![]() The reviewed criteria for the diagnosis of CBS and CBD report that AL phenomena are described in 30% of CBD patients ( 5). In context, several CBS cases exhibit pathology indicative of Alzheimer's disease, frontotemporal lobar degeneration (FTLD), Creutzfeldt–Jakob disease, and progressive supranuclear palsy ( 1, 3, 4). CBS was initially described as corticobasal degeneration, a condition characterized by progressive atrophy of frontotemporal regions and typical tau-containing inclusions known as “coiled bodies” and “astrocytic plaques” ( 2).Ī syndromic definition was eventually found to be more appropriate, as distinct CBD-related pathology is only present in <40% of subjects exhibiting CBS signs. The corticobasal syndrome (CBS) is a complex and progressive clinical picture featured by progressive asymmetric rigidity with apraxia, associated with the variable presence of cortical sensory symptoms, cortical myoclonus, alien limb (AL) phenomena, aphasia, cognitive disorders, dysarthria, bradykinesia, and tremor. In addition, we provide a complementary set of video clips depicting CBD-related involuntary movements that should not mistakenly be interpreted as signs of AL. The review also offers an overview of functional magnetic resonance imaging-based studies evaluating AL-related phenomena. Thus, in our opinion, the AL term should be employed in association with precise descriptions of the accompanying involuntary movements, sensory misperceptions, agnosia-asomatognosia contents, and the presence of utilization behavior. More discrete sets of regionally bound clinical signs that depend on dysfunctions of specific brain areas need to be assessed and presented when posing the diagnosis. We propose that the term AL is appropriate to describe clinical features associated with specific brain lesions. ![]() This catchall AL term should instead be deconstructed. We reviewed the current literature on AL in CBD and now propose that the generic use of the term AL should be avoided. Thus, to make a proper diagnosis of AL in the context of CBD, cognitive and language dysfunctions must be carefully verified and objectively assessed. However, asomatognosia requires, by definition, intact perception and cognition. In this context, the most frequent symptoms are language and praxis deficits and cortical sensory misperception. However, the gist of the behavioral features of AL is still “a matter of debate.” CBD-related AL has so far included the description of involuntary movements, frontal release phenomena (frontal AL), or asomatognosia (posterior or “real” AL). 5YDA Foundation, Institute of Immune Therapy and Advanced Biological Treatment, Pescara, ItalyĬonsensus criteria on corticobasal degeneration (CBD) include alien limb (AL) phenomena.3Department of Biomedicine, Neuroscience and Advanced Diagnostic (BiND), University of Palermo, Palermo, Italy.2Center for Advanced Studies and Technology (CAST), “G.D'Annunzio” University of Chieti-Pescara, Chieti, Italy 1Department of Neuroscience, Imaging and Clinical Sciences, “G.Sensi 1,2, Marco Onofrj 1,2,5 and Raffaella Franciotti 1 * Martina Di Pietro 1,2, Mirella Russo 1,2, Fedele Dono 1,2, Claudia Carrarini 1,2, Astrid Thomas 1,2, Vincenzo Di Stefano 1,3, Roberta Telese 1,4, Laura Bonanni 1,2, Stefano L.
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