Literature search from ms 29/4/2010

Movahed, M.-R., M. Jalili, et al. (2005). "Cardiovascular abnormalities and arrhythmias in patients with Ondine's curse (congenital central hypoventilation) syndrome." Pacing & Clinical Electrophysiology 28(11): 1226-30.

Patients with congenital central hypoventilation syndrome (CCHS) (Ondine's curse syndrome) have impaired autonomic control of ventilation with intact voluntary control of respiration. Autonomic dysfunction and cardiac abnormalities are common in CCHS. Bradyarrhythmias are life-threatening and often require pacemaker insertion. We presented a case of a patient with CCHS suffering from long sinus pauses requiring cardiac pacemaker insertion. Patients with CCHS are at risk for pulmonary hypertension and cor pulmonale secondary to chronic hypoxia. Diaphragmatic pacing has been beneficial in some patients with CCHS. In this article, we review concomitant cardiac abnormalities and the occurrence of bradyarrhythmias in patients with CCHS. [References: 71]

Mukhopadhyay, S. and P. W. Wilkinson (1990). "Cerebral arteriovenous malformation, Ondine's curse and Hirschsprung's disease." Developmental Medicine & Child Neurology 32(12): 1087-9.

A child presented with a congenital central hypoventilation syndrome and Hirschsprung's disease. Subsequent investigations demonstrated a cerebral arteriovenous malformation. The significance and interrelationship of these three conditions is discussed, together with a review of the literature.

Muller, F. and H. Rohrer (2002). "Molecular control of ciliary neuron development: BMPs and downstream transcriptional control in the parasympathetic lineage." DEVELOPMENT 129(24): 5707-5717.

The generation of noradrenergic sympathetic neurons is controlled by BMPs and the downstream transcription factors Mash1, Phox2b, Phox2a and dHand. We examined the role of these signals in developing cholinergic parasympathetic neurons. The expression of Mash1 (Cash1), Phox2b and Phox2a in the chick ciliary ganglion is followed by the sequential expression of panneuronal, noradrenergic and cholinergic marker genes. BMPs are expressed at the site where ciliary ganglia form and are essential and sufficient for ciliary neuron development. Unlike sympathetic neurons, ciliary neurons do not express dHand; noradrenergic gene expression is eventually lost but can be maintained by ectopic dHand expression. Together, these results demonstrate a common BMP dependence of sympathetic neurons and parasympathetic ciliary neurons and implicate dHand in the maintenance of noradrenergic gene expression in the autonomic nervous system.

Murthy, V. N. and E. E. Fetz (1996). "Synchronization of neurons during local field potential oscillations in sensorimotor cortex of awake monkeys." JOURNAL OF NEUROPHYSIOLOGY 76(6): 3968-3982.

