Jurnal Internasional Parkinsonisme terkait SPG11: Profil klinis, pencitraan molekuler dan respon l-dopa – Faber – – Gangguan Gerakan
Background : Molecular imaging has proven to be a powerful tool for explaining degenerated pathways in various neurological diseases and has not been systematically studied in hereditary spastic paraplegia.
Purpose : To investigate dopaminergic degeneration in a group of 22 patients with hereditary spastic paraplegia associated with SPG11 mutations and evaluate treatment responses to l-19459012 dopa.
Method : Patients and controls underwent a single photon emission tomography imaging using tracer 99m Tc – TRODAT – 1. A single blind test with 600 mg l- dopa was carried out comparing UPDRS scores.
Results : Reducing the density of dopamine transporters is universal among patients. Nigerian degeneration is symmetrical and correlates with the duration of disease and motor and cognitive defects. There is no statistically significant benefit that can be indicated by the intake of dopa l- during the trial.
Conclusion : Disruption of presynaptic dopaminergic pathways is a widespread phenomenon in patients with SPG11 mutations, even in the absence of parkinsonism. The lack of response to treatment can be associated with postmenaptic damage that needs to be further investigated.