Executive function performance in children and adolescent patients with narcolepsy type 1.

OBJECTIVE: The executive function profile in patients with narcolepsy type 1 (NT1) has been mentioned; however, limited research exists on children and adolescent patients with NT1.This study aims to assess executive function in children and adolescent patients with NT1 in China, examine potential influencing factors and evaluate the short-term treatment effect on executive function. METHODS: 53 NT1 patients (36 males, age 12.2 ± 3.4 years) and 37 healthy controls (23 males, age 12.2 ± 2.5 years) underwent self-reported measures assessing subjective sleepiness, depression, anxiety and sleep quality. A comprehensive neuropsychological test was administered to assess executive function domains, including processing speed, inhibitory control, cognitive flexibility and working memory. These assessments were repeated in NT1 patients after three-day regular drug treatment. RESULTS: NT1 patients exhibited higher levels of excessive daytime sleepiness, depression, anxiety, and poor sleep quality compared to healthy controls. Patients showed impaired processing speed, inhibitory control and cognitive flexibility (p < 0.05), whereas working memory was unaffected (p > 0.05). Regression analysis revealed that parameters from sleep monitoring, such as sleep efficiency and sleep latency, were correlated with executive function performance after controlling for age, gender, and education years. The short-term treatment led to improvements in inhibitory control, cognitive flexibility, and working memory. CONCLUSION: The findings showed that executive function was impaired among children and adolescent patients with NT1, which was associated with objective sleep parameters. Furthermore, this study emphasizes the necessity of neuropsychological assessments and early interventions among children and adolescent NT1 patients.

Changed epidemiology of narcolepsy before, during, and after the 2009 H1N1 pandemic: a nationwide narcolepsy surveillance network study in mainland China, 1990-2017.

STUDY OBJECTIVES: Increased incidence of narcolepsy was reported in children during the 2009 H1N1 pandemic following Pandemrix, a H1N1 flu vaccine. A link with A(H1N1) pdm09 infections remains controversial. Using nationwide surveillance data from China (1990 to 2017), the epidemiology of narcolepsy was analyzed. METHODS: Individual records of narcolepsy patients were collected from 15 of 42 hospitals across China known to diagnose cases. Incidence was estimated assuming the representativeness of these hospitals. Age-specific incidence, epidemiological and clinical characteristics of patients were evaluated before, during, and after the 2009 H1N1 pandemic. Sensitivity analyses were conducted by including NT1 cases only and excluding the effect of the 2009 H1N1 vaccination. RESULTS: Average annual incidence was 0.79 per 100 000 person-years (PY) from 1990 to 2017 and 1.08 per 100 000 PY from 2003 to 2017. Incidence increased 4.17 (95% CI 4.12, 4.22) and 1.42 (95% CI 1.41, 1.44) fold during and after the 2009 H1N1 pandemic when compared to baseline. These results were robust in sensitivity analyses. Patients with the onset of narcolepsy during the pandemic period were younger (notably in 5-9-year-old strata), and the age shift toward younger children reversed to baseline following the pandemic. CONCLUSIONS: Increased incidence of narcolepsy was observed during the 2009 H1N1 pandemic period. This is likely to be associated with the circulation of the wild type A(H1N1)pdm09 virus. This observation should be considered for future influenza pandemic preparedness plans.

Not Only Excessive Daytime Sleepiness but Also Depression Symptoms, Chronological Age and Onset-Age Were Associated with Impulsivity in Narcolepsy Type 1 Patients.