1. The neural activity associated with 20- to 40-Hz oscillations in sensorimotor cortex of awake monkeys was investigated by recording action potentials of single and multiple units. At a given site, activity of many units became synchronized with local field potential (LFP) oscillations. Cycle-triggered histograms (CTHs) of trait spikes aligned on cycles of LFP oscillations indicated that about two thirds of the recorded units (n=268) were entrained with LFP oscillations. On average, units had the highest probability of spiking 2.7 ms before peak LFP negativity, corresponding to a -27.6degrees phase shift relative to the negative peak of the LFP. 2. The average relative modulation amplitude (RMA), defined as the ratio of amplitude of oscillatory component of CTH and the baseline multiplied by 100, was 45 +/- 27% (mean +/- SD). The RMAs of single units did not differ significantly from those of multiple units. 3. Phase shifts and RMAs did not vary systematically with the cortical depth of recorded units. 4. Autocorrelation histograms (ACHs) of entrained units exhibited clear 20- to 40-Hz periodicity if they were compiled with spikes that occurred during oscillatory episodes in LFPs. ACHs of spikes outside oscillatory episodes usually did not show periodicity. Global ACHs of all spikes typically showed weak or no evidence on periodic activity. 5. Cross-correlation histograms (CCHs) between pairs of units compiled with all spikes, whether they occurred during or outside LFP oscillations, seldom revealed significant features (19 of 134 pairs or 14%). However, CCHs compiled with spikes that occurred during oscillatory episodes (OS-CCHs) had significant features in 67 of 134 pairs recorded ipsilaterally; in these 67 cases, units at both sites showed modulation in CTHs. 6. The latencies of the OS-CCH peaks (taking the medial unit as reference) were normally distributed about a mean of -0.5 +/- 13 ms. Normalized peak height of CCHs (peak/baseline * 100) was, on average, 14.3 +/- 11.2%. Peak latency and normalized peak amplitude did not change significantly with horizontal separation of recorded precentral pairs up to 14 mm. 7. Units in the left and right hemispheres could become synchronized during oscillations. Significant features in OS-CCH were detected in 22 of 42 pairs of units recorded bilaterally. The average peak latency was 0.2 +/- 8.0 ms and the average normalized peak amplitude was 10 +/- 8%. These parameters did not differ significantly from those for ipsilateral OS-CCHs. 8. Oscillations tended to affect both the temporal structure and net rate of unit firing. For each unit, the firing rate was clamped to a narrow range of frequencies during oscillatory episodes. The coefficient of variation (SD/mean) of firing rates was significantly reduced during oscillatory episodes compared with prior rates (P < 0.001, paired t-test). However, the overall mean firing rate of each unit during all oscillatory episodes did not differ from its average rate immediately before the episodes. Thus oscillatory episodes tended to clamp mean firing rates to the cells' average rates outside episodes. 9. The strength of synchronization between units during oscillatory episodes was unrelated to their involvement in the task. For pairs of precentral units recorded ipsilaterally, the probability of occurrence of significant features in the OS-CCH was slightly larger when both units of the pair were task related (33 of 56 pairs or 59%) than when only one unit was task related (20 of 39 pairs or 51%) or neither unit was task related (7 of 16 or 44%). However, these differences were not statistically significant. The magnitude of the correlation peak and the latency to peak were also not significantly different for the three cases. 10. These results suggest that units across wide regions can become transiently synchronized specifically during LFP oscillations, even if their spikes are uncorrelated during nonoscillatory periods. Synchronization did not occur preferentially for coactivated, task- related neurons, suggesting that synchronous o
Muzumdar, H. and R. Arens (2008). "Central Alveolar Hypoventilation Syndromes." Sleep Medicine Clinics 3(4): 601-615.

Although respiratory abnormalities and autonomic dysfunction in patients with congenital central alveolar hypoventilation disorders persist throughout life, the prognosis for these children has improved considerably in recent years. This improvement may be attributed to wider recognition of such disorders, specialized centers treating such children, and improved technology to treat and monitor these children throughout life. copyright 2008 Elsevier Inc. All rights reserved.

Nabalamba, A. and W. J. Millar (2007). "Going to the doctor." HEALTH REPORTS 18(1): 23-35.

OBJECTIVES: This article, based on the Andersen model, describes patterns of consultation with general practitioners (GPs) and specialists among Canadians aged 18 or older. Associations with health status and other factors are examined. DATA SOURCE: Estimates are based on data from the 2005 Canadian Community Health Survey (CCHS), cycle 3.1. ANALYTICAL TECHNIQUES: Cross-tabulations were used to estimate the proportion of adult Canadians who had had a GP consultation, four or more GP consultations, or a specialist consultation in the previous year. Adjusted logistic regression models were used to examine factors associated with such consultations when the effects of health need were taken into account. MAIN RESULTS: In 2005, 77% of Canadians aged 18 to 64 and 88% of seniors reported that they had consulted a GP in the previous year; 25% and 44%, respectively, had done so four or more times; and 27% and 34% had consulted a specialist. Individual health need, as measured by the presence of chronic conditions and self-reported general and mental health, was a strong determinant of service use. However, when need was taken into account, physician consultations were independently associated with age, sex, household income, race, language, urban/rural residence and having a regular family doctor. Seniors aged 75 or older and rural residents had low odds of specialist consultations, but high odds of four or more GP consultations. Visible minorities and Aboriginal people had lower odds of reporting specialist consultations than did Whites.

Nabalamba, A. and S. B. Patten (2010). "Prevalence of mental disorders in a Canadian household population with dementia." CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES 37(2): 186-194.

