Developmental change of brain volume in Rett syndrome in Taiwan / Tz-Yun JAN in Journal of Neurodevelopmental Disorders, 16 (2024)  

Public ISBD [article]  inJournal of Neurodevelopmental Disorders > 16 (2024) Titre : Developmental change of brain volume in Rett syndrome in Taiwan Type de document : texte imprimé Auteurs : Tz-Yun JAN, Auteur ; Lee-Chin WONG, Auteur ; Chia-Jui HSU, Auteur ; Chien-Feng Judith HUANG, Auteur ; Steven Shinn-Forng PENG, Auteur ; Wen-Yih Isaac TSENG, Auteur ; Wang-Tso LEE, Auteur Langues : Anglais (eng) Mots-clés : Humans  Rett Syndrome/diagnostic imaging/physiopathology/pathology  Female  Magnetic Resonance Imaging  Brain/diagnostic imaging/pathology/growth & development  Adult  Child  Young Adult  Child, Preschool  Adolescent  Taiwan  Gray Matter/diagnostic imaging/pathology  Male  Organ Size  White Matter/diagnostic imaging/pathology  Brain volume  Developmental trajectories  Gray matter volume  Magnetic resonance image  Rett syndrome  Total intracranial volume  article. Index. décimale : PER Périodiques Résumé : OBJECTIVE: Rett syndrome (RTT) is characterized by neurological regression. This pioneering study investigated the effect of age on brain volume reduction by analyzing magnetic resonance imaging findings in participants with RTT, ranging from toddlers to adults. METHODS: Functional evaluation and neuroimaging were performed. All scans were acquired using a Siemens Tim Trio 3 T scanner with a 32-channel head coil. RESULTS: The total intracranial volume and cerebral white matter volume significantly increased with age in the control group compared with that in the RTT group (p < 0.05). Cortical gray matter volume reduction in the RTT group continued to increase in bilateral parietal lobes and left occipital lobes (p < 0.05). The differences in cortical gray matter volume between typically developing brain and RTT-affected brain may tend to continuously increase until adulthood in both temporal lobes although not significant after correction for multiple comparison. CONCLUSIONS: A significant reduction in brain volume was observed in the RTT group. Cortical gray matter volume in the RTT group continued to reduce in bilateral parietal lobes and left occipital lobes. These results provide a baseline for future studies on the effect of RTT treatment and related neuroscience research. En ligne : https://dx.doi.org/10.1186/s11689-024-09549-6 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=575 [article] Developmental change of brain volume in Rett syndrome in Taiwan [texte imprimé] / Tz-Yun JAN, Auteur ; Lee-Chin WONG, Auteur ; Chia-Jui HSU, Auteur ; Chien-Feng Judith HUANG, Auteur ; Steven Shinn-Forng PENG, Auteur ; Wen-Yih Isaac TSENG, Auteur ; Wang-Tso LEE, Auteur. Langues : Anglais (eng) in Journal of Neurodevelopmental Disorders > 16 (2024) Mots-clés : Humans  Rett Syndrome/diagnostic imaging/physiopathology/pathology  Female  Magnetic Resonance Imaging  Brain/diagnostic imaging/pathology/growth & development  Adult  Child  Young Adult  Child, Preschool  Adolescent  Taiwan  Gray Matter/diagnostic imaging/pathology  Male  Organ Size  White Matter/diagnostic imaging/pathology  Brain volume  Developmental trajectories  Gray matter volume  Magnetic resonance image  Rett syndrome  Total intracranial volume  article. Index. décimale : PER Périodiques Résumé : OBJECTIVE: Rett syndrome (RTT) is characterized by neurological regression. This pioneering study investigated the effect of age on brain volume reduction by analyzing magnetic resonance imaging findings in participants with RTT, ranging from toddlers to adults. METHODS: Functional evaluation and neuroimaging were performed. All scans were acquired using a Siemens Tim Trio 3 T scanner with a 32-channel head coil. RESULTS: The total intracranial volume and cerebral white matter volume significantly increased with age in the control group compared with that in the RTT group (p < 0.05). Cortical gray matter volume reduction in the RTT group continued to increase in bilateral parietal lobes and left occipital lobes (p < 0.05). The differences in cortical gray matter volume between typically developing brain and RTT-affected brain may tend to continuously increase until adulthood in both temporal lobes although not significant after correction for multiple comparison. CONCLUSIONS: A significant reduction in brain volume was observed in the RTT group. Cortical gray matter volume in the RTT group continued to reduce in bilateral parietal lobes and left occipital lobes. These results provide a baseline for future studies on the effect of RTT treatment and related neuroscience research. En ligne : https://dx.doi.org/10.1186/s11689-024-09549-6 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=575

