Late symptomatic initiation of unilateral cerebellar hypoplasia

A 65-year-old woman developed a progressive action tremor on her left hand for the last two years. On examination an intentional left hand tremor with dysmetria and tendency to deviate to left on tandem were identified. There was no mental deterioration. CT scan and MR imaging disclosed a marked unilateral, left, cerebellar hypoplasia. MR also showed multiple lacunae. Neurological family disease and exposition to drugs or alcohol were not present. In some cases cerebellar hypoplasia could become symptomatic at advanced age as result of concurrent cerebral disease affecting the compensating structures for congenital defect. In this patient a vascular mechanism is suggested as possible late trigger.     

Computed tomography in children with cystic fibrosis: a new way to reduce radiation dose.

AIMS: To determine whether the monitoring of respiratory disease progression in children with cystic fibrosis (CF) can be made using six pre-selected computed tomography (CT) cuts in lieu of the conventional full study. METHODS: Forty one lung CT scans from 21 paediatric patients with CF were analysed. The Bhalla and Nathanson scores of the total lung CT and the six pre-selected CT cuts were compared. RESULTS: The Bhalla mean score of the total lung CT evaluated by two radiologists was 5.62. It was 5.36 when just the six pre-selected sections were evaluated. The difference between means was not statistically significant. The Nathanson mean score of the total lung CT evaluated by both radiologists was 66.11; it was 66.51 when just the six pre-selected sections were evaluated. The difference between means was not statistically significant. The mean total radiation dose from a single whole lung CT scan was 716.22 mGy.cm. A dose of 250.66 mGy.cm was estimated if only six sections were used, with a reduction in radiation of about 65%. CONCLUSION: It is possible to obtain the same radiological information from six pre-selected CT cuts as it is from a full pulmonary CT scan, thereby markedly reducing radiation exposure for children who will require repeat investigations in the future.     

Cochlear implant outcomes and quality of life in the elderly: Manchester experience over 13 years1

Objectives: To objectively evaluate the clinical and functional outcomes of cochlear implantation in an elderly population. Design: Retrospective comparative study. Setting: Neurotology unit at Manchester Royal Infirmary, a supraregional tertiary referral centre in collaboration with Adult Cochlear Implant Programme at The University of Manchester. Participants: All cochlear implant procedures (38) undertaken on post‐lingually deafened elderly patients (age range at the time of implantation 65–80 years, n = 34) in the period from 1989 to 2002. Main outcome measures: Medical and surgical outcomes. Audiological performance outcomes for isolated words, words in sentences in quiet and noise. Functional outcome measures used are self‐reported measures of the social, psychological and emotional aspects of quality of life, and the differences between expectations for functional outcomes and the realization of functional outcomes. They included expectation profiles, Glasgow Benefit inventory (GBI) and Glasgow Health Status Inventory Questionnaire (GHSI). Results: There was statistically significant improvement post‐implantation of both open and closed set test scores (P < 0.01). Eighty‐two percentage of patients were completely satisfied with their cochlear implants. Patients judged that implantation restored half the loss of quality of life that they had experienced as a result of severe‐profound deafness with a highly significant (P < 0.001) improvement in overall quality of life after implantation. The commonest post‐operative observation was transient mild pyrexia. Conclusions: The age of a cochlear implant candidate should not be a factor in the candidacy decision‐making process. The quality of life of our elderly recipients was significantly improved after cochlear implant.     

Fast blue and diamidino yellow as retrograde tracers in peripheral nerves: efficacy of combined nerve injection and capsule application to transected nerves in the adult rat

Capsule application of Diamidino Yellow (DY) to the cut end of the sciatic nerve immediately followed by capsule application of Fast Blue (FB) resulted in approximately 95% double-labelled dorsal root ganglion neurones (DRGn) and motoneurones (Mn). Nerve injection of DY followed either immediately or 2 months later by capsule application of FB resulted in approximately 90% double-labelled DRGn and Mn, indicating that DY and FB label similar populations of DRGn and Mn, and that insignificant DY fading occurred during this period. Inversing the order of application, however, i.e. nerve injection of FB followed immediately by capsule application of DY, resulted in double labelling in only approximately 10% of the DRGn and Mn. These percentages increased to 70% of the DRGn and 60% of the Mn when the FB injection was followed 1 or 2 months after by the DY application, indicating that DY uptake is blocked by recent administration of FB. The results indicate that DY and FB might be useful for sequential labelling before and after nerve injury as a tool to investigate the accuracy of sensory and motor regeneration.     

Meconium and neurotoxicants: searching for a prenatal exposure timing.