Background: Medical and mental health comorbidity in Alzheimer's disease and other dementias presents difficult challenges for health service delivery. However, existing studies have been conducted in clinical samples and may not be informative for planning community services. The Canadian Community Health Survey (CCHS) provides an opportunity to characterize associations between dementias and mental and physical comorbidity in a household population aged 55 and over. Methods: Data were obtained from the 2005 CCHS-cycle 3.1. Weighted estimates for mood and anxiety disorders and other characteristics in Canadian population with dementia were calculated and were compared to those in people without the condition. Results: According to the CCHS, the prevalence of Alzheimer's disease and other dementia increases with age, more or less doubling every decade. The increase among women is monotonic, whereas among men in the household population the rate of dementia peaks at age 85-89 and falls thereafter. Mood and anxiety disorders were found to be substantially more frequent among people with Alzheimer's disease and other forms of dementia compared to those without the disease (mood disorders: 19.5% vs. 5.3% and anxiety disorders: 16.3% vs. 4.0%). Heart disease, stroke and obesity were associated with dementia as was a lower level of education. Furthermore, people with dementia were more likely than those without the disease to report activity restrictions. Conclusions: The high prevalence of mood and anxiety disorders in household population with Alzheimer's disease and other dementia demonstrates the burden of disease that is likely to worsen quality of life over time.

Nadzam, D. M., P. M. Atkins, et al. (2005). "Cleveland Clinic Health System: A comprehensive framework for a health system patient safety initiative." Quality Management in Health Care 14(2): 80-90.

The Cleveland Clinic Health System (CCHS) is committed to the enhancement of patient safety throughout the CCHS. This article describes the CCHS patient safety initiative, the development, objectives, strategies, goals, and activities.

Nadzam, D. M., D. M. Waggoner, et al. (2003). "Introducing the quality Institute of the Cleveland Clinic Health System." AMERICAN JOURNAL OF MEDICAL QUALITY 18(5): 204-13.

Several quality measurement needs surfaced when the Cleveland Clinic Health System (CCHS) was formed, including a need for standardized measurement of clinical processes and outcomes, patient satisfaction, critical care, and patient safety. The Quality Institute (QI) facilitates system teams to address these issues, manages selection of measurement tools, collects and analyzes performance data, coordinates presentations, and presents team findings. The QI manages the CCHS performance improvement plan and coordinates activities designed to accomplish priority goals, in collaboration with multiple CCHS teams and the regional and hospital staffs. The most important outcome of the QI's operations is improved care, as demonstrated through objective measurement. Other outcomes include external recognition and funding; implementation of standardized measurement systems, data management activities, and production of quarterly reports; increased internal recognition; completion of several education programs; acceptance of data by payers and plans; and participation in attainment of Joint Commission on Accreditation of Healthcare Organizations network accreditation.

Naeije, N., C. Melot, et al. (1982). "Ondine's curse. Report of a patient treated with almitrine, a new respiratory stimulant." European Journal of Respiratory Diseases 63(4): 342-6.

Nakahara, S., K. Yokomori, et al. (1995). "Hirschsprung's disease associated with Ondine's curse: a special subgroup?" JOURNAL OF PEDIATRIC SURGERY 30(10): 1481-4.

The authors report a case of the rare occurrence of congenital central hypoventilation syndrome (Ondine's curse) and long segmental colonic aganglionosis (Hirschsprung's disease). A review of 24 reported cases showed that the proportion of females having this concurrence is higher than for ordinary Hirschsprung's disease. It also appears that the aganglionic segment is much longer in these cases than in ordinary Hirschsprung's disease. [References: 23]
Nakajima, M., K. Katsura, et al. (2000). "A case of ondine curse associated with medullary tumor. [Japanese]." Clinical Neurology 40(8): 811-815.

A 49-Year-Old woman with 6 months history of body weight loss muscle weakness and dysarthria was found with respiratory arrest resuscitated in the morning of January 1999. An MRI brain scan revealed diffuse swelling T₂/FLAIR high signal intensity with mild Gadolinium enhancement in the lower pons medulla oblongata. Although the histological diagnosis could not be obtained glioma (astrocytoma) was suspected. In the morning of July 3rd she presented sweating cyanosis. Her arterial oxygen saturation was 18%. When we asked her to breathe more. she kept breathing oxygen saturation was normalized. However she could not breathe at all when she fell asleep without stimulation. She was kept under respiratory support for 2 months. Her symptoms improved with fluctuating course after 70 Gy of radiation therapy. Ondine curse is one type of sleep apnea syndrome defined as the selective disturbance of autonomous breathing. Surgical operation stroke are the reported causes of this syndrome. Brainstem tumor is relatively common cause for children'S Ondine curse. On the other hand it rarely causes adult'S Ondine curse as a main symptom.

Nakajima, T., M. Ota, et al. (2008). "Differentiation of autonomic neurons by BMP-independent mechanisms." CELL AND TISSUE RESEARCH 332(1): 25-35.

