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PubMed1989

Three variant forms of subcortical aphasia in Chinese stroke patients.

Five right-handed patients with subcortical aphasia that involved the left hemisphere subcortical lesion sites were subjected to CT scans. Given their etiology, two cases were infarctions and the other three were hemorrhages. Two of the patients presented an involvement of the anterior limb of the internal capsule and of the basal ganglia and an anterior superior white-matter lesion extension. In both cases slow scanty dysarthric speech was noted; one had markedly impaired auditory comprehension, and the others were only partially impaired. The third patient presented an involvement of the posterior limb of the internal capsule and of the thalamus and a posterior paraventricular white-matter lesion extension. He had poor auditory comprehension, echolalia, and fluent speech. The last two patients presented an involvement of the internal capsule, the basal ganglia, and the thalamus and an anterior posterior paraventricular white-matter lesion extension. The latter two showed poor auditory comprehension with nonfluent and scanty spontaneous speech. The speech sounds were nonsensical monosyllabic words with a pattern similar to that of global aphasia. All patients had lasting right hemiplegia.

Aged↗

PubMed1983

Autistic children's hand preferences: results from an epidemiological study of infantile autism.

Twenty-six autistic children, constituting a total population sample of children diagnosed in accordance with Rutter's criteria as suffering from infantile autism, were assessed with regard to handedness and certain associated factors. They were compared with 52 age-, sex-, and IQ-matched controls. Sixty-two percent of the autistic children were non-right-handed compared with 37% of the controls. Left-handedness in autism was associated with an abundance of delayed echolalia. Heredity for left-handedness in some cases, and assumed brain damage and immature patterns of lateralization in others, were considered the cause of non-right-handedness in the autistic children. Computed tomographic (CT) brain scans and other neurobiological examinations did not provide evidence indicating clear-cut unilateral left hemisphere dysfunction in autism. Rather, a slight trend in the opposite direction (i.e., an association with right hemisphere dysfunction) was seen in the left-handed autistic children. The result points toward the need for further studies of handedness in autism.

Adolescent↗

PubMed1987

Frontal lobe degeneration of non-Alzheimer type. II. Clinical picture and differential diagnosis.

In a longitudinal prospective study of dementia, 158 patients were investigated post mortem. Sixteen patients were classified as frontal lobe dementia (FLD) of non-Alzheimer type and four cases as Pick's disease. Positive heredity for dementia was reported in 50% of these cases compared to 30% in a reference group of patients with Alzheimer's disease (AD). The typical clinical picture in FLD and Pick's disease was that of a slowly progressive dementia, at an early stage dominated by personality change, lack of insight, disinhibition, and later on stereotypy and increased apathy. There was also a progressive dynamic aphasia ending in mutism and amimia. Memory and spatial functions were comparatively spared. Disinhibition, oral/dietary hyperactivity, and echolalia were more consistently found in Pick's disease compared to FLD. The differential diagnosis against AD, cerebrovascular dementia, and other degenerative dementias and against affective disorders and psychotic reactions are discussed.

Adult↗

PubMed1988

Training social skills to severely mentally retarded multiply handicapped adolescents.

Three severely mentally retarded, multiply handicapped, adolescents were treated in a classroom setting for social skills deficits. Two of these children exhibited symptoms of autism including periods of echolalia, and fascination with tactile and visual stimulation. One of the pair was deaf. The third child was profoundly mentally retarded and had minimal expressive language skills. All had received sign language training to facilitate communication. Treatment focused on increasing the frequency of eye contact, in seat and response to verbal prompt behaviors, skills deemed necessary to facilitate use of sign language communication and to increase social interaction. Baseline and treatment were evaluated in a multiple baseline, alternating treatment design across children. Baseline was taken on responses to 10 standard questions, asked by the teacher, based on verbal presentation and sign language. This same procedure was then continued during the initial treatment phase following training sessions. During training, the children received social reinforcement, performance feedback and edible reinforcement, in the form of candy, for appropriate performance. Physical and verbal prompts as well as pictorial cues were employed to shape appropriate behavior. In the second treatment phase, training was implemented in the classroom in which baseline data had been collected. Improvement in target behaviors, via training sessions held four days a week, was noted. These data suggest that use of a combination of visual stimuli, operant and social learning methods can remediate social skills deficits in children with multiple psychological and physical deficits. The implications of these findings for current and future research are discussed.