Dietary intake and growth deficits in Rett syndrome-A cross-section study / Lee-Chin WONG in Autism Research, 14-7 (July 2021)  

Public ISBD [article]  inAutism Research > 14-7 (July 2021) . - p.1512-1521 Titre : Dietary intake and growth deficits in Rett syndrome-A cross-section study Type de document : texte imprimé Auteurs : Lee-Chin WONG, Auteur ; Yen-Tsz CHEN, Auteur ; Shu-Mei TSAI, Auteur ; Yen-Ju LIN, Auteur ; Chia-Jui HSU, Auteur ; Hsin-Pei WANG, Auteur ; Su-Ching HU, Auteur ; Hsiu-Yu SHEN, Auteur ; Wen-Che TSAI, Auteur ; Wang-Tso LEE, Auteur Article en page(s) : p.1512-1521 Langues : Anglais (eng) Mots-clés : Autism Spectrum Disorder  Body Height  Eating  Humans  Methyl-CpG-Binding Protein 2/genetics  Mutation  Rett Syndrome/complications/genetics  Rett syndrome  clinical severity  dietary intakes  dystonia  growth deficit  nutrition Index. décimale : PER Périodiques Résumé : Growth deficit is a common comorbidity and one of the supportive criteria in Rett syndrome (RTT). This study aimed to investigate the impact of dystonia, dietary intakes, and clinical severities on growth patterns in a Taiwanese cohort of RTT. We recruited 44 RTT patients with MECP2 mutation for analysis. For individuals ≤18 years of age, in comparison to the RTT-specific growth chart which comprised American RTT cohort, the body height was right-shifted to a higher percentile, whereas the body weight was left-shifted to a lower percentile. Furthermore, the body mass index was significantly decreased when compared to RTT-specific growth chart (p = 0.01). Higher degree of overall disease severity (odd ratio = 1.159; 95% CI = 1.063-1.264; p = 0.001) and hand use impairment (odd ratio = 2.017; 95% CI = 1.037, 3.921; p = 0.039) were associated with more severe growth patterns. All individuals had dystonia at certain variable degrees. The dystonia worsened with age (p < 0.001) but did not have significant impact on growth deficit. Most of our cohort had adequate protein (97.37%) and energy (58.97%) intakes. The fiber intakes were generally low, with about 38 (97.4%) individuals did not meet the daily reference intakes of fiber. The protein intake was significantly lower in individuals with severe growth deficit (p = 0.04). Our study shows that ethnicity should be considered when comparing RTT individuals' growth pattern to the RTT-specific growth chart. Further, disease severity, genotypes, and nutrition exert important impacts on RTT-growth pattern. LAY SUMMARY: Growth impairment is an important issue in Rett syndrome and the underlying patho-mechanism is multifactorial. Higher degree of overall disease severity and hand use impairment were associated with more severe growth pattern deficits. Although all individuals had dystonia at certain variable degrees and the dystonia worsened with age, but it did not have significant impact on growth deficit. Nutritional intakes may partially affect growth. Furthermore, ethnicity should be considered when comparing RTT individuals' growth pattern to the RTT-specific growth chart. En ligne : http://dx.doi.org/10.1002/aur.2508 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=449 [article] Dietary intake and growth deficits in Rett syndrome-A cross-section study [texte imprimé] / Lee-Chin WONG, Auteur ; Yen-Tsz CHEN, Auteur ; Shu-Mei TSAI, Auteur ; Yen-Ju LIN, Auteur ; Chia-Jui HSU, Auteur ; Hsin-Pei WANG, Auteur ; Su-Ching HU, Auteur ; Hsiu-Yu SHEN, Auteur ; Wen-Che TSAI, Auteur ; Wang-Tso LEE, Auteur . - p.1512-1521. Langues : Anglais (eng) in Autism Research > 14-7 (July 2021) . - p.1512-1521 Mots-clés : Autism Spectrum Disorder  Body Height  Eating  Humans  Methyl-CpG-Binding Protein 2/genetics  Mutation  Rett Syndrome/complications/genetics  Rett syndrome  clinical severity  dietary intakes  dystonia  growth deficit  nutrition Index. décimale : PER Périodiques Résumé : Growth deficit is a common comorbidity and one of the supportive criteria in Rett syndrome (RTT). This study aimed to investigate the impact of dystonia, dietary intakes, and clinical severities on growth patterns in a Taiwanese cohort of RTT. We recruited 44 RTT patients with MECP2 mutation for analysis. For individuals ≤18 years of age, in comparison to the RTT-specific growth chart which comprised American RTT cohort, the body height was right-shifted to a higher percentile, whereas the body weight was left-shifted to a lower percentile. Furthermore, the body mass index was significantly decreased when compared to RTT-specific growth chart (p = 0.01). Higher degree of overall disease severity (odd ratio = 1.159; 95% CI = 1.063-1.264; p = 0.001) and hand use impairment (odd ratio = 2.017; 95% CI = 1.037, 3.921; p = 0.039) were associated with more severe growth patterns. All individuals had dystonia at certain variable degrees. The dystonia worsened with age (p < 0.001) but did not have significant impact on growth deficit. Most of our cohort had adequate protein (97.37%) and energy (58.97%) intakes. The fiber intakes were generally low, with about 38 (97.4%) individuals did not meet the daily reference intakes of fiber. The protein intake was significantly lower in individuals with severe growth deficit (p = 0.04). Our study shows that ethnicity should be considered when comparing RTT individuals' growth pattern to the RTT-specific growth chart. Further, disease severity, genotypes, and nutrition exert important impacts on RTT-growth pattern. LAY SUMMARY: Growth impairment is an important issue in Rett syndrome and the underlying patho-mechanism is multifactorial. Higher degree of overall disease severity and hand use impairment were associated with more severe growth pattern deficits. Although all individuals had dystonia at certain variable degrees and the dystonia worsened with age, but it did not have significant impact on growth deficit. Nutritional intakes may partially affect growth. Furthermore, ethnicity should be considered when comparing RTT individuals' growth pattern to the RTT-specific growth chart. En ligne : http://dx.doi.org/10.1002/aur.2508 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=449

