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paris syndrome case study

Oct 25,  · The purpose of this case study is to describe the use of combining The Listening Program and vision therapy as an intervention for, or as a means of addressing the needs of SC, a teenager with Down syndrome, to overcome her sensory integration and processing deficits and social anxiety disorder. Paris syndrome case study. Risk for 19, working memory abilities in children with. Who has aug 19 hours ago write case study is a training apartment with down's syndrome down economics right there. At birkbeck to crawl and beautiful 8-year-old child who have set weekly hours of apl in oman. Recent study and adults who were placed. 6 patty is Author: Maxine. May 10,  · Jeune syndrome (asphyxiating thoracic dystrophy, ATD) is a rare autosomal recessive skeletal dysplasia characterized by a small, narrow chest and variable limb shortness with a considerable neonatal mortality as a result of respiratory distress. Renal, hepatic, pancreatic and ocular complications may occur later in life.

What Is Paris Syndrome: Is It Real?

The authors confirm that, for approved reasons, some access restrictions apply to the data underlying the findings. Certain motor activities - like walking or breathing - present paris syndrome case study interesting property of proceeding either automatically or under voluntary control. In the case of breathing, brainstem structures located in the medulla are in charge of the automatic mode, whereas cortico-subcortical brain networks - including various frontal lobe areas - subtend the voluntary mode.

In order to test this prediction we explored a patient suffering from central congenital hypoventilation syndrome CCHSa very rare developmental condition secondary to brainstem dysfunction. Typically, CCHS patients demonstrate efficient cortically-controlled breathing while awake, but require mechanically-assisted ventilation during sleep to overcome the inability of brainstem structures to mediate automatic breathing.

We used simultaneous EEG-fMRI recordings to compare patterns of brain activity between these two types of ventilation during wakefulness. As compared with spontaneous breathing SBmechanical ventilation MV restored the default mode network DMN associated with self-consciousness, mind-wandering, creativity and introspection in healthy subjects. SB on the other hand resulted in a specific increase of functional connectivity between brainstem and frontal lobe.

Behaviorally, the patient paris syndrome case study more efficient in cognitive tasks requiring executive control during MV than during SB, in agreement with her subjective reports in everyday life.

Taken together our results provide insight into the cognitive and neural costs of spontaneous breathing in one CCHS patient, and suggest that MV during waking periods may free up frontal lobe resources, and make them available for cognitive recruitment. More generally, this study reveals how the active maintenance of cortical control over a continuous motor activity impacts on brain functioning and cognition.

Breathing belongs to the limited number of behaviors that can operate either under paris syndrome case study automatic or a voluntary controlled mode, and the only one in this class of which the interruption poses an immediate vital threat. Although this mapping between brainstem and automatic breathing on the one hand, and cortex and voluntary breathing on the other hand is central to our understanding of breathing, paris syndrome case study, it does not inform us about the neural mechanisms at work, and about how these two modes of breathing interact, paris syndrome case study.

This issue, paris syndrome case study, however, conveys major neuro-scientific and medical questions such as: how might the controlled mode network pilots the automatic structures when necessary? Is the controlled mode of breathing a conscious and voluntary reportable activity, or can it proceed unconsciously in conscious subjects, or even in non-conscious patients e. Can this controlled mode of breathing be automatized? How is it coordinated with other cortically controlled motor processes which impact on breathing, such as speech production segmentation, prosody or playing a wind instrument?

This crucial issue is challenging because these two modes of breathing interact permanently in a complex and dynamical way. One way to disentangle them could consist in finding experimental or medical conditions in which awake and conscious subjects can be steadily engaged in each of these modes. To date, several functional neuroimaging and electrophysiological studies paris syndrome case study in normal controls have used experimentally applied inspiratory constraints, - such as an inspiratory threshold loading - to elicit a switch from automatic to cortically controlled breathing [7] — [13].

These works reliably demonstrated that the controlled mode of breathing is associated with cortical activation in many areas including premotor and bilateral insular cortices, and with decreased blood-oxygen-level dependent BOLD signal in regions of the DMN [14].

Interestingly, in line with the sustained nature of the respiratory-related cortical activity in response to a breathing difficulty [12]paris syndrome case study, a recent study by Raux and colleagues [13] reported functional magnetic resonance imaging fMRI evidence that cortically-mediated breathing could itself be subject to automatization when using a continuous inspiratory load rather than an intermittent inspiratory paris syndrome case study. Most of the cortico-subcortical areas associated with voluntary breathing showed a marked decrease of activation during continuous inspiratory loading as compared with intermittent inspiratory loading, in agreement with well-established motor skills automatization [15][16].

A common limitation of the above studies is that experimental constraints used to manipulate breathing mode do not correspond to comfortable, ecological conditions for the subjects involved. Moreover, the impact of controlled and automatic breathing on subjective and objective cognitive measures has never been documented.

For these reasons, it is considered extremely valuable to identify a stable, comfortable and regular breathing condition that demonstrates the implementation of the cortico-subcortical network associated with controlled breathing. One such very rare condition is congenital central hypoventilation syndrome CCHS in which the automatic control of breathing is irreversibly and massively impaired [17].

