Human interaction in the NICU and its association with outcomes on the Brief Infant-Toddler Social and Emotional Assessment (BITSEA).

BACKGROUND: Extremely preterm infants represent one of the highest risk categories for impairments in social competence. Few studies have explored the impact of the neonatal intensive care unit (NICU) environment on social development. However, none have specifically analyzed the effects of the care structure the infant receives during hospitalization on later social competence indicators. OBJECTIVE: To identify associations between the care structures received by extremely preterm infants in the NICU and scores on the Brief Infant-Toddler Social and Emotional Assessment (BITSEA) post-discharge. PARTICIPANTS: 50 extremely preterm infants (mean gestational age: 25 weeks during hospitalization; mean chronological age during follow-up assessment: 2 years, 4 months). METHODS: A secondary analysis of BITSEA data was performed exploring its relation to care structure data we extracted from electronic medical records (i.e., how much time infants were engaged in human interaction during their first thirty days of hospitalization and what types of interaction they were exposed to). RESULTS: Extremely preterm infants spend a considerable amount of time alone during hospitalization (80%) with nursing care comprising the majority of human interaction. Infants who experienced greater human interaction scored significantly higher on the Social Competence (p = 0.01) and lower on the Dysregulation (p = 0.03) BITSEA subscales. CONCLUSION: Human interaction and isolation in the NICU is associated with social competence and dysregulation outcomes in extremely preterm infants. Further research is needed to understand how various NICU care structures including centralized nursing teams, parental skin-to-skin care, and early therapy may synergistically play a positive role in developing social competence.

Official Position of the American Academy of Clinical Neuropsychology Social Security Administration Policy on Validity Testing: Guidance and Recommendations for Change.

UNLABELLED: The milestone publication by Slick, Sherman, and Iverson (1999) of criteria for determining malingered neurocognitive dysfunction led to extensive research on validity testing. Position statements by the National Academy of Neuropsychology and the American Academy of Clinical Neuropsychology (AACN) recommended routine validity testing in neuropsychological evaluations. Despite this widespread scientific and professional support, the Social Security Administration (SSA) continued to discourage validity testing, a stance that led to a congressional initiative for SSA to reevaluate their position. In response, SSA commissioned the Institute of Medicine (IOM) to evaluate the science concerning the validation of psychological testing. The IOM concluded that validity assessment was necessary in psychological and neuropsychological examinations (IOM, 2015 ). OBJECTIVE: The AACN sought to provide independent expert guidance and recommendations concerning the use of validity testing in disability determinations. METHOD: A panel of contributors to the science of validity testing and its application to the disability process was charged with describing why the disability process for SSA needs improvement, and indicating the necessity for validity testing in disability exams. RESULTS: This work showed how the determination of malingering is a probability proposition, described how different types of validity tests are appropriate, provided evidence concerning non-credible findings in children and low-functioning individuals, and discussed the appropriate evaluation of pain disorders typically seen outside of mental consultations. CONCLUSIONS: A scientific plan for validity assessment that additionally protects test security is needed in disability determinations and in research on classification accuracy of disability decisions.

The structural brain correlates of cognitive deficits in adults with Klinefelter's syndrome.

CONTEXT: Adults with Klinefelter's syndrome (KS) are known to present disturbances of language skills and delayed learning abilities. OBJECTIVES: The aim of this study was to assess brain morphometry in KS and to correlate eventual volumetric changes with performance on neuropsychological tests. PATIENTS: Patients included 18 KS adults and 20 age-matched controls. METHODS: All participants underwent prospectively double-spin-echo brain magnetic resonance imaging and neuropsychological testing of verbal and nonverbal domains. On the axial stack of magnetic resonance imaging slices, regional brain volumes were measured either by automated segmentation (full brain, total cerebrospinal fluid, and ventricular volume) or manual drawing with help of a neuroanatomy atlas (frontal, temporal, and parietal lobes, gray matter component of the lobes, cerebellar hemispheres, and hippocampal complexes). RESULTS: KS patients performed significantly lower than controls on language-related tasks exploring verbal processing speed and verbal executive function. They were diagnosed with significant enlargement of ventricular volume and bilateral reduction of cerebellar hemispheres. Furthermore, after separation of participants according to handedness and after correction of regional brain volumes for atrophy, a significant reduction of left temporal lobe volume was found in KS compared with controls. Ventricular volume was inversely correlated with cognitive function, whereas left temporal lobe volume was positively correlated with language-related tasks. CONCLUSION: This study hypothesizes that supernumerary X-chromosome and/or congenital hypogonadism provoke structural alterations in the subcortical pathways involved in language processing, thus providing a neurobiological substrate for cognitive deficits in KS.

