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Cognitive neuropsychology. Resolving enigmas about Wernicke's aphasia and other higher cortical disorders.

Cognitive neuropsychology is a young branch of neuroscience whose ancestral influences include a rich pool of experimental (eg, cognitive, psychology), theoretical (eg, epistemology), and clinical (eg, neurology, neuropsychology) disciplines. An essential principle of cognitive neuropsychology is that disorders of higher cortical functions can be understood in terms of breakdowns of one or more information-processing modules. Each module is the most basic element of intelligence that can be defined based on current knowledge. This approach is a refinement of-not a fundamental departure from-the 19th-century "localizationist" view of language disorders. Wernicke's aphasia, for example, classical attributed to a single cognitive deficit (loss of word sounds), is shown in this review to require damage to multiple distinct information-processing modules. Cognitive neuropsychology provides the tools for the type of fine-grained analyses of behavior that are needed to capitalize on recent advances in neuroimaging techniques, including the development of more sophisticated models of brain-behavior relationships.

Aphasia, Wernicke↗

The biology of developmental dyslexia.

Dyslexia is a relatively common disorder that, when severe, persists into adulthood. New evidence suggests that females are affected nearly as frequently as males. Neuropsychological studies characterize dyslexia as a language disorder that involves phonological deficits in particular. Educational therapies aimed at direct improvement of reading skill constitute the best available treatment. Variable genetic transmission leading to a final common pathway appears to involve deficits in phonological coding. Postmortem studies and in vivo anatomical imaging suggest altered asymmetry of structures in the temporal lobes, and neuroimaging with positron emission tomography indicates left temporoparietal dysfunction in particular. Neuromaging is providing a window into the brain that promises further insights into the biology of dyslexia.

Adult↗

Listening comprehension and working memory are impaired in attention-deficit hyperactivity disorder irrespective of language impairment.

This study investigated listening comprehension and working memory abilities in children with attention-deficit hyperactivity disorder (ADHD), presenting with and without language impairments (LI). A 4-group design classified a community sample (n = 77) of boys aged 9-12 into ADHD, ADHD + LI, LI, and Normal groups. Children completed tests of basic language and cognitive skills, verbal and spatial working memory, and passage-level listening comprehension. Multivariate analyses and post hoc comparisons indicated that ADHD children who did not have co-occurring LI comprehended factual information from spoken passages as well as normal children, but were poorer at comprehending inferences and monitoring comprehension of instructions. ADHD children did not differ from normal children in verbal span, but showed significantly poorer verbal working memory, spatial span, and spatial working memory. The ADHD + LI and LI groups were most impaired in listening comprehension and working memory performance, but did not differ from each other. Listening comprehension skills were significantly correlated with both verbal and spatial working memory, and parent-teacher ratings of inattention and hyperactivity/impulsivity. Findings that children with ADHD but no LI showed subtle higher-level listening comprehension deficits have implications for both current diagnostic practices and conceptualizations of ADHD.

Attention Deficit Disorder with Hyperactivity↗

The classifier problem in Chinese aphasia.

In recent years, research on the relationship between brain organization and language processing has benefited tremendously from cross-linguistic comparisons of language disorders among different types of aphasic patients. Results from these cross-linguistic studies have shown that the same aphasic syndromes often look very different from one language to another, suggesting that language-specific knowledge is largely preserved in Broca's and Wernicke's aphasics. In this paper, Chinese aphasic patients were examined with respect to their (in)ability to use classifiers in a noun phrase. The Chinese language, in addition to its lack of verb conjugation and an absence of noun declension, is exceptional in yet another respect: articles, numerals, and other such modifiers cannot directly precede their associated nouns, there has to be an intervening morpheme called a classifier. The appropriate usage of nominal classifiers is considered to be one of the most difficult aspects of Chinese grammar. Our examination of Chinese aphasic patients revealed two essential points. First, Chinese aphasic patients experience difficulty in the production of nominal classifiers, committing a significant number of errors of omission and/or substitution. Second, two different kinds of substitution errors are observed in Broca's and Wernicke's patients, and the detailed analysis of the difference demands a rethinking of the distinction between agrammatism and paragrammatism. The result adds to a growing body of evidence suggesting that grammar is impaired in fluent as well as nonfluent aphasia.

Aphasia, Broca↗

[Study of neurological diseases prevalence in Aratoca, a rural area of eastern Colombia].