Achievement↗

PubMed1988

Mixed transcortical aphasia in nonfamilial dysphasic dementia.

The neurolinguistic and cognitive profiles of a patient with mixed transcortical aphasia and non-familial dysphasic dementia associated with progressive, left perisylvian involution are presented. This clinicopathological entity has recently been shown to be an example of a novel class of intrinsic, focal cortical degenerations with sparing of the basal forebrain. The characteristics of the aphasia were unusual. There were occasional literal and verbal paraphasic errors, but no completion phenomenon, embellishment or significant echolalia. Evidence of a generalized lexical disruption was found on detailed analysis of residual abilities in reading, writing and spelling. A newly described form of non-lexical repetition was present, resulting in the patient's inability to correct syntactical and semantic errors. Affective prosodic repetition was intact. A combined paraphasic word-production and semantic anomia was found. These observations combined with evidence of evolving region-specific cognitive impairments suggest that the pathological process resulted in a relative disconnection of integral frontal and parieto-occipital areas from adjacent perisylvian language zones.

Agnosia↗

PubMed2006

[Behavior disturbances in emergency psychiatry or fronto-temporal dementia diagnosis? A challenge for psychiatrists].

INTRODUCTION: The diagnosis of fronto-temporal dementia (FTD) represents a challenge for the psychiatrist, especially since this insidious pathology partly mimics other psychiatric diseases. CASE-REPORT: We present a clinical case that illustrates the difficulty of FTD diagnosis particularly well. A 32 year-old woman without previous medical history presented with psychomotor agitation, logorrhea and flight of ideas. The criteria for bipolar disorder according to DSM IV were met and the patient was referred to an outpatient clinic where a mood stabilizer was initiated (lithium, 400 mg/day). An in-depth interview with her husband revealed mild but progressive modification of her personality and behavior over the course of two years. She showed signs of mild fatigue and irritability that evolved into a loss of interest for both leisure and domestic activities. In addition, she showed increasing erratic behavior and emergence of frequent episodes of verbal abuse. After the birth of her second child, the patient's clinical state worsened with the appearance of uninhibited behavior, loss of personal hygiene, sleep disturbances and nightmares. The patient was forced to stop her work as a cleaner, a steady employment that she had maintained for 10 years. The hypomanic state worsened and psychotic symptoms such as delusions and echolalia appeared within a few weeks. These events culminated in a first hospitalization in a psychiatric unit. We evoked both diagnoses of schizoaffective disorder and psychotic disorder not otherwise specified. In the following months, we conducted neurological examinations on account of the progressive deterioration of her cognitive functions. Neuroradiological results (CT scan with contrast agents, MRI, cerebral scintigraphy) coupled with her clinical evolution (neurological examination and neuropsychological testing) permitted diagnosis of fronto-temporal dementia. DISCUSSION: Fronto-temporal dementia usually presents itself as an autosomal dominant disease in 89% of reported cases, with an insidious onset associated with thymic symptoms and behavioral disturbances. The first consultation often concludes with a suspicion of a psychiatric disorder in 33% of the cases (unipolar and bipolar depressive disorders, psychotic disorders, alcohol dependence). The clinical description of such a heterogenic and neuro-psychiatric disorder should be widely disseminated, so that psychiatrists can distinguish early symptoms and diminish the risk of misdiagnosis of FDT. CONCLUSION: Such case reports emphasize the importance for psychiatrists to be aware of the clinical prodromal FDT symptoms, particularly since the neuro-imaging data of dementia are often delayed.

Adult↗

PubMed2001

Corticobasal degeneration presenting with nonfluent primary progressive aphasia: a clinicopathological study.

A 62-year-old woman initially presented with slowly progressive nonfluent aphasia with minimal intellectual involvement. Echolalia and personality change were prominent whereas parkinsonian features and signs suggesting parietal lobe dysfunctions were not present. The patient's language deficit was consistent with transcortical motor aphasia. She did not manifest extrapyramidal signs. The patient was diagnosed as having Pick's disease or frontal lobe dementia. She died at age 65, 2 years and 9 months following disease onset. Neuropathological findings including cytoskeletal abnormalities, however, were clearly distinct from those of classical Pick's disease and were consistent with those reported in corticobasal degeneration (CBD). The distribution of her cortical lesions was accentuated in the frontal language-related area. The clinical manifestations in CBD are diverse, and primary progressive nonfluent aphasia should be considered as an initial symptom of CBD. Neuropathological examination of such patients should include cytoskeletal abnormality studies.

