ERP differences of prelexical processing between dyslexic and non-dyslexic children

We concluded that impaired reading is reflected by the decreased amplitude of the early lexical component N170. The lack of a left hemispheric processing preference in both groups and similar activation for words and pseudowords can be considered a lack of reading experience and less established reading system in children. Our results indicate that dyslexic children commit fewer specialized neuronal circuits for processing print and confirm the reasoning that acquiring reading skills requires cortical reorganization over occipito-temporal regions.

from the International Journal of Psychophysiology

Statins and Cognitive Functioning in the Elderly: A Population-Based Study

In a 2009 Cochrane review, the authors concluded that there is good evidence that statins, given in late life to people at risk of vascular disease, have no effect in preventing Alzheimer’s disease or dementia. A related issue, which remains unclear, is whether statins improve cognitive function. While some studies have shown a beneficial effect of statins on cognitive function, others have observed mild detrimental effects on cognition. Our aim was to assess cognitive function in community-dwelling elderly participants treated with statins compared with their untreated counterparts (i.e., controls) living in the same population. 137 population-dwelling participants who were receiving statins and 411 matched controls age ⩾ 65 years (median=72 years) in central Spain (the Neurological Diseases in Central Spain study [NEDICES]) underwent a neuropsychological assessment, including tests of global cognitive performance, frontal-executive function, verbal fluency, and memory. Median duration of statin treatment was 2 years. Of 137 participants receiving statins, 53 (38.7%) were taking pravastatin, 38 (27.7%) simvastatin, 37 (27.0%) lovastatin, 6 (4.4%) fluvastatin, and 3 (2.2%) atorvastatin. Although initial univariate analyses indicated some differences, after adjusting for age, gender, education, depressive symptoms, premorbid intelligence, medications that potentially affect cognitive function, and blood cholesterol levels, statin users and controls performed similarly on all neuropsychological tests. In this population-based sample, elderly participants treated with statins and untreated controls performed similarly in all tested cognitive areas. These results do not support a positive benefit of statins on cognition.

from JAD Journal of Alzheimer’s Disease

Contribution of Callosal Connections to the Interhemispheric Integration of Visuomotor and Cognitive Processes

In recent years, cognitive neuroscience has been concerned with the role of the corpus callosum and interhemispheric communication for lower-level processes and higher-order cognitive functions. There is empirical evidence that not only callosal disconnection but also subtle degradation of the corpus callosum can influence the transfer of information and integration between the hemispheres. The reviewed studies on patients with callosal degradation with and without disconnection indicate a dissociation of callosal functions: while anterior callosal regions were associated with interhemispheric inhibition in situations of semantic (Stroop) and visuospatial (hierarchical letters) competition, posterior callosal areas were associated with interhemispheric facilitation from redundant information at visuomotor and cognitive levels. Together, the reviewed research on selective cognitive functions provides evidence that the corpus callosum contributes to the integration of perception and action within a subcortico-cortical network promoting a unified experience of the way we perceive the visual world and prepare our actions.

from Neuropsychology

Clinical practice guideline: hoarseness (dysphonia)

Without abstract, but includes recommendations for voice therapy.

from the National Guideline Clearinghouse

Study Shows Earlier is Better for Cochlear Implants

Receiving a cochlear implant before 18 months of age dramatically improves a deaf child’s ability to hear, understand and eventually speak, according to a multicenter study that included the UT Dallas Callier Center for Communication Disorders.

from The University of Texas at Dallas News Center

Abnormal N400 word repetition effects in fragile X-associated tremor/ataxia syndrome