BACKGROUND: Current health statistics on the prevalence of neurological diseases, in Colombia, are still lacking. AIM: To determine the prevalence of migraine, cerebrovascular disease, movement disorders, peripheral neuropathies, mental retardation and developmental disorders, epilepsy, dementia and sequels of cranial trauma in a rural population of Colombia. MATERIALS AND METHODS: We evaluated 544 subjects in Aratoca, a rural place of Santander, placed at 400 kilometers from Bogota. The world health organization (WHO) protocol for neuroepidemiological studies modified by our group, a survey to determine sequels of cranial trauma and the mini mental state examination were used as instruments. People 12 years-old or more and suspected to have a neurological disease was evaluated by adult neurologists and those less than 12 years-old were evaluated by a neuropediatrician. RESULTS: 223 out of 544 subjects surveyed were evaluated by the clinicians of which 135 had a neurological disorder. The prevalence of neurological diseases, per thousand inhabitants were as follows: migraine 189.3; epilepsy 33; febrile seizures 25.6; peripheral neuropathy 22.1; mental retardation 18.4; developmental and language disorders 11; dementia 10.5; cerebrovascular disease 4.7; and movement disorders 3.7. CONCLUSIONS: These results will allow to define appropriate control measures, as well as to prevent and treat these prevalent neurological disorders in rural places of Colombia as Aratoca and the like.

Adolescent↗

[Clinical features of epilepsy in autism spectrum disorders].

Autism is a rare disease, but is currently in vogue. An incidence of 1/1,000 has been estimated for early childhood autism, but in autism spectrum the incidence can reach 30/1,000. The clinical characteristics of autism are cognitive language disorders, lack of social interaction, obsessive behaviours with stereotypic movements and epilepsy. For some authors it is an associated symptom while for others it is a cardinal symptom of the disease. Epilepsy in autism, as an association, was reported as early as 1944 by Leo Kanner, in describing his initial work involving 11 cases and how one of them suffered from epilepsy. In 1960, the prevalence of epilepsy among autistic patients was evaluated and found to be much higher than in the normal population. The figures differ from author to author, but really they depend on age: the higher the age, the greater the prevalence of epilepsy. It was in fact this detail that drew our attention to this issue, and triggered the first questions in our research into autism and epilepsy. The series vary from 4 to 86%, depending on the methodology used to confirm the epilepsy. In our casuistics, between the ages of 1 and 18 years, 20% of these children with autism spectrum disorders suffer some kind of epileptic seizures, and 80% suffer what other researchers call subclinical seizures . In this paper, the symptomatology of the most important subclinical seizures that have been observed in these children is presented; the diseases associated with autism are commented on; the concept of primary and secondary autism is discussed; we explain why seizures occur in autism and their consequences as a neurodevelopmental disorder, and we put forward an interesting hypothesis within our general theory of autism. We also comment on the importance that the use of functional magnetic images in the study of autism has in our research.

Adolescent↗

Differential diagnosis between dementia and depression: a study of efficiency increment.

A battery of neuropsychological tests was examined to establish its value in differentiating mild dementia from depression. The battery was applied to 84 subjects: 31 dementia patients of several etiologies, 31 patients with major depression and 22 volunteers whose age and educational level was similar to that of the patients. The battery consisted of tests for immediate, recent and remote memory, the Mini Mental State examination, a screening test for language disorders, and tests measuring abstraction, calculation, judgement, praxic and gnosic functions. The results showed significant differences among the three groups in all memory tests. Deficits of abstraction, calculation, praxis and language functions were strongly associated with dementia. The epidemiological analysis of efficiency increment, with determination of sensitivity and specificity, showed a highly significant enhancement of efficiency for diagnostic purposes when the results of the tests were combined. These findings suggest that the entire battery of neuropsychological tests should be applied, and their results compared using the efficiency increment procedure, in order to establish a firm differential diagnosis between dementia and depression, and/or to detect the former when it is at a mild early stage.

Adult↗

Reversible aphasic disorder induced by lamotrigine in atypical benign childhood epilepsy.

PURPOSE: We studied an eleven year-old girl with atypical, benign partial epilepsy who acutely presented a severe aphasia associated with marked EEG deterioration after lamotrigine administration. A parallel monitoring of language disorders and EEG changes during the gradual withdrawal of lamotrigine was performed in order to evaluate their possible correlation with lamotrigine administration. METHODS: Detailed neuropsychological and linguistic examinations in association with awake and sleep EEG were periodically performed. RESULTS: The evolution of the aphasic disorder was closely related to the EEG abnormalities, and disappeared after the withdrawal of lamotrigine. CONCLUSIONS: We considered the hypothesis that our case could be an expression of a paradoxical reaction to lamotrigine, in which the transitory aphasic disorder was sustained by an epileptiform electric activation.

