Abstract
Objective: This study aims to describe and analyze both polysomnography (PSG) and clinical sleep evaluation data of patients with Alternating Hemiplegia of Childhood (AHC).
Background: Patients with AHC, a disorder that starts during the first 18 months of life, experience bouts of hemiplegia and other paroxysmal spells that resolve during sleep. Patients often have multiple comorbidities that could negatively affect sleep, yet sleep quality and sleep pathology in AHC are not well characterized.
Design/Methods: We analyzed nocturnal PSG and clinical sleep evaluation results of a cohort of 26 consecutive patients (22 children, 4 adults) with AHC evaluated in our AHC multidisciplinary clinic according to our comprehensive AHC clinical pathway. This pathway includes, irrespective of presence or absence of any sleep related initial chief complaints, both a baseline PSG and evaluation by a board-certified sleep specialist.
Results: Out of the 22 children, 21 had sleep problems, abnormal PSG, or both. Twenty had at least one type of sleep problem. On PSG, 6 out of 22 had obstructive sleep apnea (OSA) and 8 out of 22 had central sleep apnea. Patients had an abnormal mean overall apnea-hypopnea index of 5.8 (range 0–38.7) and an abnormal mean arousal index of 15.0 (range 4.8–46.6). Based on sleep history, 16 children had difficulty falling asleep, staying asleep, or both, 9 had behavioral insomnia of childhood, and 2 had delayed sleep-wake phase syndrome. Out of the 4 adults, 3 had difficulty falling asleep, 2 had difficulty staying asleep, 4 had night-time awakenings, and 3 had frequent snoring. PSG revealed OSA in 2 out of 4. Other sleep related complaints in adults included prolonged sleep, daytime sleepiness, sensations, pain, and movements during sleep.
Conclusions: Sleep dysfunction is common amongst pediatric and adult patients with AHC. Physicians should routinely screen for sleep pathology, with a low threshold to obtain a nocturnal PSG.
Disclosure: Dr. Tran has nothing to disclose. Dr. Kansagra has nothing to disclose. Dr. Ghusayni has nothing to disclose. Dr. Kherallah has nothing to disclose. Dr. Gunduz has nothing to disclose. Dr. McLean has nothing to disclose. Dr. Prange has nothing to disclose. Dr. Kravitz has nothing to disclose. Dr. Mikati has nothing to disclose.
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