Case Report: Barely Able to Speak, Can't Stop Echoing: Echolalic Dynamic Aphasia in Progressive Supranuclear Palsy.

The 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.

Emergency department visits for transient global amnesia alone or in association with substance abuse. / Amnesia global transitoria y consumo de tóxicos que motivan la consulta al servicio de urgencias.

OBJECTIVES: To study the epidemiology of emergency department visits for transient global amnesia (TGA) by itself or associated with substance abuse or sexual assault. MATERIAL AND METHODS: Retrospective study of cases treated from January to December 2018. Data for all patients with TGA were extracted, and cases were classified as associated with substance abuse (TGASUB), sexual assault (TGASEX), or neither (TGAONLY). RESULTS: A total of 287 TGA cases were found: 169 (58.9%) were TGASEX, 62 (21.6%) TGAONLY, and 56 (19.5%) TGASUB. Two hundred eighteen (76%) were female and 69 (24%) were male. Ages ranged from 16 to 90 years; 174 (60.6%) were under the age of 30 years. Two hundred one patients (72.8%) reported consuming alcohol; and 105 (49.1%) were positive on testing (mean blood alcohol concentration, 0.74 g/L; maximum, 3.9 g/L. Twenty patients (7.1%) reported using cannabis, and 39 (17.3%) had positive test results; 14 reported using cocaine (4.9%) and 28 (12.4%) tested positive; 5 (1.7%) reported using amphetamines and 20 (8.8%) tested positive. Fifty-eight (20.1%) had symptoms of intoxication. Four were admitted in coma. A computed tomography scan was ordered for 66 patients (23%), 7 patients were hospitalized, and none died. CONCLUSION: The prevalence of TGA is higher if cases of substance abuse and sexual assault are counted. Toxicolgy testing changes the epidemiology of TGA in emergencies.

OBJETIVO: Conocer la epidemiología de las consultas en urgencias por amnesia global transitoria (AGT), ya sea pura, asociada al consumo de tóxicos o en el contexto de una agresión sexual. METODO: Estudio retrospectivo de enero a diciembre de 2018. Se revisaron las AGT atendidas en intoxicados (AGTtox), en víctimas de agresiones sexuales (AGTsex) y las amnesias puras (AGTpur), evaluando la presencia de tóxicos. RESULTADOS: Se identificaron 287 AGT: 169 AGTsex (58,9%), 62 AGTpur (21,6%) y 56 AGTtox (19,5%). De ellas, 218 (76%) fueron mujeres y la edad osciló entre 16 y 90 años (60,6% menores de 30 años). Reconocieron consumo de alcohol 201 casos (72,8%), con etanolemia positiva en 105 (49,1%) (media de 0,74 g/l y máxima de 3,9 g/l). Admitieron consumo de cannabis 20 pacientes (7,1%), con analítica positiva en 39 casos (17,3%); cocaína 14 (4,9%), con analítica positiva en 28 (12,4%), y anfetaminas 5 (1,7%), con analítica positiva en 20 (8,8%). Presentaron síntomas de intoxicación 58 casos (20,1%). Cuatro pacientes ingresaron en coma. Se realizó una tomografía computarizada (TC) craneal a 66 pacientes (23%), se hospitalizaron 7 y no hubo ningún fallecimiento. CONCLUSIONES: La prevalencia de AGT es mayor si se incluyen los intoxicados y las agresiones sexuales, modificando la determinación de tóxicos la epidemiología de la AGT en urgencias.

Loss of regional accent after damage to the speech production network.

Lesion-symptom mapping studies reveal that selective damage to one or more components of the speech production network can be associated with foreign accent syndrome, changes in regional accent (e.g., from Parisian accent to Alsatian accent), stronger regional accent, or re-emergence of a previously learned and dormant regional accent. Here, we report loss of regional accent after rapidly regressive Broca's aphasia in three Argentinean patients who had suffered unilateral or bilateral focal lesions in components of the speech production network. All patients were monolingual speakers with three different native Spanish accents (Cordobés or central, Guaranítico or northeast, and Bonaerense). Samples of speech production from the patient with native Córdoba accent were compared with previous recordings of his voice, whereas data from the patient with native Guaranítico accent were compared with speech samples from one healthy control matched for age, gender, and native accent. Speech samples from the patient with native Buenos Aires's accent were compared with data obtained from four healthy control subjects with the same accent. Analysis of speech production revealed discrete slowing in speech rate, inappropriate long pauses, and monotonous intonation. Phonemic production remained similar to those of healthy Spanish speakers, but phonetic variants peculiar to each accent (e.g., intervocalic aspiration of /s/ in Córdoba accent) were absent. While basic normal prosodic features of Spanish prosody were preserved, features intrinsic to melody of certain geographical areas (e.g., rising end F0 excursion in declarative sentences intoned with Córdoba accent) were absent. All patients were also unable to produce sentences with different emotional prosody. Brain imaging disclosed focal left hemisphere lesions involving the middle part of the motor cortex, the post-central cortex, the posterior inferior and/or middle frontal cortices, insula, anterior putamen and supplementary motor area. Our findings suggest that lesions affecting the middle part of the left motor cortex and other components of the speech production network disrupt neural processes involved in the production of regional accent features.