Investigating the impact of probiotic on neurological outcomes in Rett syndrome: A randomized, double-blind, and placebo-controlled pilot study / Chia-Jui HSU ; Yen-Tzu WU ; Hsu-Feng CHU ; Jui-Hsiang LIN ; Hsin-Pei WANG ; Su-Ching HU ; Ying-Chieh TSAI ; Wen-Che TSAI ; Wang-Tso LEE in Autism, 28-9 (September 2024)  

Public ISBD [article]  inAutism > 28-9 (September 2024) . - p.2267-2281 Titre : Investigating the impact of probiotic on neurological outcomes in Rett syndrome: A randomized, double-blind, and placebo-controlled pilot study Type de document : texte imprimé Auteurs : Chia-Jui HSU, Auteur ; Yen-Tzu WU, Auteur ; Hsu-Feng CHU, Auteur ; Jui-Hsiang LIN, Auteur ; Hsin-Pei WANG, Auteur ; Su-Ching HU, Auteur ; Ying-Chieh TSAI, Auteur ; Wen-Che TSAI, Auteur ; Wang-Tso LEE, Auteur Article en page(s) : p.2267-2281 Langues : (en) Mots-clés : dystonia  microbiota-gut-brain axis  probiotics  Rett syndrome Index. décimale : PER Périodiques Résumé : This pilot study investigates the feasibility and assesses the impact of Lactobacillus plantarum PS128 probiotics on the neurological function in Rett syndrome. We conducted a randomized, double-blind, and placebo-controlled trial on Rett syndrome with MECP2 mutation aged between 1 and 50 years in Taiwan. In this pilot study, twice-daily L. plantarum PS128 or placebo was administered for 16 weeks. In addition to feasibility, we also assessed the changes utilizing the Mullen Scales of Early Learning. In total, 36 participants were finally randomized into L. plantarum PS128 (n = 18) or placebo (n = 18) groups. At the end of intervention, the retention rates were 100% for L. plantarum PS128 and 94.44% for placebo, with withdrawal rates of 5.56% for the placebo group. Both groups tolerated well, except for one L. plantarum PS128 participant who reported loose stool. The probiotic group showed a change of 2.19+3.76, while the placebo group had 0.85+5.09 (p = 0.051) in the total age-equivalent scores of Mullen Scales of Early Learning. There was a significant difference in the change of the total score on the Burke-Fahn-Marsden Movement Scale between probiotc group and placebo group ( 12.19+12.12 vs 4.59+4.20, p = 0.020). In leg dystonia, the probiotic group exhibited a change of 4.11+5.11 compared with 0.38+1.50 in the placebo group (p = 0.008). Our findings affirm the feasibility of L. plantarum PS128 in Rett syndrome. Future clinical trials are mandatory to further explore its long-term impact on Rett syndrome. Lay abstract Rett syndrome often involves gastrointestinal symptoms and gut microbiota imbalances. We conducted a study to explore the feasibility of probiotic Lactobacillus plantarum PS128 and the impact on neurological functions in Rett syndrome. The results of our investigation demonstrated that the supplementation of probiotic L. plantarum PS128 was feasible and well tolerated, with 100% retention rate and 0% withdrawal rate. In addition, there was only one participant who had loose stool after taking L. plantarum PS128. Further, there was a tendency to enhance overall cognitive developmental level, as assessed using Mullen Scales of Early Learning. In addition, it significantly improved dystonia, as assessed using the Burke-Fahn-Marsden Movement Scale, in comparison with the placebo group. This study provides a strong foundation for future research and clinical trials exploring the potential of L. plantarum PS128 