Utility of clinical criteria in differentiating frontotemporal lobar degeneration (FTLD) from AD.

OBJECTIVE: To assess the ability of the current diagnostic criteria for frontotemporal lobar degeneration (FTLD) to differentiate FTLD from AD. METHODS: Thirty cases with autopsy-proven FTLD and 30 cases of AD, matched for Mini-Mental State Examination score, were identified from the clinical databases of three dementia subspecialty centers, and their charts were reviewed for the presence of clinical features described in the current criteria for FTLD. The proportion of patients with each clinical feature at the first clinical presentation was compared across groups. RESULTS: A significantly larger proportion of patients with FTLD showed behavioral abnormalities, particularly social and personal conduct disorders and emotional blunting, than patients with AD. Few differences in language features were seen between the groups, and many of the language features detailed in the criteria were found in only a small proportion of patients. In both groups, many patients showed neuropsychological abnormalities, except for perceptual difficulties, which were present in many patients with AD but only in a few patients with FTLD. Extrapyramidal motor symptoms were more likely to be present in FTLD. Logistic regression revealed that five features-social conduct disorders, hyperorality, akinesia, absence of amnesia, and the absence of a perceptual disorder-correctly classified 93% of patients with FTLD and 97% of patients with AD. CONCLUSION: A combination of behavioral, neuropsychological, and physical findings is most useful in distinguishing FTLD from AD. Future studies should be directed at establishing more objective methods of identifying these clinical features.

Narcotic withdrawal syndrome following gastric bypass--a difficult diagnosis.

BACKGROUND: It is common for bariatric patients to experience postoperative nausea, depression and remorse for several months following surgery. Difficulty exists for the surgeon in delineating the physical from the psychological in treating these patients. Preoperative education, evaluation and preparation, although essential, will not identify nor eliminate all potential problems. METHODS: We report the case history of a patient who ultimately underwent reversal of her gastric bypass. Her symptoms required multiple procedures and hospital admissions for what appeared to be anatomical problems. All procedures were done laparoscopically. RESULTS: The patient's main complaints of persistent nausea and abdominal pain combined with radiographic evidence of sub-optimal anatomic construct led to multiple operative procedures. Psychological intervention relative to the persistent nausea and abdominal pain was ineffectual, although the supportive and consistent nature of the psychotherapy relationship was useful in overall patient stability and emotional well being. Complete reversal of the bypass did not effect improvement. Ultimately, the diagnosis of narcotic withdrawal prompted the institution of methadone treatment with complete cessation of the symptoms of nausea and pain. CONCLUSIONS: The diagnosis of narcotic withdrawal syndrome can be difficult in the postoperative bariatric patient. Psychological evaluation and support are essential elements of the program throughout the entire course of a patient's treatment experience. Laparoscopic techniques simplified the surgical care of this patient.

Neuroanatomy of the self: evidence from patients with frontotemporal dementia.