Fragile X-associated tremor/ataxia syndrome, a neurodegenerative disorder associated with premutation alleles (55–200 CGG repeats) of the FMR1 gene, affects many carriers in late-life. Patients with fragile X-associated tremor/ataxia syndrome typically have cerebellar ataxia, intranuclear inclusions in neurons and astrocytes, as well as cognitive impairment. Dementia can also be present with cognitive deficits that are as severe as in Alzheimer’s disease, however frontosubcortical type impairment is more pronounced in fragile X-associated tremor/ataxia syndrome. We sought to characterize the P600 and N400 word repetition effects in patients with fragile X-associated tremor/ataxia syndrome, using an event-related potential word repetition paradigm with demonstrated sensitivity to very early Alzheimer’s disease. We hypothesized that the fragile X-associated tremor/ataxia syndrome-affected participants with poor declarative verbal memory would have pronounced abnormalities in the P600 repetition effect. In the event-related potential experiment, subjects performed a category decision task whilst an electroencephalogram was recorded. Auditory category statements were each followed by an associated visual target word (50% ‘congruous’ category exemplars, 50% ‘incongruous’ nouns). Two-thirds of the stimuli (category statement–target word pairs) were repeated, either at short-lag (10–40 s) or long-lag (100–140 s). The N400 and P600 amplitude data were submitted to split-plot analyses of variance. These analyses of variance showed a highly significant reduction of the N400 repetition effect (F = 22.5, P < 0.001), but not of the P600 repetition effect, in mild fragile X-associated tremor/ataxia syndrome (n = 32, mean age = 68.7, mean Mini-Mental State Examination score = 26.8). Patients with fragile X-associated tremor/ataxia syndrome had significantly smaller late positive amplitude (550–800 ms post-stimulus onset) to congruous words (P = 0.04 for group effect). Reduced P600 repetition effect amplitude was associated with poorer recall within fragile X-associated tremor/ataxia syndrome patients (r = 0.66) and across all subjects (r = 0.52). Larger P600 amplitude to new congruous words also correlated significantly with higher free recall scores (r = 0.37, P < 0.01) across all subjects. We found a correlation between the amplitude of late positivity and CGG repeat length in those with fragile X-associated tremor/ataxia syndrome (r = 0.47, P = 0.006). Higher levels of FMR1 mRNA were associated with smaller N400s to incongruous words and larger positive amplitudes (between 300 and 500 ms) to congruous words. In conclusion, event-related potential word repetition effects appear sensitive to the cognitive dysfunction present in patients with mild fragile X-associated tremor/ataxia syndrome. Their more severe reduction in N400 repetition effect, than P600, is in contrast to the reverse pattern reported in amnestic mild cognitive impairment and incipient Alzheimer’s disease (Olichney et al., 2008).

from Brain

A mutation affecting the sodium/proton exchanger, SLC9A6, causes mental retardation with tau deposition

We have studied a family with severe mental retardation characterized by the virtual absence of speech, autism spectrum disorder, epilepsy, late-onset ataxia, weakness and dystonia. Post-mortem examination of two males revealed widespread neuronal loss, with the most striking finding being neuronal and glial tau deposition in a pattern reminiscent of corticobasal degeneration. Electron microscopic examination of isolated tau filaments demonstrated paired helical filaments and ribbon-like structures. Biochemical studies of tau demonstrated a preponderance of 4R tau isoforms. The phenotype was linked to Xq26.3, and further analysis identified an in-frame 9 base pair deletion in the solute carrier family 9, isoform A6 (SLC9A6 gene), which encodes sodium/hydrogen exchanger-6 localized to endosomal vesicles. Sodium/hydrogen exchanger-6 is thought to participate in the targeting of intracellular vesicles and may be involved in recycling synaptic vesicles. The striking tau deposition in our subjects reveals a probable interaction between sodium/proton exchangers and cytoskeletal elements involved in vesicular transport, and raises the possibility that abnormalities of vesicular targeting may play an important role in more common disorders such as Alzheimer’s disease and autism spectrum disorders.