Objective: To detect the factors associated with total and subscales impulsivity scores in narcolepsy and further explore the non-linear relationship between these factors and impulsivity score. Methods: One hundred and fifty-eight narcolepsy type 1 (NT1) patients were involved in this cross-sectional study. Patients completed questionnaires evaluating impulsivity symptoms (Barratt impulse scale-11, BIS-11), depression symptoms (Center for Epidemiologic Studies Depression Scale for Children, CES-DC or Self-Rating Depression Scale, SDS) and sleepiness (Epworth Sleepiness Scale, ESS). Parameters from polysomnography and multiple sleep latency test were also collected. Linear regression analysis was performed to detect the factors associated with total and subscales impulsivity scores. Then, generalized additive models and smooth curve fitting were performed to explore the non-linear relationship between chronological age and impulsivity scores. Results: Factors associated with higher total impulsivity score were higher ESS, older onset and adolescents. Factors associated with higher attentional impulsivity score were higher ESS, older onset and adolescents. Factors associated with higher motor impulsivity score were higher ESS, younger onset and depression symptoms. Factors associated with higher non-planning impulsivity score were adolescents, older onset and depression symptoms. A non-linear relationship between age and impulsivity scores (total impulsivity score, attentional impulsivity score and non-planning impulsivity score) was detected in NT1 patients. Conclusion: Not only excessive daytime sleepiness but also onset-age, depression symptoms and chronological age were associated with impulsivity in NT1 patients. The non-linear relationship between age and impulsivity scores suggested brain and mental development alterations in NT1 patients.

The month of birth has a seasonal effect in Chinese patients with narcolepsy and cataplexy.

STUDY OBJECTIVES: We assessed the yearly seasonal, environmental effects on birth pattern in Chinese patients later diagnosed with narcolepsy and cataplexy and explored if this effect persisted in patients with symptoms onset date before, following, and after the 2009 H1N1 pandemic. METHODS: A total of 1,942 patients with birth data information and diagnosed narcolepsy with cataplexy were included in this study. The birth month and seasonal effect of 1,064 patients born from 1970 to 2000 were compared to controls (n = 2,028,714) from the general population. Furthermore, birth season effect in 1,373 patients with definite disease onset month were compared among patients with onset date before (n = 595), following (from January 2010 to December 2010) (n = 325), and after (n = 453) the H1N1 pandemic. RESULTS: Patients with narcolepsy and cataplexy had a significantly different seasonality from the general population (P = .027). The monthly distribution of birth month yielded a peak in November (odds ratio = 1.23 [95% confidence interval, 1.01-1.49], P = .042) and a trough in April (odds ratio = 0.68 [95% confidence interval, 0.52-0.88], P = .004). No significant difference was observed in the birth month across patients with symptom onset dates before, following, and after the 2009 H1N1 pandemic (P = .603). CONCLUSIONS: This finding across many years of seasonal effect in Chinese narcolepsy cataplexy supports a role for early-life environmental influences on disease development. CITATION: Guo J, Xu L, Wang J, et al. The month of birth has a seasonal effect in Chinese patients with narcolepsy and cataplexy. J Clin Sleep Med. 2022;18(2):461-467.

Factors Associated with Depression and Sub-Dimension Symptoms in Adolescent Narcolepsy.

OBJECTIVE: We evaluate the association between depression symptoms, clinical features (disease onset-age, disease duration, sleep-related hallucination), sleepiness, and polysomnography parameters in adolescent narcolepsy type 1 patients. METHODS: Eighty-three adolescent narcolepsy type 1 patients were involved in this cross-sectional study. Patients completed questionnaires evaluating depression symptoms (Center for Epidemiologic Studies Depression Scale) and sleepiness (Epworth Sleepiness Scale). Parameters from polysomnography and multiple sleep latency test were also collected. RESULTS: Patients with depression symptoms (62.7%) have later disease onset-age. Depression symptoms were associated with sleep-related hallucination (OR = 2.75). Six independent variables were associated with sub-dimensional depression symptoms, including sleep latency, sleep efficiency, sleep-related hallucination, Epworth sleepiness scale, disease duration, and disease onset-age. CONCLUSION: Sleep-related hallucination is associated with total depression symptoms in adolescent narcolepsy. Subjective sleepiness is associated with depressed affect, somatic symptoms, and interpersonal problems. Lower sleep efficiency is associated with a lack of positive affect.

Anti-streptococcal antibodies in Chinese patients with type -1 narcolepsy.