Anticonvulsants↗

Joubert syndrome is not a cause of classical autism.

A previous report noted a 27% prevalence of autism in Joubert syndrome (JS), raising the question of overlapping etiologies. Family studies have shown that autism is characterized by family loading for a number of specific behavioral and psychiatric disorders and that the sib recurrence risk is around 4%. The purpose of this study is to determine whether children with Joubert and their families show behavioral or genetic characteristics similar to autism. Thirty-one volunteer Joubert families were identified. Parents completed a semi-structured family history interview and the Autism Behavioral Checklist. Rates of family loading for neuropsychiatric disorders in the JS families were compared to autism family history data and Down syndrome (DS) controls. The JS families had significantly lower rates of autism, alcoholism, cognitive, and language disorders than the autism families. Their rate of depression was lower, but not significantly different from that found in autism families. None of the JS children met the clinical cut-off for autism based on parental symptom report and the sib recurrence risk was 32% for the JS families compared to 4% for the autism and 0% for DS families. These data indicate that JS is a genetically distinct disorder from autism. Different genes with different inheritance patterns that affect neurodevelopment of the cerebellum could explain the clinical similarities previously reported in JS and autism.

Abnormalities, Multiple↗

[Transcortical sensory aphasia produced by lesions of the anterior basal ganglia area].

We reported three cases of an aphasic syndrome caused by unusual lesion distribution. Our patients, language disorders could be summarized as transcortical sensory aphasia and showed following symptoms; (1) fluent paraphasic verbal output, (2) anomia which was not facilitated by cueing, (3) impaired comprehension of spoken language, (4) preserved capacity of repetition, (5) preserved ability of reading aloud with impaired comprehension of the written material and (6) agraphia. In addition, all had no associated physical neurological signs such as hemiparesis or hemianopsia. All were right handed. All three cases showed the similar lesion distribution by computed tomographic scanning of the brain. All had low density areas in the anterior portion of the left basal ganglia including the head of the caudate nucleus, the anterior portion of the putamen, the anterior portion of the anterior limb of the internal capsule and the nearby white matter. Case 2 also had the small right hemisphere lesion in the white matter near the anterior portion of the lateral ventricle. Transcortical sensory aphasia with this lesion distribution has not been reported. We attributed the causative damage to lesions of the white matter and not to lesions of the basal ganglia per se. It was also speculated that fluent aphasia can be produced by the anteriorly situated white matter lesion if issuing fibers from the Broca's area were spared. Finally a possible anatomoclinical correlation for "transcortical alexia" (preserved oral reading and impaired reading comprehension) was attempted. The symptom is probably a reflection of the fact that the posterior speech area including the angular gyrus was left intact.

Aged↗

Describing the consequences of disorders: stuttering and the International Classification of Impairments, Disabilities, and Handicaps.

The International Classification of Impairments, Disabilities, and Handicaps (ICIDH) proposed by the World Health Organization (WHO, 1980) can be a useful method for describing the experiences of individuals exhibiting speech and language disorders such as stuttering. Unfortunately, the definitions for the terms impairment, disability, and handicap that were previously presented in the stuttering literature have not accurately represented the true purpose of the ICIDH as a tool for describing the consequences of disorders. This paper reviews the history of these terms in the stuttering literature and proposes alternate definitions that are more consistent with the use of the ICIDH elsewhere in the field of communication disorders, as well as within the health-related professions in general. Based on the ICIDH framework, a model is proposed for discussing the outcomes of various types of stuttering treatments in the context of an individual's experience of the stuttering disorder, and the implications for treatment outcomes research using this framework are discussed.

Persons with Disabilities↗

[Event-related potentials and language processing].

The contribution of cognitive event-related potentials (ERPs) and particularly of the N400 component to the study of language processing is considered. After a general presentation of the various components of cognitive evoked potentials which are differentiated from early potentials (sensitive to perceptive parameters), the neurophysiological and psychological correlates of ERPs are discussed. Before the presentation of the main studies concerned with the correlation between specific late components and linguistic processes, it is proposed an introduction to some theoretical models of language processing which serve as conceptual framework supporting most of the experimental approaches in this field. The results of EPs studies devoted to functional asymetries in language processing are briefly mentioned. Then a number of studies are analysed, these demonstrate the sensitivity of event-related potential measures to linguistic manipulations. Finally, the studies concerned with the N400 are reviewed, this potential appears to be the more specifically language-related ERP component, even if its connection with specific linguistic processes or its differentiation from N200 are still under debate. Nevertheless language-related electrophysiological investigations may become in the near future a useful complementary approach to our understanding of language processing and language disorders.