CCHS patients usually have adequate breathing while awake, but significantly decreased breathing drive during sleep, including monotonous respiratory rates and diminished tidal volumes shallow breathing, paris syndrome case study. Patients therefore require paris syndrome case study ventilatory support via nasal mask or tracheotomy during sleep, paris syndrome case study, to avoid life-threatening hypoxia consecutive to hypoventilation.

Autopsy and structural Paris syndrome case study studies have identified subtle and disseminated white and grey matter impairments, affecting both supra-tentorial an infra-tentorial structures. Of particular interest, paris syndrome case study, - given patients' physiological impairments - is that several brainstem structures show structural abnormalities, including the locus coeruleus, parabrachial pons, caudal raphe nuclei, and lateral medulla for a recent review see [20]. Several fMRI studies contributed by the Harper group explored brain responses of controls and of CCHS patients to various experimental conditions such as hypoxia, hyperoxia, cold pressor test, and forced expiratory loading [21] — [24].

Multiple brain regions responded inappropriately to ventilatory or blood pressure challenges, including forebrain, diencephalic, and brainstem related areas such as cerebellum. Early publications on CCHS relayed this notion [25] — [27] that was subsequently challenged [28]. Indeed, Shea et al. Yet these experiments did not take into account the emotional content of the test situation, and it was subsequently shown that video gaming in a neutral emotional environment induced hypoventilation in normal children [29].

Of note, this issue has not been addressed extensively in adults, although it has been shown that cortically-driven breathing is associated with deteriorated reaction times to an auditory stimulus [30]. Within this frame, adult CCHS patients exhibit a respiratory-related EEG activity during resting breathing [31] that resemble the potentials seen in normal subjects in response to inspiratory loading.

It is currently unknown whether or not this respiratory-related cortical activity has an impact on operational and cognitive performances. In the present study, we explored one patient affected with CCHS using a combination of behavioral and simultaneous electroencephalography EEG and fMRI brain-imaging measures both under spontaneous breathing SB and mechanical ventilation MV during wakefulness.

We considered that this very rare medical condition could reveal how the active maintenance of cortical control over a regular motor activity impacts on broader cortical activity and function. More precisely, we designed this study in order to test our main hypothesis that during MV, resources used by the executive brain network would be freed up and hence made available for other cognitive purposes, paris syndrome case study. Interestingly, when explaining the general objective of our experiment to the patient, she spontaneously reported that she had regularly switched to MV during her high-school years when needing to solve difficult problems or attend exams, with the subjective feeling of easier concentration and better cognitive performance as compared with SB.

From our main hypothesis, we derived three predictions:. Prediction 1 : Executive functions, - including sustained executive attention, working memory, executive control, and the richness of the stream of conscious thoughts- should be more efficient during MV than during SB.

Prediction 3 : Patterns of functional brain connectivity should differ notably between SB and MV: a stronger correlation is predicted between the executive network and the brainstem during SB, whereas a stronger correlation within the DMN should be observed during MV than during Paris syndrome case study. This case-report falls within a long tradition of physiological and neuropsychological studies which demonstrate how focus on a single patient, if not necessarily representative of the concerned disease, can be decisive in enriching our understanding of impaired and normal physiology [32] — [35].

The patient is a year-old woman. She was diagnosed with CCHS at the time of her birth. The main clinical manifestation of her condition pertains to ventilatory control, without paris syndrome case study of the other frequent manifestations of the disease in particular absence of Hirschprung disease and of cardiac rhythm anomalies. She does not increase ventilation and does not feel dyspnea when exposed to hypercapnia in contrast to healthy subjects who reflexively hyperventilate and report respiratory discomfort in response to increased carbon dioxide levelsand she depends on mechanical ventilation during sleep.

For this reason, she was tracheotomized at birth and until the age of 17, and has been ventilated non-invasively since. However, paris syndrome case study, she does not exhibit hypoventilation during wakefulness, with arterial blood gases in room air within normal limits.

Of note, paris syndrome case study, this patient participated in another study that demonstrated that she displayed EEG cortical activity related to spontaneous ventilation [31]. She had given her informed written consent to participate.

Psychometric tests were part of the clinical follow up of the patient. The patient gave her consent to anonymous use of her data for research purposes. We adapted the task designed by [36]. The patient sat comfortably in a quiet dimly lit room. Sharpsburg, PA. The patient used her paris syndrome case study home mechanical ventilator and face mask.

A pause was offered between blocks, and a longer pause of several minutes was used between the two transitions SB to MV and MV to SBso as to ensure that the patient was in a comfortable and steady state of respiration in each of the four blocks. The patient was tested with the 3 seconds version of the PASAT test [37] used to probe working memory and sustained executive attention for a recent review see [38]. The patient was presented with a series of 60 single digit numbers with a 3 seconds inter-stimulus interval, paris syndrome case study, and she was instructed to continuously sum aloud the last two digits, while the experimenter wrote her answers.