BACKGROUND: Narcolepsy type 1 (NT1) is considered to be an autoimmune disease, and streptococcal infection may be an environmental trigger. However, previous studies from Asian narcolepsy patients did not reveal elevated anti-streptolysin O [ASO]. The aim is to investigate whether large sample Chinese patients with NT1 have an increase in antistreptococcal antibody titers. METHODS: A total of 214 narcolepsy patients and 360 healthy controls were recruited. All patients were DQB1∗0602 positive with clear-cut cataplexy or had low CSF hypocretin-1. Participants were tested for ASO and anti DNAse B [ADB]. These patients were divided into five groups according to disease duration, including 29 patients less than 3 months; 25 from 3 months to 1 year; 40 from 1 to 3 years; 61 from 3 to 10 years and 59 patients over 10 years. Comparison was also made between children and adults with age matched controls, respectively. RESULTS: There were no significant differences between patients and healthy controls in regard to both ASO ≥200 IU (19.2% vs. 16.9%, p = 0.50) and ADB≥480IU (9.8% vs. 10.3%, p = 0.86). For children narcolepsy patients, ASO positive rates (19.8% vs. 18%, p = 0.68) and ADB positive rates (10.4% vs. 12%, p = 0.72) had no differences compared to age matched controls. No difference was observed in adult narcolepsy patients either, with ASO positive rates (18.5% vs. 13.8%, p = 0.39) and ADB positive rates (9.3% vs. 5.3%, p = 0.42) compared to age matched controls, respectively. ASO and ADB positive rates had no significant differences among different disease duration groups (p = 0.55 and 0.9, respectively). CONCLUSION: Streptococcus infection reflected by increase of ASO and ADB levels was not found in Chinese patients with type 1 narcolepsy, additional triggers for narcolepsy need to be addressed in this population.

Independent Component Analysis and Graph Theoretical Analysis in Patients with Narcolepsy.

The present study was aimed to evaluate resting-state functional connectivity and topological properties of brain networks in narcolepsy patients compared with healthy controls. Resting-state fMRI was performed in 26 adult narcolepsy patients and 30 matched healthy controls. MRI data were first analyzed by group independent component analysis, then a graph theoretical method was applied to evaluate the topological properties in the whole brain. Small-world network parameters and nodal topological properties were measured. Altered topological properties in brain areas between groups were selected as region-of-interest seeds, then the functional connectivity among these seeds was compared between groups. Partial correlation analysis was performed to evaluate the relationship between the severity of sleepiness and functional connectivity or topological properties in the narcolepsy patients. Twenty-one independent components out of 48 were obtained. Compared with healthy controls, the narcolepsy patients exhibited significantly decreased functional connectivity within the executive and salience networks, along with increased functional connectivity in the bilateral frontal lobes within the executive network. There were no differences in small-world network properties between patients and controls. The altered brain areas in nodal topological properties between groups were mainly in the inferior frontal cortex, basal ganglia, anterior cingulate, sensory cortex, supplementary motor cortex, and visual cortex. In the partial correlation analysis, nodal topological properties in the putamen, anterior cingulate, and sensory cortex as well as functional connectivity between these regions were correlated with the severity of sleepiness (sleep latency, REM sleep latency, and Epworth sleepiness score) among narcolepsy patients. Altered connectivity within the executive and salience networks was found in narcolepsy patients. Functional connection changes between the left frontal cortex and left caudate nucleus may be one of the parameters describing the severity of narcolepsy. Changes in the nodal topological properties in the left putamen and left posterior cingulate, changes in functional connectivity between the left supplementary motor area and right occipital as well as in functional connectivity between the left anterior cingulate gyrus and bilateral postcentral gyrus can be considered as a specific indicator for evaluating the severity of narcolepsy.

A case report of REM sleep behavior disorder, Behcet's disease, Sjogren's syndrome and cognitive dysfunction.

BACKGROUND: Behcet's disease and Sjogren's syndrome is an autoimmune disorder from which many systems of the body can suffer. Here we reported a patient with a history of Behcet's disease and Sjogren's syndrome in which REM sleep behavior disorder (RBD) was then detected by polysomnographic (PSG) monitoring. CASE PRESENTATION: A 68-year-old male patient with a history of Behcet's disease and Sjogren's syndrome was diagnosed with RBD by clinical examination and video-PSG, and he also underwent a multiple sleep latency test and cerebral magnetic resonance imaging. The patient had a history of Behcet's disease for 20 years and Sjogren's syndrome for 2 years. The cerebral magnetic resonance imaging also suggested cerebral demyelination and mild cortical atrophy, with cognitive dysfunction by a score of 28 on the mini-mental state examination (MMSE) and a score of 22 on the Montreal cognitive assessment (MoCA). CONCLUSION: RBD is common in the elderly population and is significantly related to α-synucleinopathy. Combining the decline in neuro-cognition and mild cortical atrophy, presentation of RBD in this patient could indicate an underlying α-synucleinopathy neurodegenerative disorder in the future. Considering the role of inflammation in the pathogenesis of α-synucleinopathy and a common shared HLA allelic genes in RBD and Sjogren's syndrome, it is suggested that a physiological process which is related to neuroinflammation may be involved in the pathogenesis of RBD.