Aphasia, Primary Progressive↗

PubMed2001

Imitation, mirror neurons and autism.

Various deficits in the cognitive functioning of people with autism have been documented in recent years but these provide only partial explanations for the condition. We focus instead on an imitative disturbance involving difficulties both in copying actions and in inhibiting more stereotyped mimicking, such as echolalia. A candidate for the neural basis of this disturbance may be found in a recently discovered class of neurons in frontal cortex, 'mirror neurons' (MNs). These neurons show activity in relation both to specific actions performed by self and matching actions performed by others, providing a potential bridge between minds. MN systems exist in primates without imitative and 'theory of mind' abilities and we suggest that in order for them to have become utilized to perform social cognitive functions, sophisticated cortical neuronal systems have evolved in which MNs function as key elements. Early developmental failures of MN systems are likely to result in a consequent cascade of developmental impairments characterised by the clinical syndrome of autism.

Autistic Disorder↗

PubMed2002

Comorbidity of Tourette's syndrome and schizophrenia--biological and physiological parallels.

The authors report on five patients who first developed Tourette's syndrome (TS) and later schizophrenia with the typical positive and negative symptoms; all five had an unfavorable course of schizophrenia. These observations as well as other reported cases raise the question of whether both disorders may share a common background. This is discussed under the aspects of similar symptomatology (echolalia, motor symptoms, cognitive deficits, obsessive-compulsive symptoms), similar pathophysiological signs, genetics and signs of an underlying inflammatory process in subgroups of cases, as well as common therapeutic strategies. A genetically determined susceptibility could possibly underlie both disorders, e.g., an autoimmunologically triggered inflammation or a common pathophysiology of certain symptoms. Both disorders show disturbances of the multiple functional pathways, which seem to be involved in the pathophysiology of both. The clinical overlap of TS and of schizophrenia may be due to a final common pathophysiological pathway.

Adult↗

PubMed2003

Update on the language disorders of individuals on the autistic spectrum.

Inadequate language is a defining feature of the autism spectrum disorders (autism). Autism is a behaviorally and dimensionally defined developmental disorder of the immature brain that has a broad range of severity and many etiologies, with multiple genes involved. Early studies, which focused on the language of verbal children on the autistic spectrum, emphasized aberrant features of their speech such as unusual word choices, pronoun reversal, echolalia, incoherent discourse, unresponsiveness to questions, aberrant prosody, and lack of drive to communicate. Persistent lack of speech of some individuals was attributed to the severity of their autism and attendant mental retardation rather than possible inability to decode auditory language. Clinical study of unselected children with autism indicated that the language deficits of preschoolers fall into two broad types, perhaps with subtypes, those that involve reception and production of phonology (sounds of speech) and syntax (grammar), and those that do not but involve semantics (meaning) and pragmatics (communicative use of language, processing, and production of discourse). Except for the preschoolers' universally deficient pragmatics and comprehension of speech, many of their language deficits parallel those of non-autistic preschoolers with developmental language disorders. There is now biological support for the clinical observation that young autistic children are language disordered as well as autistic. Recent electrophysiological studies disclose auditory input abnormalities in lateral temporal cortex even in verbal individuals on the autistic spectrum. Severe receptive deficits for phonology enhance the risk for epilepsy. Genetic studies indicate that linkage to chromosome 7q31-33 is limited to families with evidence for phonologic impairment as well as autism. Clearly, social and cognitive disorders alone provide an inadequate explanation for the range of language deficits in autism.

Auditory Perception↗

PubMed1999

Epilepsy surgery in children with pervasive developmental disorder.