OBJECTIVE: To evaluate the frequency and types of change in "self" seen in frontotemporal dementia (FTD) and to determine the relative involvement of the nondominant and dominant frontal and temporal brain regions in FTD patients with or without changes in a sense of self using neuropsychology tests and neuroimaging. BACKGROUND: The self has been defined as "the total, essential, or particular being of a person" involving "the essential qualities distinguishing one person from another." Some suggest that the frontal lobes play a dominant role in maintaining the self. FTD affects anterior frontal and temporal areas and can be associated with a loss of self. METHODS: Seventy-two consecutive FTD patients were evaluated with neuropsychiatric, neuropsychologic, and behavioral measures. Patients were imaged with MRI and SPECT. Charts were reviewed by a social psychologist to determine patients who exhibited a dramatic change in their self as defined by changes in political, social, or religious values. The brain areas with the most severe atrophy or hypoperfusion on neuroimaging were noted. RESULTS: Seven of 72 patients exhibited a dramatic change in self. In six of the seven, the selective dysfunction involved the nondominant frontal region. In contrast, only one of the other 65 patients without selective nondominant frontal dysfunction showed a change in self. CONCLUSIONS: FTD patients with asymmetric loss of function in the nondominant frontal lobe often exhibit a diminished maintenance of previously learned self-concepts despite intact memory and language. Normal nondominant frontal function is important for the maintenance of the self.

The influence of right frontotemporal dysfunction on social behavior in frontotemporal dementia.

BACKGROUND: Frontotemporal dementia (FTD) is associated with a variety of cognitive and behavioral dysfunctions. Symptoms may be influenced by the relative involvement of the right versus the left hemisphere, with left-sided FTD manifesting language changes and right-sided FTD presenting with aggressive, antisocial, and other socially undesirable behaviors. OBJECTIVE: To test the hypothesis that right-sided FTD is associated with socially undesirable behavior. METHODS: The authors assessed 41 patients with FTD diagnosed by the new research criteria for FTD(1) including behavioral, neuropsychologic, and neurologic testing as well as SPECT and MRI. Based on visual inspection of SPECT scans, 12 patients were classified as having predominantly right-sided and 19 patients were classified as having predominantly left-sided FTD. A clinician blinded to the imaging data reviewed medical records to tabulate the frequency of the following socially undesirable behaviors: criminal behavior, aggression, loss of job, alienation from family/friends, financial recklessness, sexually deviant behavior, and abnormal response to spousal crisis. RESULTS: Eleven of 12 right-sided and 2 of 19 left-sided FTD patients had socially undesirable behavior as an early presenting symptom (chi = 23.3, p < 0.001). CONCLUSION: The authors conclude that right-sided frontotemporal degeneration is associated with socially undesirable behavior. The early presence of socially undesirable behavior in FTD differentiates right-sided from left-sided degeneration. The results highlight the importance of the right hemisphere, especially frontotemporal regions, in the mediation of social behavior. The potential mechanism for these social losses with right-sided disease is discussed.

Neuropsychological profiles of adults with Klinefelter syndrome.

Children and adolescents with Klinefelter syndrome (XXY) have been reported to show deficits in language processing including VIQ < PIQ and a learning disability in reading and spelling. However, whether this is characteristic of adults with Klinefelter syndrome has not been established. Thirty-five men with Klinefelter syndrome, aged 16 to 61, and 22 controls were evaluated with a comprehensive neuropsychological battery. The Klinefelter patients scored significantly below controls in language skills, verbal processing speed, verbal and nonverbal executive abilities, and motor dexterity. Within the Klinefelter sample, three cognitive subgroups were identified: VIQ 7 or more points below PIQ (n = 10), VIQ within 6 points of PIQ (n = 12), and PIQ 7 or more points below VIQ (n = 12). The deficits detected in language, verbal processing speed, and verbal executive skills were found to be isolated to the VIQ < PIQ subgroup, while the abnormalities in motor dexterity and nonverbal executive skills were confined to the PIQ < VIQ subgroup. Older age was significantly correlated with increases in VIQ relative to PIQ in the patient group, which suggests the intriguing possibility that the PIQ < VIQ subgroup primarily emerges in young adulthood, perhaps in response to the reported hormonal abnormalities detected in Klinefelter syndrome patients during puberty.

Neuropsychological performance of right- and left-frontotemporal dementia compared to Alzheimer's disease.