from Brain

Clinical and imaging heterogeneity of polymicrogyria: a study of 328 patients

Polymicrogyria is one of the most common malformations of cortical development and is associated with a variety of clinical sequelae including epilepsy, intellectual disability, motor dysfunction and speech disturbance. It has heterogeneous clinical manifestations and imaging patterns, yet large cohort data defining the clinical and imaging spectrum and the relative frequencies of each subtype are lacking. The aims of this study were to determine the types and relative frequencies of different polymicrogyria patterns, define the spectrum of their clinical and imaging features and assess for clinical/imaging correlations. We studied the imaging features of 328 patients referred from six centres, with detailed clinical data available for 183 patients. The ascertainment base was wide, including referral from paediatricians, geneticists and neurologists. The main patterns of polymicrogyria were perisylvian (61%), generalized (13%), frontal (5%) and parasagittal parieto-occipital (3%), and in 11% there was associated periventricular grey matter heterotopia. Each of the above patterns was further divided into subtypes based on distinguishing imaging characteristics. The remaining 7% were comprised of a number of rare patterns, many not described previously. The most common clinical sequelae were epileptic seizures (78%), global developmental delay (70%), spasticity (51%) and microcephaly (50%). Many patients presented with neurological or developmental abnormalities prior to the onset of epilepsy. Patients with more extensive patterns of polymicrogyria presented at an earlier age and with more severe sequelae than those with restricted or unilateral forms. The median age at presentation for the entire cohort was 4 months with 38% presenting in either the antenatal or neonatal periods. There were no significant differences between the prevalence of epilepsy for each polymicrogyria pattern, however patients with generalized and bilateral forms had a lower age at seizure onset. There was significant skewing towards males with a ratio of 3:2. This study expands our understanding of the spectrum of clinical and imaging features of polymicrogyria. Progression from describing imaging patterns to defining anatomoclinical syndromes will improve the accuracy of prognostic counselling and will aid identification of the aetiologies of polymicrogyria, including genetic causes.

from Brain

Remote memory deficits in transient epileptic amnesia

Transient epileptic amnesia is a form of temporal lobe epilepsy in which sufferers often complain of irretrievable loss of remote memories. We used a broad range of memory tests to clarify the extent and nature of the remote memory deficits in patients with transient epileptic amnesia. Performance on standard tests of anterograde memory was normal. In contrast, there was a severe impairment of memory for autobiographical events extending across the entire lifespan, providing evidence for the occurrence of ‘focal retrograde amnesia’ in transient epileptic amnesia. There was a milder impairment of personal semantic memory, most pronounced for midlife years. There were limited deficits of public semantic memory for recent decades. These results may reflect subtle structural pathology in the medial temporal lobes or the effects of the propagation of epileptiform activity through the network of brain regions responsible for long-term memory, or a combination of these two mechanisms.

from Brain

Genetic mutations and aminoglycoside-induced ototoxicity in neonates

The prevalence of 12S rRNA mutations related to aminoglycoside ototoxicity in our study population was approximately one percent. Most patients with this mutation and aminoglycoside exposure showed no evidence of hearing loss. Low birth weight was one risk factor related to the presence of failing a hearing assessment.

http://www.otojournal.org/article/PIIS0194599810001348/abstract?rss=yes

Vestibular evoked myogenic potentials: Past, present and future

Since the first description of sound-evoked short-latency myogenic reflexes recorded from neck muscles, vestibular evoked myogenic potentials (VEMPs) have become an important part of the neuro-otological test battery. VEMPs provide a means of assessing otolith function: stimulation of the vestibular system with air-conducted sound activates predominantly saccular afferents, while bone-conducted vibration activates a combination of saccular and utricular afferents. The conventional method for recording the VEMP involves measuring electromyographic (EMG) activity from surface electrodes placed over the tonically-activated sternocleidomastoid (SCM) muscles. The “cervical VEMP” (cVEMP) is thus a manifestation of the vestibulo-collic reflex. However, recent research has shown that VEMPs can also be recorded from the extraocular muscles using surface electrodes placed near the eyes. These “ocular VEMPs” (oVEMPs) are a manifestation of the vestibulo-ocular reflex. Here we describe the historical development and neurophysiological properties of the cVEMP and oVEMP and provide recommendations for recording both reflexes. While the cVEMP has documented diagnostic utility in many disorders affecting vestibular function, relatively little is known as yet about the clinical value of the oVEMP. We therefore outline the known cVEMP and oVEMP characteristics in common central and peripheral disorders encountered in neuro-otology clinics.

