Facial emotion recognition and cerebral white matter lesions in myotonic dystrophy type 1

Facial memory deficits in myotonic dystrophy type 1

“…In a study on nine patients with DM1, Kobayakawa et al. concluded that patients could discriminate faces just as well as healthy controls, when using a simple facial matching task. The findings in our study however indicate that more than 1 in 3 DM1 patients may have problems in facial discrimination when the task requires the storage and retrieval of a face over time.…”

“…Consequently, it is possible to criticise this task as a weak measure regarding facial processing and subsequent remembering. However, it is also possible to argue that feature‐based strategies are used in everyday recognition of unfamiliar faces and may be a naturalistic reflection of everyday facial memory ability . Secondly, patients and healthy control persons differed in their level of education.…”

Myotonic Dystrophies: State of the Art of New Therapeutic Developments for the CNS

“…A large number of DM 1 patients showed reduced ability to recognize facial expressions in recognition tasks and have difficulty in understanding others' mental states from both interaction with others in everyday situations and from their facial expressions. They are impaired in tests assessing the theory of mind (ToM, ability to infer other people's mental states, thoughts and feelings; Takeda et al, 2009; Kobayakawa et al, 2010, 2012; Masaoka et al, 2011; Winblad et al, 2016). In addition, reduced awareness of disease burden is common (Baldanzi et al, 2016a).…”

“…Sleepiness in DM1 was found inversely associated with white matter fiber integrity (Wozniak et al, 2013). Correlations were found between the sensitivity to facial emotions and frontal, temporal, and insular white matter lesions (Takeda et al, 2009; Kobayakawa et al, 2010). Interestingly, comparison of DM1 and DM2 patients cohorts revealed widespread cortical and subcortical gray matter and white matter atrophy in both types of DM patients especially in the central motor pathways in DM1 and limbic structures in DM2 in correlation with motor dysfunction, cognitive abilities depression, daytime sleepiness, and reduced executive functions (Schneider-Gold et al, 2015; Serra et al, 2015).…”

Estudio descriptivo del perfil neuropsicológico y psicopatológico en pacientes con distrofia miotónica tipo 1

“…En la forma congénita es frecuente el retraso mental, leve o moderado, mientras que en la variante de inicio adulto son habituales otros síntomas cognitivos [4]. Se han descrito alteraciones en habilidades visuoespaciales y visuoconstructivas [5][6][7][8][9][10][11], ejecutivas, en razonamiento abstracto verbal [5][6][7][8][9][10][11][12][13][14][15], atención, memoria visuoespacial [7], facial [1] y verbal a corto y largo plazo [5], funcionamiento lingüístico [8,11,16] y cognición social [17][18][19], y déficits relacionados con las medidas de personalidad, comportamiento, emociones y fun-…”