Brain↗

Answers to wh-questions: a developmental study.

Forty children aged 3-6 years responded to items representing nine wh-question forms. Questions referred to three types of referential sources (conditions) based on immediacy and visual availability. The hierarchies of wh-question forms based on degree of difficulty were similar to those described in previous investigations. However, a significant interaction between referential conditions and wh-form was found to influence the relative complexity of the stimulus questions. The children were significantly less successful in giving appropriate and accurate responses when the question referred to objects, persons, or events not represented in the immediate setting. Recognition and delivery of the general category or kind of information required by a wh-form (functional appropriateness) appeared to predate substantially the ability to respond with fact, accuracy, logic, and credibility (functional accuracy). The results suggest consideration and control of referential source as well as appropriateness/accuracy response criteria in the evaluation and treatment of language-disordered children.

Age Factors↗

[Psychometric speech studies in Alzheimer's dementia with the Aachen aphasia test].

Dementias of the Alzheimer type seem to be frequently accompanied by language disturbances. These may represent a feature which can be of help in distinguishing them from other types of dementias. We used the Aachen aphasia test in 32 patients suffering from Alzheimer dementia according to research criteria, and a mixed sample of 35 patients suffering from other dementias. From these 2 groups 2 subsamples of 21 patients each were gathered which were comparable with regard to age, disease onset, level of education, verbal intelligence and severity of senility. Nevertheless it was possible to distinguish the groups to a certain degree on grounds of psychometric language criteria alone. Alzheimer patients were more severely handicapped communicatively, less dysarthric, produced more automatisms and discretely more phonemic paraphasias with fluent speech which was sometimes paragrammatic. A relatively better level of repetition compared to the Token test and written language was fairly specific. A computer-assisted classification yielded language disturbances similar to Wernicke's aphasia more often than with non-Alzheimer dementias. We found no Alzheimer patients with a Broca's type of language disorder, while amnestic and global types were bound to the level of overall impairment to a certain degree. The significance of these results with regard to the use of psychometric language test in the dementias, particularly Alzheimer's dementia, and to differential diagnostic considerations are reviewed briefly.

Aged↗

Magnetoencephalographic patterns of epileptiform activity in children with regressive autism spectrum disorders.

BACKGROUND: One-third of children diagnosed with autism spectrum disorders (ASDs) are reported to have had normal early development followed by an autistic regression between the ages of 2 and 3 years. This clinical profile partly parallels that seen in Landau-Kleffner syndrome (LKS), an acquired language disorder (aphasia) believed to be caused by epileptiform activity. Given the additional observation that one-third of autistic children experience one or more seizures by adolescence, epileptiform activity may play a causal role in some cases of autism. OBJECTIVE: To compare and contrast patterns of epileptiform activity in children with autistic regressions versus classic LKS to determine if there is neurobiological overlap between these conditions. It was hypothesized that many children with regressive ASDs would show epileptiform activity in a multifocal pattern that includes the same brain regions implicated in LKS. DESIGN: Magnetoencephalography (MEG), a noninvasive method for identifying zones of abnormal brain electrophysiology, was used to evaluate patterns of epileptiform activity during stage III sleep in 6 children with classic LKS and 50 children with regressive ASDs with onset between 20 and 36 months of age (16 with autism and 34 with pervasive developmental disorder-not otherwise specified). Whereas 5 of the 6 children with LKS had been previously diagnosed with complex-partial seizures, a clinical seizure disorder had been diagnosed for only 15 of the 50 ASD children. However, all the children in this study had been reported to occasionally demonstrate unusual behaviors (eg, rapid blinking, holding of the hands to the ears, unprovoked crying episodes, and/or brief staring spells) which, if exhibited by a normal child, might be interpreted as indicative of a subclinical epileptiform condition. MEG data were compared with simultaneously recorded electroencephalography (EEG) data, and with data from previous 1-hour and/or 24-hour clinical EEG, when available. Multiple-dipole, spatiotemporal modeling was used to identify sites of origin and propagation for epileptiform transients. RESULTS: The MEG of all children with LKS showed primary or secondary epileptiform involvement of the left intra/perisylvian region, with all but 1 child showing additional involvement of the right sylvian region. In all cases of LKS, independent epileptiform activity beyond the sylvian region was absent, although propagation of activity to frontal or parietal regions was seen occasionally. MEG identified epileptiform activity in 41 of the 50 (82%) children with ASDs. In contrast, simultaneous EEG revealed epileptiform activity in only 68%. When epileptiform activity was present in the ASDs, the same intra/perisylvian regions seen to be epileptiform in LKS were active in 85% of the cases. Whereas primary activity outside of the sylvian regions was not seen for any of the children with LKS, 75% of the ASD children with epileptiform activity demonstrated additional nonsylvian zones of independent epileptiform activity. Despite the multifocal nature of the epileptiform activity in the ASDs, neurosurgical intervention aimed at control has lead to a reduction of autistic features and improvement in language skills in 12 of 18 cases. CONCLUSIONS: This study demonstrates that there is a subset of children with ASDs who demonstrate clinically relevant epileptiform activity during slow-wave sleep, and that this activity may be present even in the absence of a clinical seizure disorder. MEG showed significantly greater sensitivity to this epileptiform activity than simultaneous EEG, 1-hour clinical EEG, and 24-hour clinical EEG. The multifocal epileptiform pattern identified by MEG in the ASDs typically includes the same perisylvian brain regions identified as abnormal in LKS. When epileptiform activity is present in the ASDs, therapeutic strategies (antiepileptic drugs, steroids, and even neurosurgery) aimed at its control can lead to a significa

