Sprengel’s deformity and spinal dysraphism: connecting the shoulder and the spine

Background and purpose

Sprengel’s deformity, a rare congenital malformation of the scapula, may be observed in combination with spinal dysraphism. The co-occurrence of these malformations suggests an unknown shared etiology. Therefore, we reviewed the medical records of eight children presenting with both malformations and performed a review of the literature.

Patients and methods

Databases from four university medical centers were searched for children presenting between 1992 and 2012 with spinal dysraphism and a Sprengel’s deformity.

Conclusion

The combination of spinal dysraphism and Sprengel’s deformity is rare, and is associated with segmentation defects of the spine and ribs. Although the etiology of both spinal dysraphism and Sprengel’s deformity remains unclear, all deformities of the spine, ribs, and shoulder might result from a common genetic defect affecting somitogenesis.     

Cannabis use and psychiatric and cogitive disorders: the chicken or the egg?

PURPOSE OF REVIEW: Cannabis is the world's most commonly used illicit drug. In this review, we consider the recent literature on the effects of cannabis on mental health and on cognition. RECENT FINDINGS: Cannabis use in adolescence increases the risk of later schizophrenia-like psychoses, especially in genetically vulnerable individuals. Not surprisingly, patients already suffering from psychosis who use cannabis have a worse outcome than those who do not. These effects of cannabis may be consequent on its impact on the dopamine system. There is less evidence of cannabis playing an aetiological role in other mental disorders including depression, but there have been far fewer studies. Heavy cannabis use has also been shown to affect memory and learning performance, both in healthy individuals and in patients suffering from psychosis. Combined cognitive-behavioural therapy and motivational interviewing seems a promising psychological intervention to achieve a cessation of cannabis use in patients suffering from schizophrenia. SUMMARY: Further research is needed to understand the biological mechanisms underlying the effects of cannabis on mental health, but intervention strategies to help patients abstain should currently be implemented in psychiatric services, and public education campaigns should be directed at increasing awareness of the health risks of cannabis.     

Negative schizophrenic symptoms and the frontal lobe syndrome: one and the same?

The negative symptoms of schizophrenia have been considered to be a psychiatric form of the frontal lobe syndrome. However, no studies have compared these two disorders at the clinical level. In this study, 12 negative symptom schizophrenic patients and 11 patients with behavioural variant frontotemporal dementia (bv-FTD) were rated for negative symptoms and for occurrence of frontal lobe behaviours in everyday life. They were also rated for speech disorder and were given a series of executive tests. Both patient groups showed positive ratings on negative symptoms and frontal lobe behaviours in daily life; however, the schizophrenic patients had higher negative symptom scores and the bv-FTD patients had higher carer ratings on frontal behaviours in daily life. Both groups were impaired on the executive tests, but the bv-FTD patients showed significantly greater impairment on verbal fluency and a test requiring inhibition of prepotent responses. A minority of the bv-FTD patients unexpectedly showed speech abnormalities typically associated with schizophrenia. The findings indicate that the negative syndrome in schizophrenia and the frontal lobe syndrome resemble each other clinically in important respects. Some of the differences may be attributable to the additional presence of disinhibition in the frontal lobe syndrome.     

Communication in the Laboratory and Communication in the Clinic†

IT IS now 25 years since Frieda Fromm-Reichmann died—over 30 years since she published her only book, Principles of Intensive Psychotherapy. Most of those who now attend the presentations of the annual research award of the Academy or the annual lectures of the Washington School of Psychiatry, both of which are named in her honor, never saw her. It seem appropriate to ask whether she still speaks to us today, whether in any significant way she influenced the development of what is now common practice in our field.     

What are the computations of the cerebellum,the basal ganglia and the cerebral cortex?

The classical notion that the cerebellum and the basal ganglia are dedicated to motor control is under dispute given increasing evidence of their involvement in non-motor functions. Is it then impossible to characterize the functions of the cerebellum, the basal ganglia and the cerebral cortex in a simplistic manner? This paper presents a novel view that their computational roles can be characterized not by asking what are the “goals” of their computation, such as motor or sensory, but by asking what are the “methods” of their computation, specifically, their learning algorithms. There is currently enough anatomical, physiological, and theoretical evidence to support the hypotheses that the cerebellum is a specialized organism for supervised learning, the basal ganglia are for reinforcement learning, and the cerebral cortex is for unsupervised learning.This paper investigates how the learning modules specialized for these three kinds of learning can be assembled into goal-oriented behaving systems. In general, supervised learning modules in the cerebellum can be utilized as “internal models” of the environment. Reinforcement learning modules in the basal ganglia enable action selection by an “evaluation” of environmental states. Unsupervised learning modules in the cerebral cortex can provide statistically efficient representation of the states of the environment and the behaving system. Two basic action selection architectures are shown, namely, reactive action selection and predictive action selection. They can be implemented within the anatomical constraint of the network linking these structures. Furthermore, the use of the cerebellar supervised learning modules for state estimation, behavioral simulation, and encapsulation of learned skill is considered. Finally, the usefulness of such theoretical frameworks in interpreting brain imaging data is demonstrated in the paradigm of procedural learning.     

