Our study identifies that de novo or recently worsening anxiety and specific neuropsychological profiles call for screening for LPPA, including a linguistic examination. Sometimes, there may be a continuum between LPPA and corticobasal syndrome.
Few language disorders have been reported in posterior cortical atrophy (PCA). Furthermore, no study has focused on screening for them and described these language deficits. The goal of this work was to describe linguistic examination of PCA patients and the impact of language disorders on neuropsychological performances compared to patients with other neurodegenerative syndromes and control groups. Linguistic examination of 9 PCA patients was carried out. The neuropsychological performance of the PCA group (16 patients) in the RAPID battery tests was compared with performances of patients with a logopenic variant of primary progressive aphasia (LPPA), patients with Alzheimer's disease and patients with amnestic mild cognitive impairment, as well as the control group. A "logopenic syndrome" with anomia, fluency impairment, and length-dependent deficit was found in 8/9 PCA patients. A comparison with other neurodegenerative syndromes showed that not only visual disorders but also language and verbal short-term memory disorders, such as those found in LPPA, can explain neuropsychological performances. A "logopenic syndrome" is frequently found in PCA and may be associated with poor performance on other verbally mediated neuropsychological tasks (e.g., verbal memory). Specific logopedic rehabilitation should be offered to these patients.
The aim of this work is to study the cognition, progressive gait impairment, and neuroimaging findings in two patients over 65 years old of the previously described type 3 familial cortical myoclonic tremor with epilepsy (FCMTE3). We report investigations in two of these five FCMTE3 subjects over 65 and showing progressive gait disorders. They both had a pseudo-Parkinson's way of walking and visual intolerance to bright light and brightness contrast without EEG abnormalities exacerbating cortical myoclonus or triggering seizure. Case 1 had moderate gait impairment and a severe frontal syndrome. Case 2 had severe gait impairment and diffuse cognitive disorders. Both cases had cortical hypoperfusion (predominantly in the left frontal lobe) and no cerebellar abnormality on cerebral perfusion SPECT. DAT-SPECT showed dopaminergic depletion. These data indicate fronto-striatal dysfunction associated with gait impairment and cognitive disorders appearing after several decades of disease progression. This gives clues to understanding the pathogenesis and evolution of FCMTE3. Permanent myoclonic discharges or long-term valproate treatment may cause significant toxic effects on neurons (dopaminergic and frontal neurons). Further functional and molecular analyses are required in order to better understand this pathology and the consequences of chronic cortical myoclonus.
The combination of MIS and IST could be a useful tool for screening dementias. The "or" rule combination seems particularly adapted to medical practice.
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