Neuronal Ceroid Lipofuscinosis and Associated Sleep Abnormalities

Title

Neuronal Ceroid Lipofuscinosis and Associated Sleep Abnormalities

Creator

Lehwald L M; Pappa R; Steward S; de los Reyes E

Publisher

Pediatric Neurology

Date

2016

Subject

sleep disturbance/disorders; NCL3; trajectory; characteristics; seizures; loss of vision

Description

Purpose: The aims of this study were to evaluate sleep difficulties in children with neuronal ceroid lipofuscinosis and to determine the association between the sleep difficulties and the onset of seizures and loss of vision.Method: We recruited individuals with a confirmed diagnosis of neuronal ceroid lipofuscinosis. We obtained information from the caregiver using the validated Children's Sleep Habits Questionnaire which is a sleep instrument for both behaviorally and medically based problems. Additional information was collected including onset of symptoms, treatment trials, and screen for restless leg syndrome symptoms.Results: In our cohort of 54 individuals, 96.3% had sleep scores consistent with a sleep disturbance. Sleep subscale analysis provided additional insight into the characteristics of the sleep disturbance. Fifty two of the 54 patients had at least one abnormal sleep subscale. The onset of sleep disturbance was associated with the onset of both seizures (ρ = 0.5834, P < 0.0001) and loss of vision (ρ = 0.3840, P = 0.0084). Restless leg syndrome symptoms were reported in 35.2%.Conclusion: Children with neuronal ceroid lipofuscinosis have a high burden of sleep disturbances. Using the results of a sleep disturbance screening tool can help to identify the most disturbing symptoms. Targeted treatment of sleep disturbance may improve the quality of life for the patient and family.

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Citation

Lehwald L M; Pappa R; Steward S; de los Reyes E, “Neuronal Ceroid Lipofuscinosis and Associated Sleep Abnormalities,” Pediatric Palliative Care Library, accessed October 25, 2021, https://pedpalascnetlibrary.omeka.net/items/show/16724.

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