Cases reported "Language Disorders"

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1/20. Auditory neuropathy: case study with hyperbilirubinemia.

    Auditory neuropathy (AN) has been described in the literature as presenting with a combination of audiometric findings that include elevated behavioral audiometric thresholds, auditory brainstem response findings that are not consistent with audiometric findings, poor speech recognition, and present otoacoustic emissions (OAEs) and/or cochlear microphonics. Since the availability of clinical OAE testing, AN has come to be identified with increasing frequency; however, incidence and prevalence figures are unavailable. There is a great deal of discussion about the accurate diagnosis of AN, its characteristics, and its treatment. Some of this discussion is occurring on the internet and over the telephones. The need to continue to provide information in accessible peer-reviewed journals is paramount. Following a review of the literature, a case study is presented of a boy who was diagnosed with AN as a newborn. He experienced hyperbilirubinemia and other neonatal health complications. His educational intervention was managed elsewhere until recently. Information is presented about the progression of the case over a 5-year period that includes audiologic data and communication development results.
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2/20. Deficits in irregular past-tense verb morphology associated with degraded semantic knowledge.

    Two distinct mechanisms are often considered necessary to account for generation of the past-tense of English verbs: a lexical associative process for irregular forms like speak-->spoke, and a rule-governed process ("add -ed") for regular and novel forms like talk-->talked and wug-->wugged. An alternative account based on a parallel-distributed processing approach proposes that one complex procedure processes all past-tense types. In this alternative view, neuropsychological dissociations are explained by reduced input from word meaning that plays a greater role in successful generation of the past-tense for lower frequency irregular verbs, and by phonological deficits that disproportionately affect regular and novel forms. Only limited evidence has been available concerning the relationship between knowledge of word meaning and verb-tense processing. The study reported here evaluated the past-tense verb abilities of 11 patients with semantic dementia, a neurodegenerative condition characterised by degraded semantic knowledge. We predicted and confirmed that the patients would have essentially normal ability to generate and recognise regular (and novel) past-tense forms, but a marked and frequency-modulated deficit on irregular verbs. Across the set of 11 patients, the degree of impairment for the irregular past-tense was significantly correlated with the degree of comprehension impairment as measured by verb synonym judgements. These results, plus other features of the data such as the nature of the errors to irregular verbs, are discussed in relation to currently developing theories of the language system.
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3/20. The impact of deep dysgraphia on graphemic buffer disorders.

    This article describes an investigation into the residual writing skills of a severely dysgraphic patient (DA). We found that they were powerfully influenced by a number of lexical variables (lexicality, frequency, imageability, length and geminates). His error pattern was characterized by semantic, lexical, substitution, deletion errors and fragment responses that preserved the first letter. Thus, DA's written spelling was characterized by both deep dysgraphic and graphemic output buffer effects. It is proposed that this pattern of performance represents a new"putative functional syndrome."
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4/20. Hemispheric integrative therapy in landau-kleffner syndrome: applications for rehabilitation sciences.

    A case study is presented of a 14-year-old right-handed Caucasian female diagnosed with the landau-kleffner syndrome (LKS) at the age 3 1/2 years. Her LKS symptoms presented with abrupt disruption in language after normal development, electroencephalogram (EEG) brain-wave abnormality, seizure activity, inability to read, and impairment in her motor skills. After 11 years of pharmacological and special education interventions with no significant improvement in any measurable area of function, a multimodal approach using techniques purportedly aimed at facilitating inter-hemispheric communication was provided. At completion of the program, EEG was controlled, reading, language, and auditory processing improved and objective behavioral-social measures improved significantly.
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5/20. The natural history of late-stage "pure" semantic dementia.

    Relatively little is known about the neuropsychological profile of late-stage semantic dementia. This article provides a detailed assessment of patient MK who, despite her very severe semantic impairments, remained cooperative to testing and, unusually, did not show additional behavioral/personality changes. Although MK's initial presentation was typical of semantic dementia (SD), her performance began to deviate from the normal pattern. She developed impairments of single word repetition and regular word reading, and began to produce phonological errors in picture naming and spontaneous speech. These deficits might suggest that late-stage SD includes an independent disorder of phonology. An alternative possibility, however, is that phonological processing cannot proceed normally in the face of profound semantic degradation. A series of experiments supported the latter explanation of MK's deficits. In picture naming, MK showed little effect of progressive phonological cueing, did not reveal an increased sensitivity to word length or phonological complexity and continued to show a high degree of item-specific consistency in both accuracy and errors: she tended to produce the same erroneous phonemes for each item. She remained sensitive to the effects of phonological similarity in immediate serial recall. letter substitution errors in regular word reading were more common for lower frequency letters (e.g., Q, Z). These letters also produced more item errors in immediate serial recall, suggesting that a frequency-graded loss of letter knowledge, rather than separate orthographic and phonological deficits, accounted for the deficits in both of these tasks. These findings are discussed in terms of theories that posit strong interactivity between phonology and semantics.
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6/20. epilepsy with continuous spikes and wave during slow sleep: A case report.