A number of signaling molecules and transcription factors play important roles in the development of the autonomic nervous system. Here, we show that mouse trunk neural crest cells can differentiate into autonomic neurons expressing mammalian achaete-scute homolog 1 (mash1), Phox2b, tyrosine hydroxylase, and/or dopamine-beta-hydroxylase in the absence of bone morphogenetic protein (BMP)-4. The expression of mash1 and Phox2b is induced even in the presence of noggin or chordin, which are inhibitors of BMP signaling. Whereas these autonomic neurons do not express c-ret, the receptor for glial-cell-line-derived neurotrophic factor (GDNF), GDNF promotes the differentiation of c-ret-positive autonomic neurons in the presence of noggin. Autonomic neurogenesis is completely prevented by fibroblast growth factor (FGF)-2 treatment or by activation of Notch signaling. Furthermore, the suppression of Phox2b expression by FGF-2 can be recovered by treatment with Notch-1 small interfering RNA. Our data suggest that BMP-independent mechanisms promote the differentiation of autonomic neurons, and that FGF-2 suppresses autonomic neurogenesis by means of the activation of Notch signaling. copyright 2007 Springer-Verlag.

Nakamura, A., T. Kuwaki, et al. (2000). "Normal ventilation and ventilatory responses to chemical stimuli in juvenile mutant mice deficient in endothelin-3." RESPIRATION PHYSIOLOGY 124(1): 1-9.

Congenital central hypoventilation syndrome (CCHS) and Hirschsprung's disease (HSCR) are often classified as neurocristopathies and are thought to share a common molecular pathogenesis related to the genes that control the development of neural crest cells. We examined whether endothelin-3 (ET-3), one of the developmental regulators of neural crest cells and of which null mutation results in aganglionic megacolon in mice, fulfills the requirements for such a common molecule. To investigate the possible involvement of ET-3 in central ventilatory control, we measured ventilation in mutant mice deficient in ET-3 by whole body plethysmography. Tidal volume and breathing frequency were measured during breathing of room air, hypoxic, hyperoxic, or hypercapnic gas mixtures in awake and anesthetized mice. There were no significant differences in resting ventilation as well as ventilatory responses to hypoxia and hypercapnia between ET-3-knockout mice and wild-type mice. Our results indicate that ET-3 can not be considered as a common pathogenic mechanism for CCHS and HSCR at least in mice. (C) 2000 Elsevier Science B.V.

Nam, T. K., J. I. Lee, et al. (2005). "Gamma knife radiosurgery for circumscribed choroidal hemangioma." ACTA NEUROCHIRURGICA 147(6): 651-4; discussion 654.

The authors report a case of circumscribed choroidal hemangioma (CCH) treated by stereotactic radiosurgery with gamma knife. A thirteen-year-old boy presented with a 4-year history of progressive visual loss in his right eye. Because of the large size and fluid collection under the macula, gamma knife radiosurgery was done. During 2 years of follow-up, there was resolution of retinal detachment, regression in tumor thickness, and no complications. Gamma knife radiosurgery may be a reasonable alternative treatment option for symptomatic CCHs which are difficult to manage using standard therapeutic options such as photocoagulation.

Nannapaneni, R., S. Behari, et al. (2005). "Retracing "Ondine's curse"." NEUROSURGERY 57(2): 354-63; discussion 354-63.

"Ondine's curse" is a term used to denote a rare neurological condition causing failure of automatic respiration. The patients are no longer capable of breathing spontaneously-they must consciously and voluntarily force themselves to do so. Ondine (also known as "Undine"), a mythological figure of European tradition, was a water nymph or sprite who could become human only when she fell in love with a mortal man. However, if the mortal was unfaithful to her, he was destined to forfeit his life. In the 16th century, Paracelsus coined the term "Undine" to describe the spirit that inhabited the element of water. Baron de la Motte-Fouque wrote the story of Undine in the late 18th century. It has since become a popular subject for theater productions. Jean Giraudoux, the French playwright, introduced the concept of the loss of automaticity of all functions as the "curse of Ondine." The legend was popularized in the form of the fairy tale "The Little Mermaid" by Hans Christian Andersen and as an animated motion picture by Walt Disney Productions. In this study, we look at the origins of this eponymous term, the personalities intertwined with its popularity, and its misrepresentations in the medical literature.

Nekrep, N., J. Wang, et al. (2008). "Signals from the neural crest regulate beta-cell mass in the pancreas." DEVELOPMENT 135(12): 2151-2160.

