopathies: investigation of new diagnostic methods, mechanisms of the disease and therapeutic strategies |
| Grantee: | Marcondes Cavalcante Franca Junior |
| Support Opportunities: | Research Grants - Young Investigators Grants |