probiotics as a complementary therapy for individuals with Rett syndrome. En ligne : https://dx.doi.org/10.1177/13623613231225899 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=535 [article] Investigating the impact of probiotic on neurological outcomes in Rett syndrome: A randomized, double-blind, and placebo-controlled pilot study [texte imprimé] / Chia-Jui HSU, Auteur ; Yen-Tzu WU, Auteur ; Hsu-Feng CHU, Auteur ; Jui-Hsiang LIN, Auteur ; Hsin-Pei WANG, Auteur ; Su-Ching HU, Auteur ; Ying-Chieh TSAI, Auteur ; Wen-Che TSAI, Auteur ; Wang-Tso LEE, Auteur . - p.2267-2281. Langues : (en) in Autism > 28-9 (September 2024) . - p.2267-2281 Mots-clés : dystonia  microbiota-gut-brain axis  probiotics  Rett syndrome Index. décimale : PER Périodiques Résumé : This pilot study investigates the feasibility and assesses the impact of Lactobacillus plantarum PS128 probiotics on the neurological function in Rett syndrome. We conducted a randomized, double-blind, and placebo-controlled trial on Rett syndrome with MECP2 mutation aged between 1 and 50 years in Taiwan. In this pilot study, twice-daily L. plantarum PS128 or placebo was administered for 16 weeks. In addition to feasibility, we also assessed the changes utilizing the Mullen Scales of Early Learning. In total, 36 participants were finally randomized into L. plantarum PS128 (n = 18) or placebo (n = 18) groups. At the end of intervention, the retention rates were 100% for L. plantarum PS128 and 94.44% for placebo, with withdrawal rates of 5.56% for the placebo group. Both groups tolerated well, except for one L. plantarum PS128 participant who reported loose stool. The probiotic group showed a change of 2.19+3.76, while the placebo group had 0.85+5.09 (p = 0.051) in the total age-equivalent scores of Mullen Scales of Early Learning. There was a significant difference in the change of the total score on the Burke-Fahn-Marsden Movement Scale between probiotc group and placebo group ( 12.19+12.12 vs 4.59+4.20, p = 0.020). In leg dystonia, the probiotic group exhibited a change of 4.11+5.11 compared with 0.38+1.50 in the placebo group (p = 0.008). Our findings affirm the feasibility of L. plantarum PS128 in Rett syndrome. Future clinical trials are mandatory to further explore its long-term impact on Rett syndrome. Lay abstract Rett syndrome often involves gastrointestinal symptoms and gut microbiota imbalances. We conducted a study to explore the feasibility of probiotic Lactobacillus plantarum PS128 and the impact on neurological functions in Rett syndrome. The results of our investigation demonstrated that the supplementation of probiotic L. plantarum PS128 was feasible and well tolerated, with 100% retention rate and 0% withdrawal rate. In addition, there was only one participant who had loose stool after taking L. plantarum PS128. Further, there was a tendency to enhance overall cognitive developmental level, as assessed using Mullen Scales of Early Learning. In addition, it significantly improved dystonia, as assessed using the Burke-Fahn-Marsden Movement Scale, in comparison with the placebo group. This study provides a strong foundation for future research and clinical trials exploring the potential of L. plantarum PS128 probiotics as a complementary therapy for individuals with Rett syndrome. En ligne : https://dx.doi.org/10.1177/13623613231225899 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=535