Pancreatic islet cells and neurons share common functions and similar ontogenies, but originate in different germ layers. To determine whether ectoderm-derived cells contribute instructive signals to the developing endoderm-derived pancreas, we defined the chronology of migration and differentiation of neural crest cells in the pancreas, and tested their role in the development of the islets. The homeodomain transcription factor Phox2b marks the neural precursors from the neural crest that colonize the gut to form the enteric nervous system. In the embryonic mouse pancreas, we found Phox2b expressed briefly together with Sox10 along the epithelial-mesenchymal border at E12.5 in cells derived from the neural crest. Downregulation of Phox2b shortly thereafter was dependent upon Nkx2.2 expressed in the adjacent pancreatic epithelium. In Phox2b-/- embryos, neurons and glia did not develop in the pancreas, and Nkx2.2 expression was markedly upregulated in the epithelium. In addition, the number and replication rate of insulin-expressing beta-cells increased in the Phox2b-/- mice. We conclude that, during pancreatic development, Phox2b and Nkx2.2 form a non-cell-autonomous feedback loop that links the neural crest with the pancreatic epithelium, regulates the size of the beta-cell population, and thereby impacts insulin-secretory capacity and energy homeostasis.

Nelson, C. H. and J. Park (2006). "The nature and correlates of unmet health care needs in Ontario, Canada." Social Science & Medicine 62(9): 2291-300.

Using data from the Canadian Community Health Survey (CCHS) Cycle 1.2, we examine the nature of unmet mental health care needs in Ontario, Canada and how this is associated with socio-demographic, social support, health status and mental health service use factors. Unmet mental health care needs result from experiencing barriers to three issues: acceptability, accessibility and availability. Unmet needs due to acceptability issues are the most frequent type; the largest proportion of people within this category report experiencing unmet needs because they "preferred to manage the problem themselves". Unmet needs are greater among the young and among females. Surprisingly, service users report higher rates of unmet needs than non-users. Some social support variables have associations with unmet needs. Based upon these results, unmet needs pose a major challenge to the health care system since they cannot be resolved solely by enhancing access to and availability of mental health services. Thus, to address unmet mental health care needs, efforts should be focused on the acceptability barriers that women and young people in particular face. Enhancing education and certain social support mechanisms are potential strategies.

Newbold, K. B. and J. K. Filice (2006). "Health status of older immigrants to Canada." Canadian Journal on Aging 25(3): 305-19.

Using the 2000/2001 Canadian Community Health Survey (CCHS), this paper examines the health status of the older (aged immigrant population relative to that of non-immigrants in order to identify areas where their health statuses diverge. First, we compare the health status of older immigrants (foreign-born) aged 55 and over in Canada to the Canadian-born in terms of age and gender using multiple measures of health status including self-assessed health. Second, we identify the factors associated with health status using the determinants of health framework. In both cases, the key questions are whether differences in health status exist and whether they are explained primarily by socio-economic, socio-demographic, or lifestyle factors that may point to problems with the Canadian health care system. Findings indicate that there is a relative comparability in the health status of older immigrants, even after controlling for age.

Ng, E., K. Dasgupta, et al. (2008). "An algorithm to differentiate diabetic respondents in the Canadian Community Health Survey." HEALTH REPORTS 19(1): 71-9.

OBJECTIVES: This article describes an algorithm to classify respondents to cycle 1.1 (2000/2001) of the Canadian Community Health Survey (CCHS) according to whether they have type 1, type 2 or gestational diabetes. DATA SOURCE: The data are from the chronic disease module and the drug module of cycle 1.1 of the CCHS. ANALYTICAL TECHNIQUES: A total of 6,361 respondents to cycle 1.1 of the CCHS reported that a health care professional had diagnosed them as having diabetes. The Ng-Dasgupta-Johnson algorithm classifies this group according to whether they have type 1, type 2 or gestational diabetes, based on their answers to CCHS questions about diabetes during pregnancy, use of oral medications to control diabetes, use of insulin, timing of initiation of insulin treatment, and age at diagnosis. MAIN RESULTS: Application of an earlier algorithm to CCHS cycle 1.1 results in a 10%-90% split for type 1 and type 2 diabetes. By contrast, the Ng-Dasgupta-Johnson algorithm yields a 5%-95% split. This is not unreasonable, given the rapid rise in obesity, a major risk factor for type 2 diabetes, in Canada.