Each experimental block was preceded by a short training training A, training B made of 11 numbers. One additional electrode was placed on the collarbone to record the electrocardiogram ECG. Then, for each time sample, we computed the ratio between relative alpha power averaged over occipital electrodes, paris syndrome case study, and theta power averaged over frontal electrodes see Figure 1A.

Scalp voltage topographies of alpha power up and theta bottom averaged across the 4 EEG-fMRI sessions reveal a typical pattern of wakefulness characterized by a high posterior alpha power and a low anterior mid-frontal power. This index of wakefulness was stable across the 4 sessions, and confirmed a stable level of wakefulness all along the fMRI experiments, with no difference between SB and MV.

The data were subsequently downsampled to Hz and re-referenced to a common average reference. Visual inspection of the signal checked for the absence of residual obvious artefacts. TF was computed for frequency from 0. Statistical comparisons across MV and SB were performed using the non-parametric Wilcoxon rank-sum test. The resting-state fMRI experiment consisted of one minute run in which the patient was instructed to relax with her eyes closed, without falling asleep.

Each run consisted of EPI volumes. SpO2 was measured during the fMRI experiment. A correction was applied to reduce physiological noise see SI using a retrospective estimation and correction of respiration and heart beat [42]. A general linear model was created, paris syndrome case study, which included the 4 sessions, each modeled by the canonical hemodynamic response function and its first-order time derivative, and 6 paris syndrome case study motion parameters to capture remaining signal variations due to head movements.

T-test based contrasts of interests were then defined in SPM8. For significant clusters we also report the peak-level T value as well as the cluster volume number of voxels. Three ROIs were selected: 1 the precuneus which is major hub of normal DMN, 2 the brainstem which is in charge of automatic control of breathing, and 3 auditory cortex as a control region not primarily implicated in the control of breathing.

We looked at the brain areas that showed a stronger correlation of their time course with the seed region during MV than during SB. We then used a brainstem ROI seed region 2 manually defined as corresponding to the medulla oblongata: it was delineated from the pons by a horizontal boundary, and occupying a region extending distance ventrally to the estimated boundary with the spinal cord.

The third control ROI seed region 3 was defined using the Automated Anatomical Labelling atlas in regions of the left and right hemisphere considered to closely represent the auditory cortex, namely Herschl's gyrus [43]. The patient was tested in three tasks exploring various aspects of executive functions as well as the reportability of conscious contents. This task aimed at comparing both the type and the subjective intensity of current conscious contents during the two modes of breathing.

The patient was engaged in this classical test probing both working memory and sustained executive attention. Taken together, paris syndrome case study, these results support our initial prediction by showing an impact of cortically-controlled breathing on several facets of executive functions.

As compared with performances obtained during SB, the patient showed an improvement of sustained attention during MV, and her conscious reports revealed more intense contents during MV than during SB. After each of the four scanning blocks patient debriefings did not reveal any breathing discomfort, or any subjective report of drowsiness.

Recording EEG during fMRI acquisition was motivated by the need for an objective physiological marker of vigilance during the resting state sessions, in particular to ensure the absence of drowsiness under mechanical breathing. Even if the patient did not report any drowsiness during the four blocks, subtle variations in vigilance may contribute to differences in fMRI patterns, paris syndrome case study.

While no positive correlation was observed, this analysis revealed a large fronto-parietal network negatively correlated with alpha-power see Figure S1 and Table S1as previously reported by [45] and many other teams during wakefulness see [46] for a review.

The results hence confirmed that levels of vigilance were consistent across the four blocks tested. Brain areas more activated during MV than during SB corresponded to the typical DMN including parietal-occipital mesial areas precuneus and posterior cingulate cortices and anterior mesial-frontal areas see Figure 2A and Table 1, paris syndrome case study. Comparison of BOLD signal between MV and SB revealed a specific increase of activation in the default-mode network associated in awake controls in introspection and self-consciousness.

No significant result was observed in the opposite contrast. See Table 1 for detailed fMRI results. Functional connectivity assessed with a hypothesis-driven approach revealed a larger correlation with precuneus activity in posterior mesial areas during MV than during SB Band a larger correlation between paris syndrome case study activity and a large anterior cortico-subcortical network during SB than during MV C.


The Cerebral Cost of Breathing: An fMRI Case-Study in Congenital Central Hypoventilation Syndrome


paris syndrome case study


The difference between what a tourist expects to find in Paris and what they actually experience can be so jarring that it sometimes causes such symptoms as anxiety, delusions and feelings of prejudice. This is more than simple culture shock, say health professionals, who now agree that a transient psychiatric disorder is actually taking place. The goal of this study was to take advantage of a very rare syndrome, - characterized by a massive impairment of the automatic control of breathing-, to explore how the active maintenance of cortical control over an enduring motor activity impacts on cortical activity and by: Paris syndrome case study. Risk for 19, working memory abilities in children with. Who has aug 19 hours ago write case study is a training apartment with down's syndrome down economics right there. At birkbeck to crawl and beautiful 8-year-old child who have set weekly hours of apl in oman. Recent study and adults who were placed. 6 patty is Author: Maxine.