Neuropathological findings in eight children with cerebro-oculo-facio-skeletal (COFS) syndrome

Cerebro-oculo-facial-skeletal (COFS) syndrome is a rare autosomal recessive disorder with microcephaly, severe mental retardation, and death in childhood. The pathogenesis is unknown. Neuropathological features of 8 children with COFS syndrome are presented. Seven of the children, ranging in age from 36 weeks gestation to 5 years 8 months, are of North American aboriginal background from Manitoba, Canada. The eight child is a 3-year-old Caucasian male. In all children there was severe microencephaly and mild ventriculomegaly. Cerebral myelination appeared to be delayed in one infantile case. Swollen ubiquitinated granular cells appeared in the white matter shortly after birth. Older children displayed cortical neuron loss, patchy or diffuse absence of myelin and gliosis in the white matter, and pericapillary and parenchymal mineralization in the globus pallidus and to a lesser extent the putamen and cerebral cortex. The cerebellum of older children exhibited severe degenerative changes involving the internal granular layer and Purkinje cell layer. The neuropathological changes, previously not well documented, suggest that COFS syndrome is associated with a degenerative process that begins in utero and affects many brain cell types. Similarities to Cockayne syndrome are discussed.     

Magnetic resonance imaging and behavioral analysis of immature rats with kaolin-induced hydrocephalus: pre- and postshunting observations

The motor and cognitive dysfunction associated with hydrocephalus remains a clinical problem in children. We hypothesized that young rats with hydrocephalus should exhibit similar dysfunction and that the dysfunction should be reversible by shunting. Hydrocephalus was induced in 3-week-old rats by injection of kaolin into the cisterna magna. Rats were assessed by T2-weighted images obtained with a 7-T magnetic resonance device and by repeated behavioral testing including ability to traverse a narrow beam and ability to find a hidden platform in a water pool. Some of the rats underwent a shunting procedure 1 or 4 weeks after kaolin injection. Magnetic resonance images were used to measure ventricle size. They clearly demonstrated increased signal in periventricular white matter, which corresponded to increased brain water content. A flow-void phenomenon was observed in the cerebral aqueduct. Ability to traverse the beam did not correlate with the degree of ventriculomegaly. Ability to swim to the hidden platform demonstrated a progressive impairment of learning function which may have been accentuated by motor disability. When rats were shunted after 1 week, the behavioral dysfunction was prevented. Late shunting after 4 weeks was associated with gradual recovery of the behavioral disability which was not complete after 4 weeks. We conclude that early shunting is superior to late shunting with regard to behavioral dysfunction. High-resolution MR imaging shows features in hydrocephalic rats similar to those found in hydrocephalic humans.     

A prospective study of the early postsurgical psychiatric associations of epilepsy surgery

OBJECTIVES: To examine prospectively the frequency and nature of psychiatric symptoms seen in patients during the first three months after temporal lobe surgery for chronic intractable epilepsy and in addition to study the relation between presurgical mental state, laterality of surgery, and postsurgical seizure and psychiatric course. METHOD: A consecutive series of 60 patients being assessed for temporal lobe surgery for intractable epilepsy were studied. They were interviewed before surgery and at six weeks and again at three months after operation. RESULTS: At six weeks after surgery half of those with no psychopathology preoperatively had developed symptoms of anxiety or depression and 45% of all patients were noted to have increased emotional lability. By three months after surgery emotional lability and anxiety symptoms had diminished whereas depressive states tended to persist. Patients with a left hemispheric focus were more likely to experience persisting anxiety. CONCLUSION: The early months after surgery for epilepsy are characterised by the relatively common presence of psychiatric symptoms. It is proposed that presurgical and early postsurgical neuropsychiatric involvement in programmes of surgery for epilepsy will help to improve the quality of the treatment package offered to patients.     

Changes in hospital nursing care due to the Brazilian Unified Health System

Considering changes in the Health System, this survey analyses the view of 31 nurses from a hospital institution linked to the Brazilian Unified Health System (SUS) in order to learn how they face the changes and their actions in 1995. To better understand the content of the interviews, authors use a thematic analysis proposed by BARDIN (1977). This analysis shows that some nurses recognize a different action after the SUS inclusion and others do not. However, when their actions are analysed as a whole, they are mainly directed to be a physician complementary action. The inadequate management by the nurse has contributed for a not entire nursing care. Authors suggest the management improvement of nurses in order to redirect nursing actions.     

The plasma membrane-actin linking protein, ezrin, is a glomerular epithelial cell marker in glomerulogenesis, in the adult kidney and in glomerular injury

BACKGROUND: Ezrin belongs to a family of plasma membrane-cytoskeleton linking, actin binding proteins (Ezrin-radixin-Moesin family) involved in signal transduction, growth control, cell-cell adhesion, and microvilli formation. METHODS: The expression of ezrin was examined in glomerular cells in culture, during kidney development, in the mature kidney, and in five different experimental kidney disease models in the rat. RESULTS: Ezrin was specifically expressed in glomerular epithelial cells in developing glomeruli in mature glomeruli and in glomerular epithelial cells in culture. Distinct from its other family members, moesin and radixin, which are predominantly expressed in glomerular endothelial and mesangial areas, ezrin protein (by immunohistochemistry) was specifically and exclusively modulated during podocyte injury and regeneration. Ezrin immunohistochemistry was able to visualize cell body attenuation, pseudocysts, and in particular vacuolation of injured podocytes, a feature that usually has to be identified at the ultrastructural level, and was strikingly increased in binucleated podocytes or podocytes that were partially or completely detached from the underlying GBM (frequently also binucleated). Infiltrating macrophages also express ezrin, but can easily be differentiated from podocytes by their round shape and higher level of expression. CONCLUSIONS: Ezrin likely has a role in the cytoskeletal organization, such as reassembling of acting filaments accompanying podocyte injury and regeneration. Since suitable light microscopic markers for the identification of glomerular epithelial cells are rare, ezrin may also be a useful marker for podocytes in normal and injured glomeruli.     

Effect of Explotab on the tabletability of a poorly soluble drug

OBJECTIVE: To examine what is known about engaging and maintaining suicidal individuals in treatment and what can be learned from the randomized, controlled trials of psychosocial interventions concerning the reduction of suicidal behaviours. METHOD: Review of the relevant literature. RESULTS: Treatment follow-through is low among those who attempt suicide. The evidence to date is inconclusive as to whether psychosocial interventions can reduce the repetition of suicidal behaviour. CONCLUSIONS: Certain groups may benefit from psychosocial interventions. Future research must overcome several methodological issues. As treatment relies on adequate follow-through, changes in the delivery of care may enhance treatment effectiveness.