Pervasive developmental disorder (PDD) is occasionally associated with medically intractable complex partial seizures. The outcome of PDD was explored in three males and two females who underwent epilepsy surgery at 32 months to 8 years of age (mean = 4 years) after onset of epilepsy at 1 week to 21 months of age (mean = 11 months). Four children had temporal lobe resections (three right, one left; two for focal cortical dysplasia, and two for tumors), and one had a right temporoparieto-occipital resection (for focal cortical dysplasia). Each child underwent repeated evaluations by a pediatric neuropsychologist and psychiatrist. Fourteen to 47 months (mean = 23 months) after operation, one child with persistent seizures had moderate developmental and behavioral improvement, three children (two seizure free, one with rare staring spells) had mild developmental and behavioral improvement, and the remaining child (seizure free) experienced a worsening of her PDD. The four children with mild-to-moderate improvement in postoperative cognitive and behavioral development still demonstrated persistent delay. Cognitive gains were confirmed by neuropsychologic testing in the oldest patient but were not reflected in test results from the three younger children, who had more modest improvement. The child with worsening of her PDD had cognitive and emotional deterioration to babbling, echolalia, aggressiveness, decreased social interaction, and increased mouthing of objects beginning several months postoperatively. These results suggest that families should be counseled that PDD symptoms in children with focal epileptogenic lesions may or may not improve after epilepsy surgery, even if the surgery is successful with respect to seizure control.

Child↗

PubMed1997

Language disorders in children with autism.

Language development is delayed in most children on the autistic spectrum. The children are dysphasic as well as autistic. Comprehension and pragmatics are invariably affected. Lower level mixed receptive/expressive disorders involve phonological and syntactical processing, whereas higher level processing disorders involve semantics and formulation of discourse. In some children, lower level disorders may be so severe as to preclude speech, whereas in others phonology may be deficient in spontaneous production but not in repetition. Abnormal features of autistic language include aberrant prosody, immediate and delayed echolalia (scripts), and perseveration. Electrophysiological studies indicate that brainstem-evoked potentials are normal. Even in fully verbal individuals with autism, early and late cortical components of auditory, but not visual, event-related potentials are abnormal. Appropriate intervention must address language and behavioral issues. In children with severely defective auditory language, provision of visual language to supplement speech is essential.

Autistic Disorder↗

PubMed2001

Behaviour in frontotemporal dementia, Alzheimer's disease and vascular dementia.

OBJECTIVES: The study aimed to increase understanding of behavioural changes in frontotemporal dementia (FTD) and identify features that best differentiate FTD from Alzheimer's disease (AD) and cerebrovascular dementia (CvD). METHODS: A semi-structured questionnaire was administered to carers of 30 FTD, 75 AD and 34 CvD patients. RESULTS: Behavioural changes that strongly discriminated FTD from AD and to a lesser extent CvD were loss of emotions and insight, selfishness, disinhibition, personal neglect, gluttony and sweet food preference, wandering, motor and verbal stereotypies, loss of pain, echolalia and mutism. Irritability, hyposexuality and hypersomnia did not discriminate. Emotional, eating and stereotyped behaviours correctly classified 95% of patients using regression analysis. CONCLUSIONS: Behavioural characteristics accurately differentiate FTD from AD and CvD. The findings highlight the particular importance of affective change in FTD, and underline the role of the frontotemporal lobes in emotion.

Adult↗

PubMed1976

A therapeutic milieu for establishing and expanding communicative behaviors in psychotic children.

This study describes a clinical intervention program that was used to facilitate development of receptive and expressive language skills in a group of four psychotic children. A group format utilizing an interactive language development teaching procedure combined with a therapeutic milieu was shown to be effective in establishing and expanding communicative behaviors in psychotic children. Nine diagnostic measures were utilized to assess children's performance prior to therapy and at the end of three-month and six-month intervals. Results indicated substantial reduction of delayed and immediate echolalia, jargon, inappropriate stress, pitch, and intonation. Substantial improvement was noted in expressive syntactic and morphological functioning and in the children's ability to generate novel utterances about day-to-day experiences, family, and toys. Increases in children's concept of body image and ego functioning paralleled their improvement in receptive and expressive language development.

Body Image↗

PubMed1998

A five-year follow-up study of older long-stay clients with intellectual disability using the Disability Assessment Schedule.

