The Influence of Language Context on Repetitive Speech Use in Children With Autism Spectrum Disorder.

Purpose Although repetitive speech is a hallmark characteristic of autism spectrum disorder (ASD), the contributing factors that influence repetitive speech use remain unknown. The purpose of this exploratory study was to determine if the language context impacts the amount and type of repetitive speech produced by children with ASD. Method As part of a broader word-learning study, 11 school-age children with ASD participated in two different language contexts: storytelling and play. Previously collected language samples were transcribed and coded for four types of repetitive speech: immediate echolalia, delayed echolalia, verbal stereotypy, and vocal stereotypy. The rates and proportions of repetitive speech were compared across the two language contexts using Wilcoxon signed-ranks tests. Individual characteristics were further explored using Spearman correlations. Results The children produced lower rates of repetitive speech during the storytelling context than the play-based context. Only immediate echolalia differed between the two contexts based on rate and approached significance based on proportion, with more immediate echolalia produced in the play-based context than in the storytelling context. There were no significant correlations between repetitive speech and measures of social responsiveness, expressive or receptive vocabulary, or nonverbal intelligence. Conclusions The children with ASD produced less immediate echolalia in the storytelling context than in the play-based context. Immediate echolalia use was not related to social skills, vocabulary, or nonverbal IQ scores. These findings offer valuable insights into better understanding repetitive speech use in children with ASD.

Association between speech-language, general cognitive functioning and behaviour problems in individuals with Williams syndrome.

BACKGROUND: Williams syndrome (WS) phenotype is described as unique and intriguing. The aim of this study was to investigate the associations between speech-language abilities, general cognitive functioning and behavioural problems in individuals with WS, considering age effects and speech-language characteristics of WS sub-groups. METHODS: The study's participants were 26 individuals with WS and their parents. General cognitive functioning was assessed with the Wechsler Intelligence Scale. Peabody Picture Vocabulary Test, Token Test and the Cookie Theft Picture test were used as speech-language measures. Five speech-language characteristics were evaluated from a 30-min conversation (clichés, echolalia, perseverative speech, exaggerated prosody and monotone intonation). The Child Behaviour Checklist (CBCL 6-18) was used to assess behavioural problems. RESULTS: Higher single-word receptive vocabulary and narrative vocabulary were negatively associated with CBCL T-scores for Social Problems, Aggressive Behaviour and Total Problems. Speech rate was negatively associated with the CBCL Withdrawn/Depressed T-score. Monotone intonation was associated with shy behaviour, as well as exaggerated prosody with talkative behaviour. WS with perseverative speech and exaggerated prosody presented higher scores on Thought Problems. Echolalia was significantly associated with lower Verbal IQ. No significant association was found between IQ and behaviour problems. Age-associated effects were observed only for the Aggressive Behaviour scale. CONCLUSIONS: Associations reported in the present study may represent an insightful background for future predictive studies of speech-language, cognition and behaviour problems in WS.

The pathophysiology of echopraxia/echolalia: relevance to Gilles de la Tourette syndrome.

Echopraxia and echolalia are subsets of imitative behavior. They are essential developmental elements in social learning. Their persistence or reemergence after a certain age, though, can be a sign of underlying brain dysfunction. Although echophenomena have been acknowledged as a typical sign in Gilles de la Tourette syndrome (GTS) since its first description, their clinical significance and neural correlates are largely unknown. Here, we review the course of their scientific historical development and focus on their clinical phenomenology and differential diagnosis with a particular view to GTS. The neural basis of echophenomena will also be addressed. © 2012 Movement Disorder Society.

Ambient echolalia in a patient with germinoma around the bilateral ventriculus lateralis: a case report.

Ambient echolalia is a rare condition with few reported cases. We report the case of a 20-year-old man with a germinoma around the bilateral ventriculus lateralis who exhibited ambient echolalia. Clinical features included instinctive grasp reaction and compulsive manipulation of tools in his right hand. Speech or mental deterioration has been cited as a cause of ambient echolalia, but neither dementia nor aphasia was present. We propose that ambient echolalia in our case could be interpreted as a disinhibition of pre-existing essentially intact motor subroutines due to damage of the medial frontal lobe.

