Quantitative insights on de/repolymerization and deoxygenation of lignin in subcritical water.

The objective of the study was to investigate alkali lignin polymerization/depolymerization pathways in subcritical water (SW) without additives. Following a SW treatment at 200, 250, 275 and 300 °C, the products were subjected to a comprehensive suite of analyses addressing the product speciation and molecular weight (MW) distribution. The MW reduction (1.

Simple and sensitive dilute-and-shoot analysis of carotenoids in human plasma.

Carotenoids in human plasma are used as biomarkers of vegetable and fruit intake. Large sample volumes and intensive sample processing make measurement of these species cumbersome. We developed a dilute-and-shoot method for the quantitation of α-carotene, β-carotene, β-cryptoxanthin, lycopene and lutein/zeaxanthin using 10 μL of plasma.

Biodegradation of lignin by fungi, bacteria and laccases.

Indulin AT biodegradation by basidiomycetous fungi, actinobacteria and commercial laccases was evaluated using a suite of chemical analysis methods. The extent of microbial degradation was confirmed by novel thermal carbon analysis (TCA), as the treatments altered the carbon desorption and pyrolysis temperature profiles in supernatants. Laccase treatments caused only minor changes, though with increases occurring in the 850°C and char precursor fractions.

Improving clinical cognitive testing: report of the AAN Behavioral Neurology Section Workgroup.

Objective: To evaluate the evidence basis of single-domain cognitive tests frequently used by behavioral neurologists in an effort to improve the quality of clinical cognitive assessment.

Methods: Behavioral Neurology Section members of the American Academy of Neurology were surveyed about how they conduct clinical cognitive testing, with a particular focus on the Neurobehavioral Status Exam (NBSE). In contrast to general screening cognitive tests, an NBSE consists of tests of individual cognitive domains (e.

Pelizaeus-Merzbacher syndrome: neurocognitive function in a family with carrier manifestations.

A Cajun kindred with Pelizaeus-Merzbacher disease was found to have a p.Q128X mutation in exon 3B of proteolipid protein 1 (PLP1). The affected males were globally delayed in development, nonambulatory, and severely dysarthric.

Altered neurodevelopment associated with mutations of RSK2: a morphometric MRI study of Coffin-Lowry syndrome.

Coffin-Lowry syndrome (CLS) is a rare form of X-linked mental retardation caused by mutations of the RSK2 gene, associated with cognitive impairment and skeletal malformations. We conducted the first morphometric study of CLS brain morphology by comparing brain volumes from two CLS families with healthy controls. Individuals with CLS consistently showed markedly reduced total brain volume.

Allan-Herndon-Dudley syndrome and the monocarboxylate transporter 8 (MCT8) gene.

Allan-Herndon-Dudley syndrome was among the first of the X-linked mental retardation syndromes to be described (in 1944) and among the first to be regionally mapped on the X chromosome (in 1990). Six large families with the syndrome have been identified, and linkage studies have placed the gene locus in Xq13.2.

Psychiatric implications of language disorders and learning disabilities: risks and management.

This article reviews the relationship between different learning disabilities, language disorders, and the psychiatric disorders that are commonly associated with learning disabilities and language disorder: attention-deficit hyperactivity disorder (ADHD), anxiety disorders, depression, and conduct or antisocial personality disorder. The complex associations between language disorders and specific learning disabilities--dyslexia, nonverbal learning disorder, dyscalculia--and the various psychiatric disorders are discussed. Clinical vignettes are presented to highlight the impact of these disorders on a child's social and psychological development and the importance of early recognition and treatment.

Attention-deficit hyperactivity disorder (ADHD).

Approaches to the diagnosis and treatment of attention-deficit hyperactivity disorder (ADHD) are undergoing a major change as a result of information from studies on the genetics of ADHD and the use of new neuroimaging technologies. Moreover, pharmacogenomics, although still in its infancy, will provide a basis for much more sophisticated treatment strategies for ADHD, particularly once more information is available about the genetics of ADHD. Even at this point in time, there is some pertinent information available that, although not ready for application in clinical settings, nonetheless provides a broader perspective for the clinician.

Prefrontal executive function syndromes in children.

"Executive function" is a term describing the processes required for conscious control of thought, emotion, and action that are central to the management of one's day-to-day life. Executive function is subserved by the prefrontal cortex and related subcortical structures. Disorders affecting the prefrontal cortex-subcortical system are numerous and heterogeneous, but contemporary research has begun to elucidate the mechanisms and consequences of dysfunction in various subsystems with increasing specificity.