The performance of 16 patients with Alzheimer's disease (AD) was compared to 11 patients with right-frontotemporal dementia (FTD) and 11 patients with left-FTD on a comprehensive neuropsychological battery. Standardized scores (i.e., z scores based on normal control data) were analyzed for 5 cognitive domains. The results revealed that the AD group displayed significant impairment in visual-constructional ability relative to the two FTD groups; however, no significant difference was found between the groups on memory scores (verbal and nonverbal). Patients with left-FTD scored significantly below patients with AD on the language measures (e.g., word retrieval, verbal semantic memory), and verbal executive ability (phonemic fluency); AD patients did not differ from patients with right-FTD on these measures. Patients with right-FTD exhibited significantly more perseverative behavior than AD patients; AD patients did not differ from left-FTD patients on this parameter. These results indicate that the pattern of neuropsychological performance of AD patients is distinguishable from patients with left and right frontal frontotemporal dementia.

Post retention: the effect of sequence of post-space preparation, cementation time, and different sealers.

Many endodontic sealers contain constituents that have been shown to inhibit the polymerization of resin cements. This may be important when prefabricated posts are cemented at the same appointment as root canal obturation. This study evaluated the effects of cementing posts with a resin cement immediately or at a delayed time period after obturation using Roth's 801 Elite Grade or AH26 sealer cements. The contribution of mechanical post-space preparation was also assessed as a critical variable. One hundred twenty extracted canines were randomly divided into eight experimental groups. The variables evaluated were the order of post preparation (either before or after obturation), the type of sealer used, and the time of post cementation. All teeth received a stainless steel #6 Parapost XP cemented with a resin cement, Panavia 21. Each experimental group underwent tensile testing for retention using an Instron universal testing machine. For both sealers posts cemented in teeth in which the canal was obturated before post-space preparation and thus had sealer-contaminated dentin removed by the space preparation procedure had significantly higher retentive values than those obturated after post-space preparation in which contaminated dentin might remain. Sealer used and time of cementation had no specific effect on retention. Achieving a clean, "freshened" dentinal surface during mechanical post-space preparation seems to be a critical variable for post retention when a resin cement is used.

Laparoscopic Roux-en-Y gastric bypass: technique and 3-year follow-up.

BACKGROUND: The purpose of this study is to evaluate our experience with the laparoscopic gastric bypass. The technique, weight loss data, and complications are described. METHODS: 1,500 consecutive patients were evaluated prospectively. All patients met NIH criteria for bariatric surgery. Although there have been modifications with respect to staplers, suture material, and dissection techniques, the basic anatomical construct has remained the same, including performing a completely hand-sewn gastrojejunostomy. RESULTS: There were no anastomotic leaks from the hand-sewn gastrojejunostomy. Operative times now are consistently 60 minutes or less, although the learning curve is quite long. Average hospital stay was 1.5 days. Average excessive weight loss was 69% at one and two years and 62% at three years. Overall complication rate was 14.8%. Perioperative death rate was 0.2%. CONCLUSIONS: The laparoscopic gastric bypass is a viable alternative to traditional open techniques. It is as safe and effective and can be performed with equal or greater efficiency. Adoption of hand-suturing techniques helps to improve the surgeon's skill and ability to cope with the occasional stapler misfire or complication.

Molecular, biochemical and functional analysis of a novel and developmentally important fibrillar collagen (Hcol-I) in hydra.