Echolalia or functional repetition in conversation--a case study of an individual with Huntington's disease.

PURPOSE: In this case study, we investigated the use of repetition in an individual with a neurogenic communication disorder. METHOD: We present an analysis of interaction in natural conversations between a woman with advanced Huntington's disease (HD), whose speech had been described as sometimes characterised by echolalia, and her personal assistant. The conversational interaction is analysed on a sequential level, and recurrent patterns are explored. RESULTS: Although the ability of the person with HD to interact is affected by chorea, word retrieval problems and reduced comprehension, she takes an active part in conversation. The conversational partner's contributions are often adapted to her communicative ability as they are formulated as questions or suggestions that can be elaborated on or responded to with a simple 'yes' or 'no'. The person with HD often repeats the words of her conversational partner in a way that extends her contributions and shows listenership, and this use of repetition is also frequent in ordinary conversations between non-brain-damaged individuals. CONCLUSIONS: The results show that the conversation partners in this case cooperate in making the conversation proceed and evolve, and that verbal repetition is used in a way that works as a strategy for compensating for the impairment.

Verbal repetitions and echolalia in Alzheimer's discourse.

This article reports on an investigation of echolalic repetition in Alzheimer's disease (AD). A qualitative analysis of data from spontaneous conversations with MHI, a woman with AD, is presented. The data come from the DALI Corpus, a corpus of spontaneous conversations involving subjects with AD. This study argues that echolalic effects can be explained through an analysis of their formal linguistic aspects, such as intonational-prosodic and enunciative-syntactic features. The analysis shows that the description of echolalic repetitions in these terms can help find parameters for the description of the linguistic and communicative characteristics of AD. This analysis even shows how a previous speech turn serves as a base for the elaboration of the next turn by the participant with AD. It also contributes to the understanding of echolalic productions in AD.

Hyperlexia and ambient echolalia in a case of cerebral infarction of the left anterior cingulate cortex and corpus callosum.

We report the case of a 69-year-old woman with cerebral infarction in the left anterior cingulate cortex and corpus callosum. She showed hyperlexia, which was a distinctive reading phenomenon, as well as ambient echolalia. Clinical features also included complex disorders such as visual groping, compulsive manipulation of tools, and callosal disconnection syndrome. She read words written on the cover of a book and repeated words emanating from unrelated conversations around her or from hospital announcements. The combination of these two features due to a focal lesion has never been reported previously. The supplementary motor area may control the execution of established subroutines according to external and internal inputs. Hyperlexia as well as the compulsive manipulation of tools could be interpreted as faulty inhibition of preexisting essentially intact motor subroutines by damage to the anterior cingulate cortex reciprocally interconnected with the supplementary motor area.

Abnormal laughter-like vocalisations replacing speech in primary progressive aphasia.

We describe ten patients with a clinical diagnosis of primary progressive aphasia (PPA) (pathologically confirmed in three cases) who developed abnormal laughter-like vocalisations in the context of progressive speech output impairment leading to mutism. Failure of speech output was accompanied by increasing frequency of the abnormal vocalisations until ultimately they constituted the patient's only extended utterance. The laughter-like vocalisations did not show contextual sensitivity but occurred as an automatic vocal output that replaced speech. Acoustic analysis of the vocalisations in two patients revealed abnormal motor features including variable note duration and inter-note interval, loss of temporal symmetry of laugh notes and loss of the normal decrescendo. Abnormal laughter-like vocalisations may be a hallmark of a subgroup in the PPA spectrum with impaired control and production of nonverbal vocal behaviour due to disruption of fronto-temporal networks mediating vocalisation.

Interpreting problematic behavior: systematic compensatory adaptations as emergent phenomena in autism.

Based upon an emergent account of pragmatic ability and disability, this article provides theoretical and empirical support for a conceptually deeper understanding of some systematic behaviors that have served as diagnostic indices in communicatively impaired populations. Specifically, by employing conversation analysis, several examples of problematic behaviors in autism are analysed as a specific type of compensatory adaptation. Theoretical and clinical implications are discussed.

