Movement disorders : official journal of the Movement Disorder Society · 2018
SPG11-related parkinsonism: Clinical profile, molecular imaging and l-dopa response.
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Faber Ingrid, Martinez Alberto Rolim Muro, Martins Carlos Roberto, Maia Maidane Luise, Souza Juliana Pasquotto, Lourenço Charles Marques, Marques Wilson, Montecchiani Celeste, Orlacchio Antonio, Pedroso Jose Luiz, Barsottini Orlando Graziani Povoas, Ramos Celso Darío, Lopes-Cendes Íscia, Friedman Joseph H, Amorim Bárbara Juarez, França Marcondes Cavalcante
Аннотация
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 99m Tc-TRODAT-1 tracer. A single-blind trial with 600 mg 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.
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 99m Tc-TRODAT-1 tracer. A single-blind trial with 600 mg 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.
Предметные рубрики MeSH
AdultAntiparkinson AgentsBrainCognition DisordersCohort StudiesDopamine Plasma Membrane Transport ProteinsFemaleHumansLevodopaMaleMental DisordersMiddle AgedMutationNeuropsychological TestsOrganotechnetium CompoundsParkinsonian DisordersProteinsSingle-Blind MethodStatistics, NonparametricTomography Scanners, X-Ray ComputedTomography, Emission-Computed, Single-PhotonTropanesYoung Adult