Intensive remedial instruction for children with severe reading disabilities: immediate and long-term outcomes from two instructional approaches.

Sixty children with severe reading disabilities were randomly assigned to two instructional programs that incorporated principles of effective instruction but differed in depth and extent of instruction in phonemic awareness and phonemic decoding skills. All children received 67.5 hours of one-to-one instruction in two 50-minute sessions per day for 8 weeks.

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.

Obsessive compulsive disorder: is there an association with childhood streptococcal infections and altered immune function?

During the last few years, an increased interest in the possibility of immune mediated pathophysiology of obsessive compulsive disorder (OCD) and related disorders has been seen. In the late 1980s, the National Institute of Mental Health reported an increase of obsessive compulsive symptoms in patients with Sydenham chorea (SC). Subsequently, a precipitating streptococcal infection in children with sudden onset of OCD symptoms but no chorea led to the coining of PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcus).

On defining Sydenham's chorea: where do we draw the line?

Sydenham's chorea (SC) is a major manifestation of rheumatic fever characterized by an array of neuropsychiatric symptoms that vary in severity, timing, and character. Some of the same symptoms are seen in Tourette's syndrome and childhood-onset obsessive-compulsive disorder. Genetic vulnerability appears to play a role in all three conditions.

XLMR syndrome characterized by multiple respiratory infections, hypertelorism, severe CNS deterioration and early death localizes to distal Xq28.

We report on a family with severe X-linked mental retardation (XLMR) and progressive, severe central nervous system deterioration. Three of the five affected males died of secondary complications before the age of 10 years and none have survived past the age of 10. These complications included swallowing dysfunction and gastroesophageal reflux with secondary recurrent respiratory infections.

Developmental dyslexia: a motor-articulatory feedback hypothesis.

Reading is mediated by parallel and widely distributed modular systems. There are, therefore, multiple loci in these systems where dysfunction may lead to developmental dyslexia. However, most normal children learn to read using the alphabetic system.

Attention deficit-hyperactivity disorder and asymmetry of the caudate nucleus.

The neurologic basis of attention deficit-hyperactivity disorder (ADHD) is poorly understood. Based on previous studies that have implicated metabolic deficiencies in the caudate-striatal region in ADHD, we employed magnetic resonance imaging to investigate patterns of morphology of the head of the caudate nucleus in normal and ADHD children. In normal children, 72.

Anomalous cerebral structure in dyslexia revealed with magnetic resonance imaging.

Objective: To develop quantitative methods for identifying cerebral anomalies on magnetic resonance images of subjects with language disorders and other learning disabilities.

Design: Partially blinded comparison of subjects with dyslexia, unaffected relatives, and a control group balanced for age and socioeconomic status. Criterion standard: clinical diagnosis of dyslexia by physician or learning disabilities specialist on the basis of clinical assessment and family history.

Angelman and Prader-Willi syndrome: a magnetic resonance imaging study of differences in cerebral structure.

Recent improvements in magnetic resonance imaging techniques now allow the developing brain to be visualized in sufficient detail to perform "in vivo neuropathology." In this study we compared the cortical morphology in six children with Angelman and four with Prader-Willi syndrome. These two syndromes are of special interest because, although they are both caused by deletions in the same region of chromosome 15, Angelman children are far more severely affected, and do not speak.

Phonological awareness training and remediation of analytic decoding deficits in a group of severe dyslexics.

The goal of the present study was to evaluate the effectiveness of the Auditory Discrimination in Depth Program (ADD) in remediating the analytic decoding deficits of a group of severe dyslexics. A group of ten severely dyslexic students ranging in age from 93 to 154 months were treated in a clinic setting for 38 to 124 hours (average of 65 hours). Pre- and post-treatment testing was done with the Woodcock Reading Mastery Test and the Lindamood Auditory Conceptualization to assess changes in phonological awareness and analytic decoding skills.

A possible pathophysiologic substrate of attention deficit hyperactivity disorder.

The attention deficit hyperactivity disorder (ADHD) is associated with defective attention and response inhibition and motor restlessness. Inattention, defective response inhibition, and impersistence are more commonly seen in adults with right than with left hemisphere dysfunction. In light of this fact and because children with ADHD not only appear to demonstrate these symptoms but also neglect the left side and have decreased activation of their right neostriatum, we propose that these children have a right hemisphere dysfunction.