The interactional significance of formulas in autistic language.

The phenomenon of echolalia in autistic language is well documented. Whilst much early research dismissed echolalia as merely an indicator of cognitive limitation, later work identified particular discourse functions of echolalic utterances. The work reported here extends the study of the interactional significance of echolalia to formulaic utterances. Audio and video recordings of conversations between the first author and two research participants were transcribed and analysed according to a Conversation Analysis framework and a multi-layered linguistic framework. Formulaic language was found to have predictable interactional significance within the language of an individual with autism, and the generic phenomenon of formulaicity in company with predictable discourse function was seen to hold across the research participants, regardless of cognitive ability. The implications of formulaicity in autistic language for acquisition and processing mechanisms are discussed.

[A case of crossed aphasia with echolalia after the resection of tumor in the right medial frontal lobe].

We report a right-handed woman, who developed a non-fluent aphasia after resection of astrocytoma (grade III) in the right medial frontal lobe. On admission to the rehabilitation department, neurological examination revealed mild left hemiparesis, hyperreflexia on the left side and grasp reflex on the left hand. Neuropsychologically she showed general inattention, non-fluent aphasia, acalculia, constructional disability, and mild buccofacial apraxia. No other apraxia, unilateral spatial neglect or extinction phenomena were observed. An MRI demonstrated resected areas in the right superior frontal gyrus, subcortical region in the right middle frontal gyrus, anterior part of the cingulate gyrus, a part of supplementary motor area. Surrounding area in the right frontal lobe showed diffuse signal change. She demonstrated non-fluent aprosodic speech with word finding difficulty. No phonemic paraphasia, or anarthria was observed. Auditory comprehension was fair with some difficulty in comprehending complex commands. Naming was good, but verbal fluency tests for a category or phonemic cuing was severely impaired. She could repeat words but not sentences. Reading comprehension was disturbed by semantic paralexia and writing words was poor for both Kana (syllabogram) and Kanji(logogram) characters. A significant feature of her speech was mitigated echolalia. In both free conversation and examination setting, she often repeated phrases spoken to her which she used to start her speech. In addition, she repeated words spoken to others which were totally irrelevant to her conversation. She was aware of her echoing, which always embarrassed her. She described her echolalic tendency as a great nuisance. However, once echoing being forbidden, she could not initiate her speech and made incorrect responses after long delay. Thus, her compulsive echolalia helped to start her speech. Only four patients with crossed aphasia demonstrated echolalia in the literature. They showed severe aphasia with markedly decreased speech and severe comprehension deficit. A patient with a similar lesion in the right medial frontal lobe had aspontaneity in general and language function per se could not be examined properly. Echolalia related to the medial frontal lesion in the language dominant hemisphere was described as a compulsive speech response, because some other 'echoing' phenomena or compulsive behavior were also observed in these patients. On the other hand, some patients with a large lesion in the right hemisphere tended to respond to stimuli directed to other patients, so called 'response-to-next-patient-stimulation'. This behavior was explained by disinhibited shift of attention or perseveration of the set. Both compulsive speech responses and 'response-to-next-patient-stimulation' like phenomena may have contributed to the echolalia phenomena of the present case.

Echolalia as a novel manifestation of neuropsychiatric systemic lupus erythematosus.

"That tongue of yours, by which I have been tricked, shall have its power curtailed and enjoy the briefest use of speech." With these words, Hera, of Greek mythology, deprived the nymph Echo of spontaneous speech, constraining her instead to merely repeating the words of others. Echolalia, which derives from the word "echo," is disordered speech in which an individual persistently repeats what is heard. Echolalia has been described in patients with a number of neuropsychiatric illnesses including autism and Tourette's syndrome. Neuropsychiatric systemic lupus erythematosus (NPSLE) is a heterogeneous disease with protean manifestations that may occur in approximately 25% to 50% of patients with systemic lupus erythematosus (SLE). Although the most common manifestations include cognitive dysfunction (50%) and seizures (20%), NPSLE may also present as peripheral neuropathy (15%), psychosis (10%), or other central nervous system abnormalities. We report the case of a 57-year-old woman with SLE and echolalia.