Validity of auditory perceptual assessment of velopharyngeal function and dysfunction – the VPC-Sum and the VPC-Rate

ABSTRACT

Overall weighted or composite variables for perceptual auditory estimation of velopharyngeal closure or competence have been used in several studies for evaluation of velopharyngeal function during speech. The aim of the present study was to investigate the validity of a composite score (VPC-Sum) and of auditory perceptual ratings of velopharyngeal competence (VPC-Rate). Available VPC-Sum scores and judgments of associated variables (hypernasality, audible nasal air leakage, weak pressure consonants, and non-oral articulation) from 391 5-year olds with repaired cleft palate (the Scandcleft project) were used to investigate content validity, and 339 of these were compared with an overall judgment of velopharyngeal competence (VPC-Rate) on the same patients by the same listeners. Significant positive correlations were found between the VPC-Sum and each of the associated variables (Cronbachs alpha 0.55–0.87, P < 0.001), and a moderately significant positive correlation between VPC-Sum and VPC-Rate (Rho 0.698, P < 0.01). The latter classified cases well when VPC-Sum was dichotomized with 67% predicted velopharyngeal competence and 90% velopharyngeal incompetence. The validity of the VPC-Sum was good and the VPC-Rate a good predictor, suggesting possible use of both measures depending on the objective.     

Preliminary investigations on melatonin and 5-methoxytryptophol synthesis in the pineal,retina, and Harderian gland of the mole rat and in the pineal of the mouse “eyeless”

Summary Hydroxyindole-O-methyltransferase (HIOMT) activity for the synthesis of melatonin and 5-methoxytryptophol, both 5-methoxyindoles, was measured in the pineal, the Harderian gland and the retina of the mole rat and in the pineal of the mouse eyeless. In the pineal and the Harderian gland of the mole rat a larger amount of 5-methoxytryptophol than of melatonin is synthesized. 5-Methoxyindole synthesis is extremely high in the Harderian gland, whereas in the retina HIOMT activity is low and variable. In the pineal of the mouse eyeless, a low 5-methoxyindole synthesis showing no circadian rhythm is demonstrated. It is concluded that, besides the generally accepted regulation of the indole metabolism by light, in species with atrophied eyes having Harderian glands (mole rat) and in species without eyes other factors than light might be responsible for the indole metabolism in the pineal gland.     

Do the French and the English differ for hand skill asymmetry? Handedness subgroups in the sample of Doyen and Carlier (2002) and in English schools and universities

Doyen and Carlier (2002) reported peg-moving performance in a sample of French adults classified in the hand preference subgroups of Annett (1970, 1985). The findings were said to differ from those for English samples. This paper describes new analyses of English data, drawn from schools and universities. Comparison of the school and university samples showed that relationships between hand skill asymmetry and subgroup handedness were reliable, but also revealed a new finding. Asymmetries were stronger in the school than university samples at all levels of hand preference. The French findings resembled those for the English university sample for all measures and for all subgroups except right-handed writers with strong left tendencies. The performance of the latter in the French sample was like that of left-handers rather than right-handers. An explanation might be that some members of this group were left-handers who had been forced to use the right hand for writing.     

Psychodynamics and managed care: the art of the impossible?

Many mental health providers believe that psychodynamic psychotherapy under managed care is virtually impossible. Notwithstanding the many adversities posed by managed care, there are ways to productively apply psychodynamic principles within this health care financing system. This paper critically discusses the possibilities and costs of conducting psychodynamic psychotherapy under managed care using theory, practice applications, and a case vignette. Central to the discussion is elaboration of five central differences between traditional psychodynamic treatment versus that conducted under managed care.     

Diuretics and epilepsy: Will the past and present meet?

Clinical studies from over half a century ago suggested efficacy of a variety of diuretics in focal and generalized epilepsies as well as in status epilepticus, but these findings have not been translated into modern epilepsy training or practice. Recent advances in our understanding of neuronal maturation and the pathophysiology of neonatal seizures provide fresh insight into the mechanisms by which diuretics might reduce susceptibility to seizures. In vitro and in vivo rodent studies and human epilepsy surgical cases have shown that specific diuretic agents targeting the cation-chloride cotransporters decrease neuronal synchrony and neuronal hyperexcitability. These agents are thought to convey their antiepileptic activity by either expanding the extracellular space or promoting a cellular chloride transport balance that reflects a more developmentally "mature," less excitable state. It may be time to reexamine whether diuretics could serve as adjunctive therapies in the treatment of refractory epilepsies.