Sleep problems in children with autism, attention-deficit hyperactivity disorder, and epilepsy / Fang-Ju TSAI in Research in Autism Spectrum Disorders, 6-1 (January-March 2012)  

Public ISBD [article]  inResearch in Autism Spectrum Disorders > 6-1 (January-March 2012) . - p.413-421 Titre : Sleep problems in children with autism, attention-deficit hyperactivity disorder, and epilepsy Type de document : texte imprimé Auteurs : Fang-Ju TSAI, Auteur ; Huey-Ling CHIANG, Auteur ; Chi-Mei LEE, Auteur ; Susan Shur-Fen GAU, Auteur ; Wang-Tso LEE, Auteur ; Pi-Chuan FAN, Auteur ; Yu-Yu WU, Auteur ; Yen-Nan CHIU, Auteur Année de publication : 2012 Article en page(s) : p.413-421 Langues : Anglais (eng) Mots-clés : Autism spectrum disorders  Attention-deficit/hyperactivity disorder  Epilepsy  Sleep problems  Children Index. décimale : PER Périodiques Résumé : This study aimed to examine sleep problems in children with autism spectrum disorders (ASD), attention-deficit/hyperactivity disorder (ADHD), and epilepsy in clinical settings. We assessed 64 children with ASD, 64 with ADHD, 64 with epilepsy, and 64 typically developing children without any neuropsychiatric disorders by using a sex-and age-matched case–control study design. The parents reported their children's sleep problems. Parents of children with ASD and ADHD reported more current and lifetime sleep problems of their children than parents of children with epilepsy, especially in snoring and restless legs syndrome. Current or lifetime sleep problems did not differ between children with ASD and children with ADHD, or between children with epilepsy and typically developing children. Demographic characteristics and medication status could not fully explain the increased risk of sleep problems in children with ASD and ADHD. Our findings lend evidence to support more sleep problems in children with ASD and ADHD than typically developing children. Our study adds that children with epilepsy do not. These findings emphasize the importance to assess sleep problems in children with neurodevelopmental disorders highly comorbid with ASD or ADHD in clinical practice. En ligne : http://dx.doi.org/10.1016/j.rasd.2011.07.002 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=146 [article] Sleep problems in children with autism, attention-deficit hyperactivity disorder, and epilepsy [texte imprimé] / Fang-Ju TSAI, Auteur ; Huey-Ling CHIANG, Auteur ; Chi-Mei LEE, Auteur ; Susan Shur-Fen GAU, Auteur ; Wang-Tso LEE, Auteur ; Pi-Chuan FAN, Auteur ; Yu-Yu WU, Auteur ; Yen-Nan CHIU, Auteur . - 2012 . - p.413-421. Langues : Anglais (eng) in Research in Autism Spectrum Disorders > 6-1 (January-March 2012) . - p.413-421 Mots-clés : Autism spectrum disorders  Attention-deficit/hyperactivity disorder  Epilepsy  Sleep problems  Children Index. décimale : PER Périodiques Résumé : This study aimed to examine sleep problems in children with autism spectrum disorders (ASD), attention-deficit/hyperactivity disorder (ADHD), and epilepsy in clinical settings. We assessed 64 children with ASD, 64 with ADHD, 64 with epilepsy, and 64 typically developing children without any neuropsychiatric disorders by using a sex-and age-matched case–control study design. The parents reported their children's sleep problems. Parents of children with ASD and ADHD reported more current and lifetime sleep problems of their children than parents of children with epilepsy, especially in snoring and restless legs syndrome. Current or lifetime sleep problems did not differ between children with ASD and children with ADHD, or between children with epilepsy and typically developing children. Demographic characteristics and medication status could not fully explain the increased risk of sleep problems in children with ASD and ADHD. Our findings lend evidence to support more sleep problems in children with ASD and ADHD than typically developing children. Our study adds that children with epilepsy do not. These findings emphasize the importance to assess sleep problems in children with neurodevelopmental disorders highly comorbid with ASD or ADHD in clinical practice. En ligne : http://dx.doi.org/10.1016/j.rasd.2011.07.002 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=146