[Hyperlexia in an adult patient with lesions in the left medial frontal lobe].

A 69-year-old right-handed woman developed a transcortical motor aphasia with hyperlexia following resection of a glioma in the left medial frontal lobe. Neurological examination revealed grasp reflex in the right hand and underutilization of the right upper extremity. An MRI demonstrated lesions in the left medial frontal lobe including the supplementary motor area and the anterior part of the cingulate gyrus, which extended to the anterior part of the body of corpus callosum. Neuropsychologically she was alert and cooperative. She demonstrated transcortical motor aphasia. Her verbal output began with echolalia. Furthermore hyperlexia was observed in daily activities and during examinations. During conversation she suddenly read words written on objects around her which were totally irrelevant to the talk. When she was walking in the ward with an examiner she read words written on a trash bag that passed by and signboards which indicated a name of a room. Her conversation while walking was intermingled with reading words, which was irrelevant to the conversation. She also read time on analog clocks, which were hung on a wall in a watch store. In a naming task, she read words written on objects first and named them upon repeated question about their names. When an examiner opened a newspaper in front of her without any instructions she began reading until the examiner prohibited it. Then she began reading again when an examiner turned the page, although she remembered that she should not read it aloud. She showed mild ideomotor apraxia of a left hand. Utilization behavior, imitation behavior, hypergraphia, or compulsive use of objects was not observed throughout the course. Hyperlexic tendency is a prominent feature of this patient's language output. Hyperlexia was often reported in children with pervasive developmental disorders including autism. There are only a few reports about hyperlexia in adults and some of them were related to diffuse brain dysfunction. Hyperlexia of our patient was associated with echolalia but not with the other "echo" phenomena, which may be because the lesion was unilateral on the left side. Dysfunction of the left supplementary motor area could lead to disinhibition of regulatory mechanism of verbal output in response to auditory and visual stimuli.

Effortful echolalia.

We report three cases of effortful echolalia in patients with cerebral infarction. The clinical picture of speech disturbance is associated with Type 1 Transcortical Motor Aphasia (TCMA, Goldstein, 1915). The patients always spoke nonfluently with loss of speech initiative, dysarthria, dysprosody, agrammatism, and increased effort and were unable to repeat sentences longer than those containing four or six words. In conversation, they first repeated a few words spoken to them, and then produced self initiated speech. The initial repetition as well as the subsequent self initiated speech, which were realized equally laboriously, can be regarded as mitigated echolalia (Pick, 1924). They were always aware of their own echolalia and tried to control it without effect. These cases demonstrate that neither the ability to repeat nor fluent speech are always necessary for echolalia. The possibility that a lesion in the left medial frontal lobe, including the supplementary motor area, plays an important role in effortful echolalia is discussed.

Environment-driven responses in progressive supranuclear palsy.

The neurological signs and behaviors that accompany degenerative diseases associated with fronto-striatal dysfunction are incompletely described. We observed several novel environmentally-driven behaviors in seven patients with progressive supranuclear palsy (PSP). All patients had cognitive deficits with greatest impairments on tests of frontal lobe function, and frontal lobe cerebral perfusion was significantly reduced in 4 of the 5 who had single photon emission computed tomography (SPECT) brain scans. Visual grasping, in which a patient's gaze was attracted to an incidental object in the environment such as a TV set or mirror, was preeminent. Once fixed, there was inability to release the gaze and shift to another object. In other instances, removing a table placed in front of a patient or unbuckling of his seat belt would make him stand up, which was impossible on command. Similarly, playing music would induce rhythmic foot beating, which was never obtained on command. There were compulsive utilization behaviors, such as repetitively picking up and replacing the telephone for no apparent reason. As expected, there were signs of heightened facial reflexes, grasp reflexes, apraxia of eyelid opening, echolalia and echopraxia. We postulate that these stimuli-oriented behaviors stem from parietal lobe disinhibition due to fronto-striatal dysfunction.