- What is the SNS?
- Does Narcolepsy Symptom Severity Vary According to HLA-DQB1*0602 Allele Status?
- How often are you unable to fall asleep?
- Measure Scaled PDF Drawings with PDF Measure It - How to Calibrate it for any scale
- Key questions to ask to help identify cataplexy.
- Ullanlinna Narcolepsy Scale (UNS)
- How often do you feel bad or not well rested in the morning?
- “How do I know if I may have cataplexy?”
- Ullanlinna narcolepsy scale pdf plans
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What is the SNS?
Learn more. Several studies have reported abnormal cerebral activation in patients with narcolepsy with cataplexy. It remains unclear, however, whether these functional changes are related to structural alterations, particularly at the cortical level.
Statistical analyses accounted for an effect of age and gender. We observed decreased cortical volume in the left paracentral lobule and increased cortical volume in the left caudal part of the middle frontal gyrus in narcoleptic patients compared with controls.
Does Narcolepsy Symptom Severity Vary According to HLA-DQB1*0602 Allele Status?
Cortical thickness in prefrontal areas was inversely correlated with the severity of narcolepsy. Further, we observed several clusters of cortical thinning in patients with childhood or adolescent onset of narcolepsy compared with patients with adult onset of the disease.
Our results suggest that specific anatomical changes may differentiate subgroups of narcolepsy patients with different clinical profiles such as varying symptom severity or different age at onset. Narcolepsy with cataplexy is a sleep—wake disorder affecting one in individuals, and is characterized by excessive daytime sleepiness and cataplexy, i.
Hypocretin neurons are connected to multiple brainstem and midbrain arousal systems, and have widespread excitatory connections to the cortex.
However, only a few studies to date used structural MRI to identify whether morphological brain changes are associated with the disease. Aside from the fact that functional brain changes are often related to structural brain changes, many other arguments suggest altered cortical or subcortical structures in narcolepsy.
Secondly, lesions in other regions than the hypothalamus — e. Finally, the higher prevalence of depression and cognitive deficits which are occasionally associated with narcolepsy, such as attention deficit and learning difficulties Fulda and Schulz, , may be a consequence of excessive daytime sleepiness, but could also reflect abnormal cortical functioning.
How often are you unable to fall asleep?
The variability and inconsistencies in previous findings may rely on patient sampling, on methodological issues, or both. Twelve individuals with narcolepsy with cataplexy who have been reported previously participated in the current study seven females, five males. Cerebrospinal fluid CSF hypocretin concentrations were available in eight of the 12 participants and were abnormally low in all tested participants. The 12 patients had an average age of Patients had an average Epworth sleepiness scale score of Scores higher than 14 at the Epworth sleepiness scale or at the Ullanlinna narcolepsy scale are typical for narcolepsy.
Conversely, scores at the Swiss narcolepsy scale are suggestive of a narcolepsy when below 0. The patients also completed the Sleep Apnea—Sleep Disorders Questionnaire, and had an average sleep apnoea score of Sleep questionnaires were completed by 10 of the 12 control participants.
The control group had an average score of 5. Furthermore, the control group had an average sleep apnoea score of Written informed consent was received from all patients and controls in accordance with protocols approved by the local ethics committee.
Both intensity and continuity information from the entire 3D MR volume are used in segmentation and deformation procedures, thus producing accurate representation of cortical thickness or volumes.
At the end of the reconstruction process, the following volumes were available: total cerebral and cerebellar grey and white matter volume, and the volumes of subcortical structures including thalamus, putamen, pallidum and caudate nucleus, as well as amygdala and hippocampus.
This parcellation method based on major sulci has been shown to be both valid and reliable, with high intraclass correlation coefficients between the manual and automated procedures for both cortical volume estimates and region boundaries. In the present study, the frontal pole and the banks of the superior temporal sulcus regions, which exhibited relatively poor reliability in the validation article, were excluded from statistical analyses.
Cortical volume was therefore estimated for 32 regions of interest ROIs in each hemisphere for each subject.
Cortical thickness was measured in the native space of the images, as the shortest distance between the white grey—white boundary and the pial grey—CSF interface surfaces. We used analyses of covariance ancova s to compare cerebral, cerebellar and subcortical volumes between groups, adding age and gender as covariates.
In order to identify potential regional cortical alterations, we subsequently applied a multivariate analyses of variance mancova on the 32 gyral regions in each hemisphere, with diagnosis as the fixed factor and both age and gender as covariates.
Statistical analyses employed a general linear model GLM to estimate the effect of diagnosis, age and gender on thickness or gyrification at each cortical point. Cortical thickness or gyrification changes with age were fitted using a linear model.
Apart from searching an effect of disease duration, we also aimed at identifying whether cortical volume and thickness differed between patients with early and patients with late onset of narcolepsy. For that purpose, we divided our sample of participants into two groups according to the median age at onset of the narcolepsy symptoms.
This resulted in a group of patients with narcolepsy onset during childhood or adolescence mean age at onset: All the analyses used a GLM and corrected for an effect of age and gender on cortical thickness or regional cortical volumes. First, to avoid multiple comparisons between cortical structure and similar measurements of symptom severity, we searched for an eventual colinearity between the different measurements available i.
For that purpose, we conducted a principal component analysis PCA using the scores from the Epworth sleepiness, Ullanlinna narcolepsy and Swiss narcolepsy scales.
The correlation analyses were thus conducted between cortical thickness or volume and the composite score of symptom severity. Because the MSLT did not correlate with any of the scores obtained at the questionnaires, correlation analyses were also conducted between cortical thickness or volume and MSLT. Similarly, no group difference was observed in total cerebellar volume patients: None of the examined subcortical structures showed any significant volume difference between groups.