BACKGROUND: Exposure to organochlorine compounds (OCs) has been a subject of interest in recent years, given their potential neurotoxicity. Meconium is easily available and accumulates neurotoxicants and/or metabolites from the 12th week of gestation. AIMS: To determine whether neurotoxicants, specifically OCs, could be detected in serially collected meconium, and to compare the results with those obtained in cord blood samples. METHODS: A sample of cord blood and three serial stool samples were analysed in 10 newborns. Pentachlorobenzene (PeCB), hexachlorobenzene (HCB), polychlorinated biphenyls (PCBs), dichlorodiphenyl trichloroethane (p,p'-DDT) and its metabolite dichlorodiphenyl dichloroethylene (p,p'-DDE), and hexachlorocyclohexane isomers (alpha-, beta-, gamma-, and delta-HCH) were analysed by gas chromatography. RESULTS: From serial stool collection and analysis in newborns, there was an increase in the concentrations of HCB, p,p'-DDE, PCBs, and beta-HCH between the first and last stools of the newborn. Levels of DDT diminished as pregnancy progressed. Concentrations in cord blood were positively associated with concentrations in meconium for p,p'-DDE and beta-HCH. CONCLUSIONS: Meconium is a very useful instrument for the investigation of fetal exposure to neurotoxicants; serial collection and analysis of meconium should estimate the timing and degree of in utero exposure of the fetus to neurotoxicants. Analysis and interpretation of neurotoxicants in meconium results is a complex process. Measurement in meconium of a wide range of neurotoxic substances should facilitate early identification of harmful exposures, and enable rehabilitation and instigation of preventive measures.     

TV watching modifies obesity risk linked to the 27Glu polymorphism of the ADRB2 gene in girls.

OBJECTIVE: A matched case-control study was conducted in a population of Spanish children and adolescents (5-18 years old), to assess the interaction between the Gln27Glu polymorphism of the ADRB2 and television (TV) watching on obesity risk. PATIENTS: Obese (n=165) and control subjects (n=165) matched by sex and age were recruited and classified according to Spanish reference data. Results. Using conditional logistic regression, we calculated the obesity risk linked to the polymorphism. A statistically significant association was found for 27Glu carrier allele girls (OR = 1.95; 95% CI = 1.02-3.70), but no association was apparent among boys. In the fully adjusted model, the odds ratio for obesity linked to the genotype Glu27Glu in the female population rose to 4.84 (95% CI = 1.37-17.10). Moreover, we found a significant negative interaction between hours of TV watching and the Gln27Glu polymorphism for obesity risk in girls. Surprisingly, among 27Glu carrier subjects, even girls with a low level of TV watching ( < 12.5 h/week) had a high obesity risk (OR = 4.60; 95% CI = 1.01-20.02), which was not very different to the odds ratio values for sedentary girls carrying the 27 Glu allele watching TV more than 12.5 h/week (OR = 6.05; 95% CI = 1.31-27.71). Conclusion. A higher risk of obesity was found for girls carrying the 27Glu allele of the ADRB2 gene even when they spent less than 12.5 h/week watching TV. In addition, our results suggest that the effect of sedentary lifestyle on obesity risk may depend on the genotype of the subject.     

Sensory and motor aspects of the coital reflex in the spinal male rat

In the present study the sensory and motor aspects of the coital reflex model in male rats were evaluated. Electromyographic reflex activity after mechanical stimulation of the urethra, penis and scrotal skin was recorded in all genital muscles. The possibility that a facilitatory mechanism of these muscular responses was located in the rat spinal cord was evaluated by removing the genital afferents. Results showed that urethral, penile and scrotal stimulation evoked the coital reflex in all genital muscles. Similarly, coital responses were obtained spontaneously in deafferentated animals. The parameters among the motor patterns evoked with the different mechanical stimuli were very similar. Parameters of spontaneous motor patterns were not importantly different from those obtained reflexively. A conspicuous difference between these responses was the presence of an after-discharge activity in genitally-stimulated animals. Additionally, it was found that this motor pattern can be exhausted with repeated stimulation. Spontaneous responses did not show the exhaustion phenomenon. Results are discussed in the context of the sensory-motor aspects of male sexual behaviour.     

Subcellular compartmentalization of a potassium channel (Kv1.4): preferential distribution in dendrites and dendritic spines of neurons in the dorsal cochlear nucleus

Voltage-dependent ion channels have specific patterns of distribution along the neuronal plasma membrane of dendrites, cell bodies and axons, which need to be unravelled in order to understand their contribution to neuronal excitability and firing patterns. We have investigated the subcellular compartmentalization of Kv1.4, a transient, fast-inactivating potassium channel, in fusiform cells and related interneurons of the rat dorsal cochlear nucleus. A polyclonal antibody which binds to a region near the N-terminus domain of a Kv1.4 channel was raised in rabbits. Using a high-resolution combination of immunocytochemical methods, Kv1.4 was localized mainly in the apical dendritic trunks and cell bodies of fusiform cells, as well as in dendrites and cell bodies of interneurons of the dorsal cochlear nucleus, likely cartwheel cells. Quantitative immunogold immunocytochemistry revealed a pronounced distal to proximal gradient in the dendrosomatic distribution of Kv1. 4. In plasma membrane localizations, Kv1.4 was preferentially present in dendritic spines, either in the spine neck or in perisynaptic locations, always away from the postsynaptic density. These findings indicate that Kv1.4 is largely distributed in dendritic compartments of fusiform and cartwheel cells of the dorsal cochlear nucleus. Its preferential localization in dendritic spines, where granule cell axons make powerful excitatory synapses, suggests a role for this voltage-dependent ion channel in the regulation of dendritic excitability and excitatory inputs.