The body wall of hydra (a member of the phylum Cnidaria) is structurally reduced to an epithelial bilayer with an intervening extracellular matrix (ECM). Previous studies have established that cell-ECM interactions are important for morphogenesis and cell differentiation in this simple metazoan. The ECM of hydra is particularly interesting because it represents a primordial form of matrix. Despite progress in our understanding of hydra ECM, we still know little about the nature of hydra collagens. In the current study we provide a molecular, biochemical and functional analysis of a hydra fibrillar collagen that has similarity to vertebrate type I and type II collagens. This fibrillar collagen has been named hydra collagen-I (Hcol-I) because of its structure and because it is the first ECM collagen to be identified in hydra. It represents a novel member of the collagen family. Similar to vertebrate type I and II collagens, Hcol-I contains an N-terminal propeptide-like domain, a triple helical domain containing typical Gly-X-Y repeats and a C-terminal propeptide domain. The overall identity to vertebrate fibrillar collagens is about 30%, while the identity of the C-terminal propeptide domain is 50%. Because the N-terminal propeptide domain is retained after post-translational processing, Hcol-I does not form thick fibers as seen in vertebrates. This was confirmed using transmission electron microscopy to study rotary shadow images of purified Hcol-I. In addition, absence of crucial lysine residues and an overall reduction in proline content, results in reduced crosslinking of fibrils and increased flexibility of the molecule, respectively. These structural changes in Hcol-I help to explain the flexible properties of hydra ECM. Immunocytochemical studies indicate that Hcol-I forms the 10 nm fibrils that comprise the majority of molecules in the central fibrous zone of hydra ECM. The central fibrous zone resides between the two subepithelial zones where hydra laminin is localized. While previous studies have shown that basal lamina components like laminin are expressed by the endoderm, in situ hybridisation studies show that Hcol-I mRNA expression is restricted to the ectoderm. Hcol-I expression is upregulated during head regeneration, and antisense studies using thio-oligonucleotides demonstrated that blocking the translation of Hcol-I leads to a reversible inhibition of head morphogenesis during this regenerative process. Taken in total, the data presented in this study indicate that Hcol-I is required for morphogensis in hydra and represents a novel fibrillar collagen whose structural characteristics help to explain the unique biophysical properties of hydra ECM. Interestingly, the structure of Hcol-I mimics what is seen in Ehlers-Danlos syndrome type VII in humans; an inherited pathological condition that leads to joint and skin abnormalities. Hcol-I therefore illustrates an adaptive trait in which the normal physiological situation in hydra translates into a pathological condition in humans.

Laparoscopic Roux-en-Y gastric bypass for morbid obesity: technique and preliminary results of our first 400 patients.

HYPOTHESIS: A technique of the laparoscopic Roux-en-Y gastric bypass can be developed that is safe, effective, and practical in the community setting. DESIGN: A case series of 400 morbidly obese and superobese individuals who underwent the laparoscopic Roux-en-Y gastric bypass over a 22-month period. SETTING: Community private practice in Fresno, Calif. PATIENTS: A consecutive sample of 400 patients (70 males and 330 females) who met National Institutes of Health criteria for recommendation of a bariatric procedure. Only patients who had a previous gastric or bariatric procedure were excluded from this sample. INTERVENTION: Laparoscopic Roux-en-Y gastric bypass with a hand-sewn gastrojejunal anastomosis. MAIN OUTCOME MEASURES: Weight loss, complications, length of hospital stay, successful completion of the operation, and operative times were measured. RESULTS: Open conversion was required in 12 patients (6 males and 6 females) and a secondary operation for incomplete division of the stomach was required in 2 patients early in the case series. Alternative exposure and fixation techniques greatly reduced these occurrences. There were 6 staple-line failures owing to a change in the manufacture of the instrument. There were no leaks at the gastrojejunal anastomosis, but 21 patients required endoscopic balloon dilation for significant stenosis. The average hospital stay was 1.6 days for the patients who underwent laparoscopy and 2.7 days for patients requiring open conversion. Average excessive weight loss was 69% at 12 months. Operative times are between 60 and 90 minutes. Other complications are described. CONCLUSION: The Roux-en-Y gastric bypass can be safely and effectively performed in the community setting using advanced laparoscopic techniques.

First symptoms--frontotemporal dementia versus Alzheimer's disease.

Frontotemporal dementia (FTD) is often misdiagnosed as Alzheimer's disease (AD). We hypothesized that the first symptoms associated with FTD would be different from those seen in AD and that the first symptoms in FTD would reflect loss of function in the frontal region with the greatest degree of degeneration. The objective of the study was to compare the earliest symptoms in patients with FTD and AD, and to delineate the symptoms that were associated with right, left or bilateral frontotemporal degeneration in FTD. The first symptoms in 52 FTD and 101 AD patients were determined in retrospect. Based on functional imaging studies, the FTD patients were divided into those with predominantly bilateral (n = 15), left-sided (n = 19) and right-sided (n = 18) patterns of atrophy. The results showed that disinhibition, social awkwardness, passivity and loss of executive function were more common in FTD, while memory loss was more common in AD. Disinhibition was greatest in the asymmetric right-sided group, language dysfunction was commonest in the asymmetric left-sided group and loss of executive function was most frequent in the bilateral group. In summary, different first symptoms appeared in FTD and AD, which may help distinguish between the diseases. The anatomic site for FTD largely determined the kind of first symptoms.