As illustrated in Fig.
Cortical changes in patients with narcolepsy compared to controls. Only one cluster of increased cortical thickness was observed in patients compared with controls.
No cluster of decreased cortical thickness was evidenced. No differences in local gyrification were found between patients and controls.
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We did not find any significant correlation between regional cortical volumes and disease duration. Similarly, no significant cluster of correlation between cortical thickness and disease duration was shown.
Thinner cortex was revealed in four clusters in the group of patients with young age at onset compared with the group of patients with adult onset of the disease.
Key questions to ask to help identify cataplexy.
These regions encompass the left precentral gyrus, the right inferior parietal cortex and the right middle and superior temporal gyri see Fig. No region of significantly thicker cortex in the group of patients with early compared with late age at onset was observed. As detailed in Fig. When testing for correlations between the cortical thickness and clinical variables within the group of patients, no significant correlation was observed between cortical volume or cortical thickness and the mean sleep latency on MSLT.
No correlation was shown between the composite score and any of the regional cortical volumes.
Ullanlinna Narcolepsy Scale (UNS)
However, two clusters of significant correlation between cortical thickness and the composite score were observed see Fig. These two clusters were located in the right prefrontal region and had the same direction of correlation: higher severity of narcolepsy was associated with decreased cortical thickness.
Correlations between cortical thickness and the clinical composite score lateral and frontal views. In both clusters, the significant correlation reflects that severe sleep disturbances are associated with thinner cortex. Moreover, our study may have clinical implications by identifying brain circuits more vulnerable in narcoleptic patients, as revealed by the correlations analyses between structural measurements and severity of the disease.
We would like to emphasize that our method identifies the boundaries of anatomical structures to measure their raw volumes. Small structures with unclear boundaries are thus not identified easily and reliably; this is why we do not provide volumetric measurements for the hypothalamus.
Further analyses within the group of patients with narcolepsy revealed: d four clusters of significantly decreased cortical thickness in patients with early compared with adult onset of the disease; and e significant inverse correlations between cortical thickness in two prefrontal regions of the right hemisphere and a composite score of the severity of narcolepsy.
How often do you feel bad or not well rested in the morning?
The volumetric group difference was restricted to the left hemisphere and was not particularly consistent with previously published studies. It is currently unknown if a similar phenomenon plays a role in narcolepsy. Additionally to a role for the paracentral region in sleep maintenance, altered function of the paracentral cortex may be associated more specifically with narcolepsy with cataplexy. Both studies observed increased cortical metabolism, which was most prominent around the most dorsal and the medial parts of the sensorimotor cortex.
The second difference in cortical volume that we observed was an enlarged left caudal middle frontal gyrus, a region corresponding to the dorsolateral prefrontal cortex DLPFC. It is widely recognized that the dorsolateral prefrontal cortex plays a critical role in executive functioning, with its left counterpart being involved mainly in verbal working memory.
“How do I know if I may have cataplexy?”
The left DLPFC may be particularly sensitive to sleep quality and quantity, as increased activation was required to achieve the same task difficulty after sleep deprivation Chee and Choo, Thus, it remains elusive whether the increased volume of the left DLPFC in our narcoleptic patients reflects a compensatory mechanism to maintain cognitive performances despite excessive daytime sleepiness.
A similar mechanism could explain the increased cortical thickness observed in Fig. Unfortunately, in the present study we did not have any neurocognitive measurements available. However, we speculate that the unexpected direction of structural changes in the frontal lobe of patients with narcolepsy support the hypothesis of an increased cognitive pressure to sustain adequate executive functions. Along with the various findings published to date, the absence of any striking direction of changes in the comparison between patients and controls support the existence of heterogeneous structural brain changes in narcolepsy.
Compared with patients who develop the narcolepsy from the age of 17, we observed that patients with earlier onset of the disease showed thinner cortex in the left precentral, right middle and superior temporal gyri and in the right inferior parietal region, along with increased CSF volume.
Several explanations may account for the observed differences. First, we can speculate that the neuroanatomical differences reveal that the childhood onset and the adult onset represent distinct subtypes of narcolepsy, resulting from potentially different mechanisms and associated with specific symptoms.
A second explanation for the cerebral differences related to the age at onset would be that neurodegenerative changes are associated with the ongoing disease process. According to this hypothesis, longer disease duration would be associated with increased cortical thinning.
Among the previous morphometric studies, two explored correlations between cortical volume and duration of disease, reporting divergent results. In the present study, we also did not observe any significant correlation between any of the neuroanatomical variables and disease duration.
Even if most of the clusters depicted in Fig. Independently of the mechanisms responsible for the cortical thinning in the group of patients with early onset of narcolepsy, the location of the most significant finding may have implication for the cognitive function in the disease. As a result, the thinner cortex observed in cluster 2 of Fig. Finally, we conducted correlation analyses to search for a relationship between cortical thickness and the clinical severity of narcolepsy using a score combining the results obtained at the Epworth sleepiness, Ullanlinna and Swiss narcolepsy scales.
Among the morphometric studies published to date, only few conducted correlations between cerebral structure and symptom severity.
Ullanlinna narcolepsy scale pdf plans
In this study, we observed two prefrontal regions in the right hemisphere detailed in Fig. The measurement of symptom severity combined both the excessive daytime sleepiness and the severity of cataplexy.
It is noteworthy that we did not identify any significant correlation between cortical thickness or volume and the propensity to fall asleep alone i. Indeed, the location of the most significant cluster in the right orbitofrontal cortex can be explained in the context of altered function of the reward circuitry in patients with narcolepsy with cataplexy. The abnormal activity of the VTA, along with the altered orbitofrontal cortex cluster 1 in Fig. Volume 21 , Issue 5.
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