Autistic Disorder↗

The sum and substance of it: the appreciation of mass and count quantifiers in Parkinson's disease.

We performed three experiments in order to determine whether patients with Parkinson's disease (PD) have a sentence comprehension impairment. In the first experiment, we found that nondemented PD patients were impaired at matching a short sentence containing a mass or count quantifier (e.g., "Point to the jar with many") with one of four pictures. Patients erred by pointing to the incorrect mass or count type of substance, but pointed correctly to the large or small amount of a substance in the picture. In the second experiment, we asked the same PD patients to judge short sentences containing mass or count nouns modified by mass or count adjectives (e.g., *"The glass contains many milk"). PD patients were significantly impaired at detecting errors in the agreement between a noun and a quantifying adjective and errors in the appropriate use of the plural associated with a noun. A sentence completion task was performed in order to determine whether patients impaired on both sentence-picture matching and sentence judgment tasks are also compromised in their expression of quantifiers. Some patients impaired on both tasks also had difficulty expressing quantifiers, and it is these patients who may have difficulty performing grammatical computations. Other patients were selectively impaired in their sentence-picture matching or their sentence judgments, suggesting a performance deficit concerned with a particular task. We conclude that sentence comprehension impairments in PD are multifactorial in nature.

Adult↗

Impairments of lexical-semantic processing in aphasia: evidence from the processing of lexical ambiguities.

Broca's and Wernicke's aphasics performed speeded lexical decisions on the third member of auditorily presented triplets consisting of two word primes followed by either a word or a nonword. In three of the four priming conditions, the second prime was a homonym with two unrelated meanings. The relation of the first prime and the target with the two meanings of the homonym was manipulated in the different priming conditions. The two readings of the ambiguous words either shared their grammatical form class (noun-noun ambiguities) or not (noun-verb ambiguities). The silent intervals between the members of the triplets were varied between 100, 500, and 1250 msec. Priming at the shortest interval is mainly attributed to automatic lexical processing, and priming at the longest interval is mainly due to forms of controlled lexical processing. For both Broca's and Wernicke's aphasics overall priming effects were obtained at ISIs of 100 and 500 msec, but not at an ISI of 1250 msec. This pattern of results is consistent with the view that both types of aphasics can automatically access the semantic lexicon, but might be impaired in integrating lexical-semantic information into the context. Broca's aphasics showed a specific impairment in selecting the contextually appropriate reading of noun-verb ambiguities, which is suggested to result from a failure either in the on-line morphological parsing of complex word forms into a stem and an inflection or in the on-line exploitation of the syntactic implications of the inflectional suffix. In a final experiment patients were asked to explicitly judge the semantic relations between a subset of the primes that were used in the lexical decision study. Wernicke's aphasics performed worse than both Broca's aphasics and normal controls, indicating a specific impairment for these patients in consciously operating on automatically accessed lexical-semantic information.

Aged↗

Strategic search and retrieval inhibition: the role of the frontal lobes.

Recall of words from categorised lists was examined in 77 patients and 12 normal control subjects. In Experiment 1, both left temporal-lobe excisions that included the hippocampus (LTH) and left frontal-lobe removals (LF) impaired free recall, but the LF group performed normally when encoding and retrieval strategies were supplied. In Experiment 2, experimentally induced interference during cued recall abnormally hampered performance for the LF, but not for the LTH group. Subsequent removal of the interfering cues resulted in improved performance for the LF group. Thus, the integrity of the left frontal lobe seems indispensable for normal strategic retrieval and for the suppression of potentially interfering items in verbal memory.

Adolescent↗