1/25. A case study of an English-Japanese bilingual with monolingual dyslexia.We report the case of AS, a 16 year-old English/Japanese bilingual boy, whose reading/writing difficulties are confined to English only. AS was born in japan to a highly literate Australian father and English mother, and goes to a Japanese selective senior high school in japan. His spoken language at home is English. AS's reading in logographic Japanese Kanji and syllabic Kana is equivalent to that of Japanese undergraduates or graduates. In contrast, his performance in various reading and writing tests in English as well as tasks involving phonological processing was very poor, even when compared to his Japanese contemporaries. Yet he has no problem with letter names or letter sounds, and his phoneme categorisation is well within the normal range of English native speakers. In order to account for our data that show a clear dissociation between AS's ability to read English and Japanese, we put forward the 'hypothesis of granularity and transparency'. It is postulated that any language where orthography-to-phonology mapping is transparent, or even opaque, or any language whose orthographic unit representing sound is coarse (i.e. at a whole character or word level) should not produce a high incidence of developmental phonological dyslexia.- - - - - - - - - - ranking = 1keywords = reading, dyslexia (Clic here for more details about this article) |
2/25. Central auditory processing disorders and reduced motivation: three case studies.The central auditory test results for three normal-hearing children who were initially diagnosed as having a central auditory processing disorder and learning disability are presented. They were referred to the authors for second-opinion consultations. Central auditory processing retesting was performed by the authors under the condition of no reinforcement and then the condition of reinforcement with the child's favorite food, hobby, or toy. For all three cases, the central auditory test scores improved markedly bilaterally under the condition of reinforcement as compared with the condition of no reinforcement. We hypothesize that the improvement was related to increased motivation associated with the reinforcement and that these children represented false-positive results on the central auditory test battery. Large-sample studies are needed to investigate the effect of reinforcement on test performance in children with reduced central auditory test scores.- - - - - - - - - - ranking = 0.008502213437216keywords = disability (Clic here for more details about this article) |
3/25. A developmental phonological short-term memory deficit: a case study.A developmental case of phonological short-term memory deficit was studied in a highly educated subject. The subject, BS, who had obtained a Ph.D. in molecular biology, demonstrated striking deficits on some short-term memory tasks, particularly for auditorily presented nonword lists. With visual presentation and with meaningful words, he often scored at a normal level. The results indicate a deficit in retaining phonological information but an ability to use visual, lexical, and semantic information to boost recall. Despite this phonological short-term memory deficit, BS scored at a normal level on a syntactic comprehension test and on reading of nonwords. He was impaired, however, on repeated list learning, learning of foreign vocabulary, and transcribing dictated materials. The implications of these results for models of short-term memory and the uses of phonological retention in cognitive processing are discussed.- - - - - - - - - - ranking = 0.17470885827284keywords = reading (Clic here for more details about this article) |
4/25. Deletion of 8p: a report of a child with normal intelligence.The case is presented of a female infant with a distal deletion of 8p (8p23.1-->pter) whose development was monitored over a 5-year period from 12 months of age. Although previous literature has suggested that 8p deletion is associated with mild to moderate intellectual disability, the child reported here has normal intelligence. Despite initial delays in gross motor and language skills, cognitive development (assessed with the Bayley Scales of infant Development) and intellectual ability (measured on the Stanford-Binet intelligence Scale) were within average range. It is argued that the small number of previous case reports may have created a misleading impression of intellectual development in individuals with distal deletions of 8p.- - - - - - - - - - ranking = 0.008502213437216keywords = disability (Clic here for more details about this article) |
5/25. A family with a grand-maternally derived interstitial duplication of proximal 15q.About 1% of individuals with autism or types of pervasive developmental disorder have a duplication of the 15q11-q13 region. These abnormalities can be detected by routine G-banded chromosome study, showing an extra marker chromosome, or demonstrated by fluorescence in situ hybridization (FISH) analysis, revealing an interstitial duplication. We report here the molecular, cytogenetic, clinical and neuropsychiatric evaluations of a family in whom 3 of 4 siblings inherited an interstitial duplication of 15q11-q13. This duplication was inherited from their mother who also had a maternally derived duplication. Affected family members had apraxia of speech, phonological awareness deficits, developmental language disorder, dyslexia, as well as limb apraxia but did not have any dysmorphic clinical features. The observations in this family suggest that the phenotypic manifestations of proximal 15q duplications may also involve language-based learning disabilities.- - - - - - - - - - ranking = 0.095174685036295keywords = dyslexia (Clic here for more details about this article) |