Cortical representation of facial and tongue movements: a task functional magnetic resonance imaging study.

BACKGROUND: Functional magnetic resonance imaging (fMRI) mapping can present the activated cortical area during movement, while little is known about precise location in facial and tongue movements. OBJECTIVE: To investigate the representation of facial and tongue movements by task fMRI. METHODS: Twenty right-handed healthy subjects were underwent block design task fMRI examination. Task movements included lip pursing, cheek bulging, grinning and vertical tongue excursion. Statistical parametric mapping (SPM8) was applied to analysis the data. RESULTS: One-sample t-test was used to calculate the common activation area between facial and tongue movements. Also, paired t-test was used to test for areas of over- or underactivation in tongue movement compared with each group of facial movements. CONCLUSIONS: The common areas within facial and tongue movements suggested the similar motor circuits of activation in both movements. Prior activation in tongue movement was situated laterally and inferiorly in sensorimotor area relative to facial movements. Prior activation of tongue movement was investigated in left superior parietal lobe relative to lip pursing. Also, prior activation in bilateral cuneus lobe in grinning compared with tongue movement was detected.

Hashimoto's encephalopathy: a report of three cases and relevant literature reviews.

Hashimoto's encephalopathy (HE) is a very rate condition characterized by various clinical features consisting of psychiatric manifestations, seizures and focal neurologic deficits. In this study, three Hashimoto's encephalopathy cases were reported, including two female patients and one male patient. The two female patients (22-year-old and 49-year-old) were presented with brainstem involvement and the symptoms progressed gradually. The 70-year-old male patient was diagnosed with space-occupying lesion that seemed to be malignant, but the symptom was substantially ameliorated right after glucocorticoid therapy. Pathological studies indicate abnormal blood vessels are important in the progression of the disease. Compared with current reports, the male case was the first patient diagnosed of Hashimoto's encephalopathy but presented with malignant features and local occupying effect. Intracranial lesions were found in all of the three patients, but these lesions responded well to glucocorticoid therapy. And the lesions were remarkably reduced after treatment. Meanwhile, the prognosis of diffuse Hashimoto's encephalopathy is better than the vascular type.

Time-course of Changes in Activation Among Facial Nerve Injury: A Functional Imaging Study.

Patients suffering different intervals of facial nerve injury were investigated by functional magnetic resonance imaging to study changes in activation within cortex.Forty-five patients were divided into 3 groups based on intervals of facial nerve injury. Another 16 age and sex-matched healthy participants were included as a control group. Patients and healthy participants underwent task functional magnetic resonance imaging (eye blinking and lip pursing) examination.Functional reorganization after facial nerve injury is dynamic and time-dependent. Correlation between activation in sensorimotor area and intervals of facial nerve injury was significant, with a Pearson correlation coefficient of -0.951 (P < 0.001) in the left sensorimotor area and a Pearson correlation coefficient of 0.333 (P = 0.025) in the right sensorimotor area.Increased activation in integration areas, such as supramarginal gyrus and precunes lobe, could be detected in the early-middle stage of facial dysfunction compared with normal individuals. Decreased activation in sensorimotor area contralateral to facial nerve injury could be found in late stage of facial dysfunction compared with normal individuals. Dysfunction in the facial nerve has devastating effects on the activity of sensorimotor areas, whereas enhanced intensity in the sensorimotor area ipsilateral to the facial nerve injury in middle stage of facial dysfunction suggests the possible involvement of interhemispheric reorganization. Behavioral or brain stimulation technique treatment in this stage could be applied to alter reorganization within sensorimotor area in the rehabilitation of facial function, monitoring of therapeutic efficacy, and improvement in therapeutic intervention along the course of recovery.