from Clinical Neurophysiology

Vestibular evoked myogenic potentials: Past, present and future

Since the first description of sound-evoked short-latency myogenic reflexes recorded from neck muscles, vestibular evoked myogenic potentials (VEMPs) have become an important part of the neuro-otological test battery. VEMPs provide a means of assessing otolith function: stimulation of the vestibular system with air-conducted sound activates predominantly saccular afferents, while bone-conducted vibration activates a combination of saccular and utricular afferents. The conventional method for recording the VEMP involves measuring electromyographic (EMG) activity from surface electrodes placed over the tonically-activated sternocleidomastoid (SCM) muscles. The “cervical VEMP” (cVEMP) is thus a manifestation of the vestibulo-collic reflex. However, recent research has shown that VEMPs can also be recorded from the extraocular muscles using surface electrodes placed near the eyes. These “ocular VEMPs” (oVEMPs) are a manifestation of the vestibulo-ocular reflex. Here we describe the historical development and neurophysiological properties of the cVEMP and oVEMP and provide recommendations for recording both reflexes. While the cVEMP has documented diagnostic utility in many disorders affecting vestibular function, relatively little is known as yet about the clinical value of the oVEMP. We therefore outline the known cVEMP and oVEMP characteristics in common central and peripheral disorders encountered in neuro-otology clinics.

from Clinical Neurophysiology

Brain Imaging Findings in Dyslexia

Dyslexia is a brain-based disorder that has been intensively studied in the Western world for more than a century because of its social burden. However, affected individuals in Chinese communities are neither recognized nor formally diagnosed. Previous studies have concentrated on the disadvantages of reading deficits, and few have addressed non-linguistic skills, which are included in the symptoms. In addition, certain dyslexics possess visual spatial talents that have usually been ignored. In this review, we discuss the available information regarding brain imaging studies of dyslexia based on studies in Caucasian subjects. Gray matter deficits have been demonstrated in dyslexics using structural magnetic resonance imaging. Reduced neural activities in the left temporal and left parietal cortices, and diffuse widespread activation patterns in the cerebellum could be detected using functional magnetic resonance imaging. Changes in lactate levels, N-acetylaspartate/choline-containing compounds and N-acetylaspartate/creatine ratios, and phosphomonoester peak area were detected in magnetic resonance spectroscopy studies. Lower fractional aniso tropy values in bilateral white matter tracts have been demonstrated by diffusion tensor imaging. Abnormal Broca’s area activation was found using positron emission tomography imaging. Increased activities in the right frontal and temporal brain regions were detected using electroencephalography. Reduced hemispheric asymmetry and increased left inferior frontal activation were reported following magnetoencephalography. Although these imaging modalities are not currently diagnostic or prognostic, they are able to provide information on the causes of dyslexia beyond what was previously provided by behavioral or cognition studies.

from Pediatrics and Neonatology

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Defining Dyslexia

In 2007, the New Zealand Ministry of Education formally recognized the condition of dyslexia for the first time and has subsequently developed a working definition of the condition. The aim of this article is to draw on contemporary theory and research on reading development, reading difficulties, and reading intervention to describe what the authors believe are four key components of a definition of dyslexia/reading disability.They begin by discussing some preliminary factors that need to be considered in developing a definition of dyslexia. The authors then present the four components of their proposed definition, drawing on a framework for conceptualizing reading difficulties derived from the simple view of reading. They conclude by comparing their definition of dyslexia with the working definition put forward by the ministry.

from the Journal of Learning Disabilities

One School’s Implementation of RTI With English Language Learners: “Referring Into RTI”

The purpose of this study was to determine how a response-to-intervention (RTI) model was implemented with a large percentage of Latino English language learners who were having reading difficulties in an urban elementary school at the primary level (K—2). The authors sought to describe school personnel’s perceptions of RTI, what the model looked like in their school, and the challenges they faced. The authors focused on how teachers’ understandings, beliefs, judgments, professional development, and training affected the RTI decision-making process by investigating classroom-based literacy instruction and problem-solving meetings. This study contributes to the literature by presenting a qualitative, in-depth description of how teachers implemented an RTI model for English language learners. These themes were intertwined and functioned as a negative cycle that created a deficits-based RTI literacy model.

from the Journal of Learning Disabilities