6/25. Christine: a case study of literacy acquisition by an adult with developmental disabilities.Christine, an adult with developmental disabilities, had no history of education. A nonreader, she began to receive literacy tutoring at age 35. In 7 years she was educated through an eclectic approach that primarily entailed echo reading of brief passages, such as sentences and stories. She moved from holistic recognition of print to an ability to respond to instruction about analysis of some features of print, thereby gaining insights into decoding and spelling. This approach contrasts with literacy instruction that teaches adults with developmental disabilities to memorize small units of decontextualized print (letter-sound correspondences or survival words) before reading full text. Implications for facilitating literacy in adults with developmental disabilities by using contextually supported reading instruction are explored.- - - - - - - - - - ranking = 0.52412657481853keywords = reading (Clic here for more details about this article) |
7/25. speech and language deterioration in benign rolandic epilepsy.A 5-year-old boy presented with typical clinical and electrophysiologic features of benign rolandic epilepsy. His neurodevelopment, language, and behavior prior to the onset of epilepsy were appropriately normal. He demonstrated marked deterioration of language and cognitive function during the course to a mild and then a moderate disability range. Serial sleep electroencephalographic recordings initially showed continuous and bilateral rolandic discharges with evolution to localized left rolandic spikes. language and cognitive improvements were subsequently seen. Educational support and evolution of the electroencephalogram to a localized focus could have been contributory. It is anticipated, however, that he will have significant long-term problems in complex language.- - - - - - - - - - ranking = 0.008502213437216keywords = disability (Clic here for more details about this article) |
8/25. Developmental amnesia: a new pattern of dissociation with intact episodic memory.A case of developmental amnesia is reported for a child, CL, of normal intelligence, who has intact episodic memory but impaired semantic memory for both semantic knowledge of facts and semantic knowledge of words, including general world knowledge, knowledge of word meanings and superordinate knowledge of words. In contrast to the deficits in semantic memory, there are no impairments in episodic memory for verbal or visual material, assessed by recall or recognition. Lexical decision was also intact, indicating impairment in semantic knowledge of vocabulary rather than absence of lexical representations. The case forms a double dissociation to the cases of Vargha-Khadem et al. [science 277 (1997) 376; Episodic memory: new directions in research (2002) 153]; Gadian et al. [brain 123 (2000) 499] for whom semantic memory was intact but episodic memory was impaired. This double dissociation suggests that semantic memory and episodic memory have the capacity to develop separately and supports models of modularity within memory development and a functional architecture for the developmental disorders within which there is residual normality rather than pervasive abnormality. knowledge of arithmetical facts is also spared for CL, consistent with adult studies arguing for numeracy knowledge distinct from other semantics. reading was characterised by difficulty with irregular words and homophones but intact reading of nonwords. CL has surface dyslexia with poor lexico-semantic reading skills but good phonological reading skills. The case was identified following screening from a population of normal schoolchildren suggesting that developmental amnesias may be more pervasive than has been recognised previously.- - - - - - - - - - ranking = 0.61930125985482keywords = reading, dyslexia (Clic here for more details about this article) |
9/25. Neurodevelopmental pattern of succinic semialdehyde dehydrogenase deficiency (gamma-hydroxybutyric aciduria).Succinic semialdehyde dehydrogenase (SSADH deficiency) (MIM 271980) is a defect in gamma-aminobutyric acid catabolism, resulting in the accumulation of gamma-hydroxybutyric acid (GHB) and causing neurological and cognitive disorders of varying severity. The non-specific nature and the difficulties in detection of urinary GHB explain why this disorder is largely underdiagnosed. Of 350 patients identified worldwide, to date only six adults with SSADH deficiency have been reported in the literature. Here we describe two additional cases in brothers up to ages 26 and 28 years. This retrospective report sheds light on the clinical features of SSADH deficiency in relation to the physiopathological involvement of GHB, and tries to identify the specific neurodevelopmental pattern of this learning disability.* Features of this are: early impaired psychomotor development with hypotonia and disturbances in motor coordination; impaired development of language, mainly due to poor auditory perception; and seizures and psychotic features in late adolescence or adulthood. Moreover, narcolepsy-like symptoms could be a consistent feature of the disease.- - - - - - - - - - ranking = 0.008502213437216keywords = disability (Clic here for more details about this article) |
10/25. reading in callosal agenesis.It has been suggested that deficits in explicit phonological processing are causal in developmental dyslexia. Deficits in such skills have been reported in developmental phonological dyslexia, though not in developmental surface dyslexia. The reading performance of two children with callosal agenesis, who have been previously shown to have impairments on rhyming tasks, are reported. Neither child is dyslexic in the traditional sense, since word reading levels are appropriate for age. However, both children have impaired development of the phonological reading route despite normal lexical skills. The pattern of their reading is therefore comparable to developmental phonological dyslexia. Problems in explicit phonological processing may be causal in the failure to establish an efficient phonological reading route but this is insufficient to create difficulty with word recognition itself. The corpus callosum may be essential for the normal development of a phonological reading route.- - - - - - - - - - ranking = 1.4289518897822keywords = reading, dyslexia (Clic here for more details about this article) |
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