    This report presents a 5.5-year-old girl who presented with multiple types of seizure disorder along with behavioral change, cognitive deterioration, and language impairment. electroencephalography showed nearly continuous spike-wave during slow wave sleep. Both clinical and electrographic findings were consistent with epilepsy with continuous spikes and wave during slow sleep (CSWS). Although the seizures were well controlled with conventional antiepileptic drugs, improvement of behavioral, cognitive, and language functions was observed only after adding corticosteroid as an adjunctive treatment. Corticosteriod may have a role in treatment in children with CSWS.
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7/20. When Ciarli Ciaplin drives a Pejio' in Itali: writing loan words in a shallow language.

    With increasing frequency the lexicon of every language incorporates words taken from other languages (loan words) that become of common use, but whose orthography does not necessarily conform to the rules of the native language. This is particularly true for languages like Italian, where most words can be correctly written by the application of a sublexical phonology-to-orthography conversion procedure. Here we report the case of a neurologically unimpaired, highly educated Italian person, with a specific deficit in writing loan words. The aim of the study is to clarify the nature of her dysgraphic impairment, if linked to a semantic deficit, or alternatively reflecting an acquisition deficit, present both in the phonological and orthographic output lexicons, or specific only for the orthographic lexicon. A qualitative analysis of her errors and a comparison with the performance of a group of age and education matched subjects showed that her error pattern was not an amplification of the task difficulty effect observed in the control subjects, but rather an expression of an impairment in acquiring specific writing procedures.
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8/20. Acquired aphasia, dementia, and behavior disorder with epilepsy and continuous spike and waves during sleep in a child.

    Severe persistent neuropsychological disorders sometimes develop in the course of a focal epilepsy of unknown origin in previously normal children. Very frequent bilateral focal or generalized discharges are often noted on the sleep EEG records of these patients with no evidence of clinical seizures. The relation between these paroxysms and the observed deterioration remains unclear. We report a child with a partial complex epilepsy and severe disturbances of language, cognition, and behavior acquired in the early years of development who was followed for 15 years. A correlation between the evolution of the striking EEG abnormalities during sleep and the neuropsychological disorders could be established retrospectively. The observed sequence of onset and recovery of the aphasia, the dementia, and the "psychotic" behavior makes a direct causal relation between the deficits quite unlikely. Rather it suggests an association of independent symptoms with a specific language disorder becoming manifest in the course of the evolution. This child shows many of the main characteristics of the syndromes of "acquired aphasia with convulsive disorder" (landau-kleffner syndrome) and "epilepsy with continuous spike waves during sleep." Both syndromes describe probably different facets of a similar underlying, still unexplained cerebral dysfunction.
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9/20. Rule invention in the acquisition of morphology revisited: a case of transparent semantic mapping.

    This paper presents a case study of a language-impaired child who signaled the distinction between English singular and plural using suprasegmental cues rather than the usual segmental form used within the parent language. Acoustic analyses performed within the first study in the paper revealed that the suprasegmental features used to maintain this distinction included various duration, fundamental frequency, and intensity parameters. Acoustic analyses were also performed on a set of matched two- and four-item plural forms within a second study. The results of these analyses indicated that the same acoustic parameters were used to distinguish two-item plural forms from four-item plural forms. This case of linguistic creativity is offered as further evidence in support of the model of language acquisition that emphasizes the active role children take in the acquisition process. Additionally, the phonological, morphological, and psycholinguistic factors that may contribute to such rule invention are discussed.
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10/20. The implicit memory ability of a patient with amnesia due to encephalitis.

    The implicit memory ability of a patient (S.S.) with severe amnesia due to encephalitis was assessed using five independent paradigms: Perceptual priming with real words and pseudowords; Word-stem completion with and without contextual cues; Word-stem completion following presentation of high- vs. low-frequency words; Biasing of the spelling of ambiguous (homophonic) words; and Conceptual priming. On the tasks in which previously acquired knowledge could potentially be activated by a prime (e.g., perceptual priming with real words), both S.S. and the Korsakoff patients performed on a normal level. However, when new learning or new associations had to be formed prior to implicit memory testing (e.g., perceptual priming of pseudowords or contextual word-stem completion), S.S.'s implicit memory performance was superior to Korsakoff amnesics. These results suggested that new learning differentially affects the priming abilities of S.S. and alcoholic Korsakoff patients. Since S.S.'s amnesia is as severe as Korsakoff patients, it was also concluded that severity of amnesia is clearly not the sole determinant of priming capacity; etiology may be equally important.
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