Functional correlates of musical and visual ability in frontotemporal dementia.

BACKGROUND: The emergence of new skills in the setting of dementia suggests that loss of function in one brain area can release new functions elsewhere. AIMS: To characterise 12 patients with frontotemporal dementia (FTD) who acquired, or sustained, new musical or visual abilities despite progression of their dementia. METHOD: Twelve patients with FTD who acquired or maintained musical or artistic ability were compared with 46 patients with FTD in whom new or sustained ability was absent. RESULTS: The group with musical or visual ability performed better on visual, but worse on verbal tasks than did the other patients with FTD. Nine had asymmetrical left anterior dysfunction. Nine showed the temporal lobe variant of FTD. CONCLUSION: Loss of function in the left anterior temporal lobe may lead to facilitation of artistic or musical skills. Patients with the left-sided temporal lobe variant of FTD offer an unexpected window into the neurological mediation of visual and musical talents.

Neurobehavioral phenotype of Klinefelter syndrome.

A defined genetic syndrome with neurobehavioral components offers an unusual paradigm for the correlation of genetic defects with neurodevelopmental abnormalities. The power of the combination of detailed behavioral, neuroanatomical, and genetic studies has been demonstrated in studies of other conditions involving the sex chromosomes, such as Fragile X syndrome (Mazzocco [2000] Ment Retard Develop Disabil Res Rev. 6:96-106) and Turner syndrome (Ross [2000] Ment Retard Develop Disabil Res Rev. 6:135-141). Although the behavioral and neurologic difficulties that have been identified in Klinefelter syndrome (KS) are in most cases milder than the consequences of many other genetic syndromes, the deficits in KS cause significant morbidity, representing a more common, but poorly understood, subtype of those with learning disabilities. Both as children and as adults, KS subjects appear to offer a powerful genetic model for the study of language and language-based learning disabilities. Although it has been proposed that the language-based learning difficulties of KS boys are similar to those of nonaneuploidic dyslexics [Bender et al., 1986; Geschwind et al., 1998], this is not yet well established. The co-morbid frontal-executive dysfunction observed in KS is also a likely contributor to learning difficulties and, perhaps, social cognition, in many KS patients. It is also proposed that altered left-hemisphere functioning, whether causing, or due to, altered functional and anatomical cerebral dominance, is at the core of KS subjects' language problems. Although X chromosomal loci can provide only part of the picture, the study of KS subjects, a population with a relatively homogeneous etiology for dyslexia/dysphasia and frontal-executive dysfunction, offers many advantages over such a study in the general population, in which both dyslexia and attentional disorders are quite genetically heterogeneous [Decker and Bender, 1988; Pennington, 1990; Grigorenko et al., 1997; Geschwind et al., 1998]. Furthermore, the interaction of genetic factors and hormonal influences in the cognitive phenotypes described remains an unexplored area for future investigation. MRDD Research Reviews 2000;6:117-124.

Electronic memory aids for outpatient brain injury: follow-up findings.

The introduction of highly portable computers extends the range of tools potentially useful to persons with functional impairments of prospective memory resulting from brain injury. This study reviews the experience of 12 patients with brain injury undergoing outpatient treatment using palmtop computers to assist with memory dependent activities in their everyday lives. During the initial supervised trial period, each was provided a palmtop computer based memory aid capable of generating audible and visible reminder cues. Subsequently, patients were contacted for follow-up between 2 months and 4 years after initial trial usage, and surveyed as to the utility of the computer. Nine patients found palmtop computers were useful during supervised trials. Seven of nine patients actually continued to use such devices after the usage trials had ended. Experience with this technology has shown it to be useful in a high proportion of patients for assisting with memory dependent functions.