The emphasis on community care means closure of the long-stay institutions for people with intellectual disability. Studies have indicated that older people with intellectual disability in particular may not be adequately cared for because of poor monitoring of their changing needs and inadequate provision of services. The use of rating instruments to monitor changes, and to predict outcome or needs in this population may help to improve care by assisting with planning and projection of service requirements. In 1991, all residents of a long-stay hospital for people with intellectual disability were assessed using the Disability Assessment Schedule (DAS). Five years later, the 1991 scores of the older residents (aged > 50 years) were reviewed and compared under three outcome groups: in-patients, discharged and deceased. Furthermore, all older people resident in the hospital in 1996 were reassessed using the DAS. Out of the 144 older clients resident in 1991, five years later, 78 were still in-patients, 38 had been discharged into the community and 28 were deceased. In 1991, the decreased group had the greatest problems with continence and symbolic behaviour, while the discharged group had the greatest problems with self-help, vision, hearing, communication, social interaction, echolalia and repetitive speech. In comparison with 1991, the 1996 DAS scores of older residents showed that there were increasing problems with vision, hearing, communication, behaviour and symbolic activities. The present study suggested that the DAS is a useful instrument for monitoring change and predicting outcome in older people with intellectual disability.

Activities of Daily Living↗

PubMed1999

Frontal type dementia preceding amyotrophic lateral sclerosis: a neuropsychological and SPECT study of five clinical cases.

Between 1993 and 1995, we observed five sporadic cases of frontotemporal dementia (FTD) which in all cases preceded the appearance of typical amyotrophic lateral sclerosis (ALS). The FTD rapidly became severe (within 12-18 months) and the delay between the presumed onset of mental change and ALS was short (12-26 months). The frontal dysfunction was characteristic (disinhibited, jocular, impatient, gluttonous, stereotypical gestures). The language impairment (less talkative, persistent errors, fantastic and semantic paraphasia, neologistic errors, echolalia) was constant. Single photon emission computed tomography (SPECT) with 99 Tcm HMPAO (hexamethyl propylamine oxime) was done at the same time as neuropsychological testing in four cases and showed serious diffuse bifrontal defect, sometimes with less serious internal temporal hypofixation. All patients died with bulbar ALS complications. The total course can last from 14-48 months. Most of the reported cases suggested a relationship between dementia-ALS and frontal dysfunction. The mechanism underlying dementia-ALS remains to be solved. Our five cases resemble those reported by Mitsuyama (1993), who suggested that dementia-ALS has the same clinicopathological entity.

Aged↗

PubMed2002

Postictal mixed transcortical aphasia.

Postictal aphasia has been described in left temporal lobe seizures. It may be of fluent, non-fluent or global type. We present here a patient who displayed signs of mixed transcortical aphasia (MTCA). The patient was a 67 year old man who underwent excision of a left frontal parasagittal meningioma in 1987. Since then he has been treated with phenytoin for generalized tonic-clonic seizures (GTCS). He was admitted in status epilepticus. On awakening, the patient was non-fluent with palilalia and echolalia. His repetition was relatively preserved but all the other language functions were impaired. This picture faded away within a few hours. Brain CT, performed during this postictal state, was normal except for signs related to frontal craniotomy. SPECT, which was performed after language functions returned to normal, displayed left frontal, cingular and insular hypoperfusion. The postictal language dysfunction of the patient corresponded to MTCA. Although our case has frontal, he had no other structural lesion that could explain either diffuse ischemia of the left hemisphere or watershed areas secondary to the generalized seizures. The uniqueness of this case is the combination of postictal MTCA with good prognosis.

Aged↗

PubMed2001

Application of genomeceuticals to the molecular and immunological aspects of autism.

Autism is a developmental disease affecting as many as 1 in 300 children and is often characterized as a mental disorder originating in infancy that is associated with self-absorption, inability to interact socially, behavior, and language dysfunction (e.g. echolalia). Current theories indicate an important role of diet in the development of disease. It is thought that, as a result of maldigestion of casein and gluten, opioid-type peptides, or exorphins, are produced. Additionally, because of the time-frame of development of the disease, there has been an association with childhood vaccination. Consequently, prevailing therapies attempt to address these causes in one, or a combination, of three ways: diet restriction (removing casein and gluten); supplementation with exogenous enzymes; and probiotic bacteria. Until recently, none of the therapies addressed the molecular mechanisms that may be at work in the development and progression of autism. This paper presents potential molecular and cellular mechanism related to autism as well as discusses their application to the treatment of the disease through the application of genomeceuticals. Additionally, a link between developmentally associated aberrant immune and inflammatory responses, and autism is suggested and explored.

Autistic Disorder↗