The clinical and sleep manifestations in children with FOXG1 syndrome / Lee-Chin WONG in Autism Research, 16-5 (May 2023)  

Public ISBD [article]  inAutism Research > 16-5 (May 2023) . - p.953-966 Titre : The clinical and sleep manifestations in children with FOXG1 syndrome Type de document : texte imprimé Auteurs : Lee-Chin WONG, Auteur ; Cheng-Hsien HUANG, Auteur ; Wan-Yun CHOU, Auteur ; Chia-Jui HSU, Auteur ; Wen-Che TSAI, Auteur ; Wang-Tso LEE, Auteur Article en page(s) : p.953-966 Langues : Anglais (eng) Index. décimale : PER Périodiques Résumé : Abstract FOXG1 syndrome is a rare neurodevelopmental disorder associated with severe cognitive dysfunction, autistic behavior, and early-onset hyperkinetic movement disorders. Patients have also been reported to experience sleep disturbances. However, these findings are mainly based on subjective caregivers' reports, and limited by small case numbers. Moreover, no studies using objective evaluation tools, such as actigraphy, have been reported. We analyzed the clinical and sleep manifestations of children with FOXG1 syndrome registered in the FOXG1 Research Foundation Patient Registry database. A total of 258 individuals with FOXG1 syndrome were included in this research. 132 (51.16%) had sleep disturbances. The more impaired of language acquisitions (absence of speech, OR: 3.99, 95%CI = 1.69-9.42, p?= 0.002), hyperkinetic movement disorders (OR: 2.64, 95%CI = 1.34-5.20 p = 0.005) and feeding difficulties (OR: 2.81, 95% CI = 1.52-5.19, p 0.001) were significantly associated with an increase in odds of sleep disturbance after adjusting for age, sex, and antiepileptic drugs. We also performed sleep studies on six individuals with FOXG1 syndrome using The Children's Sleep Habits Questionnaire (CSHQ), the Sleep Disturbance Scale for Children (SDSC), and 7-day data from Actiwatch. The Pittsburgh Sleep Quality Index (PSQI) and 7-day data from Actiwatch were also used to evaluate the sleep condition of their parents. The CSHQ scores revealed bedtime resistance, sleep onset delay, sleep duration, sleep anxiety, night-waking, and parasomnia. Sleep-wake transition disorders and disorders of initiating and maintaining sleep were also suggested by the SDSC scores. The children's actigraphy revealed short sleep durations, impaired sleep efficiency, longer wake after sleep onset, and frequent night-waking. All caregivers reported significantly higher PSQI scores, mildly declined sleep efficiency, and shorter total sleep duration. Sleep disturbances, especially in initiating and maintaining sleep, are common in individuals with FOXG1 syndrome and their caregivers. Sleep disorders in patients with FOXG1 syndrome and their caregivers should be investigated. En ligne : http://dx.doi.org/https://doi.org/10.1002/aur.2916 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=503 [article] The clinical and sleep manifestations in children with FOXG1 syndrome [texte imprimé] / Lee-Chin WONG, Auteur ; Cheng-Hsien HUANG, Auteur ; Wan-Yun CHOU, Auteur ; Chia-Jui HSU, Auteur ; Wen-Che TSAI, Auteur ; Wang-Tso LEE, Auteur . - p.953-966. Langues : Anglais (eng) in Autism Research > 16-5 (May 2023) . - p.953-966 Index. décimale : PER Périodiques Résumé : Abstract FOXG1 syndrome is a rare neurodevelopmental disorder associated with severe cognitive dysfunction, autistic behavior, and early-onset hyperkinetic movement disorders. Patients have also been reported to experience sleep disturbances. However, these findings are mainly based on subjective caregivers' reports, and limited by small case numbers. Moreover, no studies using objective evaluation tools, such as actigraphy, have been reported. We analyzed the clinical and sleep manifestations of children with FOXG1 syndrome registered in the FOXG1 Research Foundation Patient Registry database. A total of 258 individuals with FOXG1 syndrome were included in this research. 132 (51.16%) had sleep disturbances. The more impaired of language acquisitions (absence of speech, OR: 3.99, 95%CI = 1.69-9.42, p?= 0.002), hyperkinetic movement disorders (OR: 2.64, 95%CI = 1.34-5.20 p = 0.005) and feeding difficulties (OR: 2.81, 95% CI = 1.52-5.19, p 0.001) were significantly associated with an increase in odds of sleep disturbance after adjusting for age, sex, and antiepileptic drugs. We also performed sleep studies on six individuals with FOXG1 syndrome using The Children's Sleep Habits Questionnaire (CSHQ), the Sleep Disturbance Scale for Children (SDSC), and 7-day data from Actiwatch. The Pittsburgh Sleep Quality Index (PSQI) and 7-day data from Actiwatch were also used to evaluate the sleep condition of their parents. The CSHQ scores revealed bedtime resistance, sleep onset delay, sleep duration, sleep anxiety, night-waking, and parasomnia. Sleep-wake transition disorders and disorders of initiating and maintaining sleep were also suggested by the SDSC scores. The children's actigraphy revealed short sleep durations, impaired sleep efficiency, longer wake after sleep onset, and frequent night-waking. All caregivers reported significantly higher PSQI scores, mildly declined sleep efficiency, and shorter total sleep duration. Sleep disturbances, especially in initiating and maintaining sleep, are common in individuals with FOXG1 syndrome and their caregivers. Sleep disorders in patients with FOXG1 syndrome and their caregivers should be investigated. En ligne : http://dx.doi.org/https://doi.org/10.1002/aur.2916 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=503

The microstructural change of the brain and its clinical severity association in pediatric Tourette syndrome patients / Chia-Jui HSU in Journal of Neurodevelopmental Disorders, 15 (2023)  

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The sleep problems in individuals with Rett syndrome and their caregivers / Lee-Chin WONG ; Yen-Ju CHU ; Chia-Jui HSU ; Hsin-Pei WANG ; Wen-Che TSAI ; Wang-Tso LEE in Autism, 28-12 (December 2024)  

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Variations of stereotypies in individuals with Rett syndrome: A nationwide cross-sectional study in Taiwan / Lee CHIN WONG in Autism Research, 10-7 (July 2017)  

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