Atypical mixed logopenic and semantic progressive primary aphasia (PPA) in Arabic with white matter abnormalities

Abstract

AbstractBackgroundThe concept of primary progressive aphasia (PPA) or Mesulam syndrome (Mesulam, 2014) is an isolated and progressive deterioration of language, usually due to progressive focal atrophy of the left perisylvian regions (of the dominant hemisphere). Four types of PPA have been already identified: Non‐fluent variant or Nfv‐PPA; Logopenic variant or Lv‐PPA; Semantic variant or Sv‐PPA and Mixed variant M‐PPA (Gorno‐Tempini et al., 2011; Mesulam et al., 2014).MethodWe report MH who displayed a Mixed‐variant of PPA (M‐PPA) in Arabic. Her daily activities were preserved. Neuroimaging using MRI coronal cut (FLAIR) showed a bi‐hippocampal atrophy, an increase in the sylvian valley and ventricular dilation; signs of cortical and subcortical vascular leukoencephalopathy. While the axial T2 cut showed a peri‐ventricular leukoencephalopathy. In FDG‐PET, there was a hypometabolism in the left anterior and the upper temporal gyrus. A neuropsychological battery was administered to the patient as well as a neurolinguistic assessment using an adapted and validated version of Moroccan Mini‐Linguistic State Examination (MLSE). It has been developed in English and Italian (Catricalà et al, 2017; Patel et al, 2020).ResultThe clinical profile of MH clearly showed an atypical overlap of neuropsychological, neurolinguistic and neuroradiological features which objectified a mixed variant of PPA predominantly logopenic and semantic with a slight syntactic impairment. These linguistic disturbances included some elements of Lv‐PPA (impaired sentence repetition and dysnomia) and some features of Sv‐PPA (dysnomia, impaired word comprehension, semantic association and sentence comprehension).ConclusionThe findings of this case study were mainly based on clinical and semiological criterias which are consistent with Mesulam’s descriptions (2014). It is possible that the presence of multi‐domain linguistic deficits may be linked eventually to a mixed underlying neuropathology which might be both neurodegenerative and vascular. This study is a call for a cross‐cultural neuropsychological, neuro‐anatomical and neurolinguistic characterization of PPA and Mixed‐PPA in non‐European languages