1. The clinical and sleep manifestations in children with FOXG1 syndrome.
- Author
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Wong, Lee‐Chin, Huang, Cheng‐Hsien, Chou, Wan‐Yun, Hsu, Chia‐Jui, Tsai, Wen‐Che, and Lee, Wang‐Tso
- 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. Lay Summary: FOXG1 syndrome is a rare disorder with several comorbidities, including sleep disorders. However, studies focusing on sleep conditions in FOXG1 syndrome are scarce. Our studies showed that sleep disturbances occur across different genders, ages, and genotypes in FOXG1 syndrome. Hyperkinetic movements, feeding difficulties, and absence of language acquisition significantly increased the probability of sleep disturbance. Our results also demonstrated greater difficulty in initiating and maintaining sleep, as identified in actigraphy studies, and sleep‐disordered breathing symptoms, as revealed by the report questionnaires submitted by the parents of six children with FOXG1 syndrome. Caregivers of children with this condition also tended to have shorter total sleep durations. [ABSTRACT FROM AUTHOR]
- Published
- 2023
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