Berthier, Marcelo L.Hoet, FlorenciaBeltrán-Corbellini, ÁlvaroSantana-Moreno, DanielEdelkraut, LisaDávila, Guadalupe2022-12-122022-12-122021-05-04Berthier ML, Hoet F, Beltrán-Corbellini Á, Santana-Moreno D, Edelkraut L, Dávila G. Case Report: Barely Able to Speak, Can't Stop Echoing: Echolalic Dynamic Aphasia in Progressive Supranuclear Palsy. Front Aging Neurosci. 2021 May 4;13:635896http://hdl.handle.net/10668/4482The diagnostic criteria for progressive supranuclear palsy (PSP) incorporate two speech-language disturbances (SLDs), non-fluent/agrammatic primary progressive aphasia and progressive apraxia of speech, but overlook the inclusion of other SLDs, including dynamic aphasia (DA). Thus, there is a need to reappraise the broad spectrum of SLDs in PSP to include other presenting phenotypes. Here we report findings from the study of two elderly patients with PSP presenting with DA and irrepressible echolalia. Both patients had markedly impoverished verbal production, but their performance in other tasks (repetition and naming) and auditory comprehension were preserved or only mildly impaired. Experimental tests of DA revealed impaired word and sentence generation in response to verbal and non-verbal stimuli. Additional language and cognitive testing revealed different types of echolalia (mitigated, automatic, and echoing approval) as well as impaired inhibitory control and social cognition (mentalizing). Both patients had negative neuropsychiatric alterations (i.e., apathy, aspontaneity, and indifference/emotional flatness). Brain magnetic resonance imaging in both patients showed atrophy of the midbrain tegmentum and superior medial frontal cortex suggestive of PSP, yet further evaluation of the neural correlates using multimodal neuroimaging and neuropathological data was not performed. However, based on the already known neural basis of DA and echolalia in PSP and stroke, we suggest that, in the present cases, neurodegeneration in the midbrain tegmentum, superior medial frontal lobe, and caudate nucleus was responsible for DA and that decreased activity in these regions may play a permissive role for eliciting verbal echoing via disinhibition of the perisylvian speech-language network.enAtribución 4.0 Internacionalhttp://creativecommons.org/licenses/by/4.0/Dynamic aphasiaEcholaliaProgressive supranuclear palsyPrimary progressive aphasiaInhibition deficitsApathyComprehensionAtrophyAfasiaEcolaliaParálisis supranuclear progresivaAfasia progresiva primariaApatíaComprensiónAtrofiaPatología del habla y lenguajeMedical Subject Headings::Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::HumansMedical Subject Headings::Persons::Persons::Age Groups::Adult::AgedMedical Subject Headings::Diseases::Nervous System Diseases::Central Nervous System Diseases::Brain Diseases::Basal Ganglia Diseases::Supranuclear Palsy, ProgressiveMedical Subject Headings::Psychiatry and Psychology::Behavior and Behavior Mechanisms::Behavior::Communication::Verbal Behavior::SpeechMedical Subject Headings::Diseases::Nervous System Diseases::Neurologic Manifestations::Neurobehavioral Manifestations::Communication Disorders::Language Disorders::Speech Disorders::EcholaliaMedical Subject Headings::Psychiatry and Psychology::Behavior and Behavior Mechanisms::Emotions::ApathyMedical Subject Headings::Anatomy::Nervous System::Central Nervous System::Brain::Prosencephalon::Telencephalon::Cerebrum::Basal Ganglia::Corpus Striatum::Neostriatum::Caudate NucleusMedical Subject Headings::Psychiatry and Psychology::Psychological Phenomena and Processes::Mental Processes::Cognition::ComprehensionMedical Subject Headings::Psychiatry and Psychology::Behavioral Disciplines and Activities::Psychological Tests::Neuropsychological TestsMedical Subject Headings::Diseases::Pathological Conditions, Signs and Symptoms::Signs and Symptoms::Neurologic Manifestations::Neurobehavioral Manifestations::Communication Disorders::Language Disorders::Speech Disorders::AphasiaMedical Subject Headings::Diseases::Pathological Conditions, Signs and Symptoms::Signs and Symptoms::Neurologic Manifestations::Neurobehavioral Manifestations::Communication Disorders::Language Disorders::Speech Disorders::Aphasia::Aphasia, Primary ProgressiveMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Diagnostic Techniques and Procedures::Diagnostic Imaging::Magnetic Resonance ImagingMedical Subject Headings::Anatomy::Nervous System::Central Nervous System::Brain::Prosencephalon::Telencephalon::Cerebrum::Cerebral Cortex::Frontal LobeMedical Subject Headings::Diseases::Pathological Conditions, Signs and Symptoms::Pathological Conditions, Anatomical::AtrophyMedical Subject Headings::Anatomy::Nervous System::Central Nervous System::Brain::Brain Stem::Mesencephalon::Tegmentum MesencephaliMedical Subject Headings::Phenomena and Processes::Genetic Phenomena::PhenotypeMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Diagnostic Techniques and Procedures::Diagnostic Imaging::NeuroimagingMedical Subject Headings::Diseases::Nervous System Diseases::Neurologic Manifestations::Neurobehavioral Manifestations::Psychomotor Disorders::ApraxiasMedical Subject Headings::Disciplines and Occupations::Health Occupations::Allied Health Occupations::Speech-Language Pathologyresearch article34017242Acceso abierto10.3389/fnagi.2021.6358961663-4365PMC8129544