RESUMEN
OBJECTIVES: To characterize cognitive impairment in primary progressive multiple sclerosis (PPMS) and to correlate the pattern of cognitive deficits with brain magnetic resonance imaging (MRI) volumetric data. MATERIALS AND METHODS: In a multicenter cross-sectional study, we recruited consecutive patients with PPMS as well as age, sex, and education level-matched healthy controls (HC). All participants underwent neuropsychological (NP) assessment, and brain MRI was performed in patients with PPMS for analysis of lesion load, subcortical GM volumes, and regional cortical volumes. RESULTS: We recruited 55 patients with PPMS and 36 HC. Thirty-six patients were included in the MRI analysis. Patients with PPMS performed significantly worse than HC in all NP tests. Subcortical GM volume was significantly correlated with all NP tests, except for Stroop Test, with the largest effect for the thalamus (r=-.516 [BVMT-R DR, P=.016 FDR-corrected] to r=.664 [SDMT, P<.001 FDR-corrected]). In the stepwise linear regression model, thalamic volume was the only predictor of performance in all NP tests. CONCLUSION: Cognitive impairment is common in PPMS and affects all evaluated cognitive domains. Subcortical GM volume, particularly of the thalamus, is a strong predictor of cognitive performance, suggesting it has a central role in the pathophysiology of PPMS-related cognitive dysfunction.
Asunto(s)
Disfunción Cognitiva/diagnóstico por imagen , Disfunción Cognitiva/psicología , Imagen por Resonancia Magnética , Esclerosis Múltiple Crónica Progresiva/diagnóstico por imagen , Esclerosis Múltiple Crónica Progresiva/psicología , Adulto , Disfunción Cognitiva/epidemiología , Estudios Transversales , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Esclerosis Múltiple Crónica Progresiva/epidemiología , Pruebas Neuropsicológicas , Tálamo/diagnóstico por imagenRESUMEN
La enfermedad de Madelung o lipomatosis simétrica múltiple es una enfermedad rara caracterizada por la presencia de masas de tejido adiposo, distribuidas de forma simétrica en cuello, nuca, tronco, hombros y parte proximal de los miembros. Afecta fundamentalmente a hombres entre los 30 y 60 años con historia de etilismo crónico, siendo la resección quirúrgica el único tratamiento efectivo (AU)
Madelung's disease or Benign Symmetric Lipomatosis is a rare disease characterised by masses of adipose tissue spread symmetrically along the neck, trunk, shoulders and upper arms. It affects men from 30 to 60 years old with a previous history of alcoholism. Surgery is the only effective treatment (AU)