RESUMEN
Idiopathic rapid eye movement (REM) sleep behavior disorder (iRBD) is a prodromal stage of α-synucleinopathies, such as Parkinson's disease (PD), which are characterized by the loss of dopaminergic neurons in substantia nigra, associated with abnormal iron load. The assessment of presymptomatic biomarkers predicting the onset of neurodegenerative disorders is critical for monitoring early signs, screening patients for neuroprotective clinical trials and understanding the causal relationship between iron accumulation processes and disease development. Here, we used Quantitative Susceptibility Mapping (QSM) and 7T MRI to quantify iron deposition in Nigrosome 1 (N1) in early PD (ePD) patients, iRBD patients and healthy controls and investigated group differences and correlation with disease progression. We evaluated the radiological appearance of N1 and analyzed its iron content in 35 ePD, 30 iRBD patients and 14 healthy controls via T2*-weighted sequences and susceptibility (χ) maps. N1 regions of interest (ROIs) were manually drawn on control subjects and warped onto a study-specific template to obtain probabilistic N1 ROIs. For each subject the N1 with the highest mean χ was considered for statistical analysis. The appearance of N1 was rated pathological in 45% of iRBD patients. ePD patients showed increased N1 χ compared to iRBD patients and HC but no correlation with disease duration, indicating that iron load remains stable during the early stages of disease progression. Although no difference was reported in iron content between iRBD and HC, N1 χ in the iRBD group increases as the disease evolves. QSM can reveal temporal changes in N1 iron content and its quantification may represent a valuable presymptomatic biomarker to assess neurodegeneration in the prodromal stages of PD.
Asunto(s)
Sobrecarga de Hierro , Enfermedad de Parkinson , Trastorno de la Conducta del Sueño REM , Sinucleinopatías , Biomarcadores , Progresión de la Enfermedad , Humanos , Hierro , Sobrecarga de Hierro/diagnóstico por imagen , Enfermedad de Parkinson/diagnóstico por imagen , Enfermedad de Parkinson/patología , Síntomas Prodrómicos , Trastorno de la Conducta del Sueño REM/diagnóstico por imagen , Trastorno de la Conducta del Sueño REM/patologíaRESUMEN
Abducens nerve palsy is a common ocular motor paralysis with a broad set of etiopathogenetic causes. Magnetic resonance imaging is a key diagnostic technique to investigate organic causes of sixth nerve palsy, as it allows a detailed representation of the course of the nerve, particularly in its intracisternal tract. Anatomical variants of the sixth cranial nerve comprise duplications and fenestrations in various traits. Anatomical variants of cerebellar arteries have also been described. We report the case of a patient with abducens nerve palsy presumably related to a neurovascular conflict due to a peculiar anatomical variant, which consists in a cerebellar artery passing through the intracisternal duplication of the abducens nerve.