Abstract Introduction Dopamine agonists are infrequently used for restless leg syndrome (RLS) in pediatrics. This case describes a rare example of restless leg augmentation in pediatrics. Report of case(s) A 14-year-old female with Kufor-Rakeb syndrome presented with snoring, difficulty initiating and maintaining sleep, and hypersomnia. One year prior, she began carbidopa/levodopa for management of her muscle rigidity, bradykinesia, and tremors. While her parkinsonism symptoms improved, she developed significant restlessness at night with frequent awakenings. Polysomnography indicated mild-to-moderate obstructive sleep apnea and an elevated periodic limb movement index of 16.4 events/hr. Sleep apnea resolved after adenotonsillectomy. However, restlessness and limb movement severity persisted. Over time, restlessness began occurring earlier in the evening and spreading to her upper body. These changes were noted to correlate with dose increases of carbidopa/levodopa therapy raising concerns for dopaminergic augmentation. Carbidopa/levodopa could not be discontinued given limited alternate therapies approved for pediatric patients with this condition. To avoid further augmentation, sleep and neurology providers worked with the patient to administer her nighttime total dose as two separate doses, with one given 2 hours prior to bedtime and the other at bedtime. While not standard administration, the patient’s restlessness, nighttime awakenings, and parkinsonism symptoms drastically improved. Given augmentation concerns, sleep and neurology providers made the decision to gradually wean the patient off carbidopa/levodopa therapy with interval use of a low-dose ropinirole patch. After this transition, the sleep provider will consider supplementing an alpha-2-delta ligand to assist with any sub-optimal control of restless legs. Lastly, her ferritin was notably low, prompting iron supplementation. After ferritin levels were optimized, nighttime restlessness significantly improved. Conclusion Augmentation of RLS is rarely seen in pediatrics. This case emphasizes the importance of multidisciplinary management of patients on dopaminergic medication with RLS. Given limited agents are available to treat pediatric movement disorders, this specific population may benefit from non-standard interventions such as more frequent and strategically timed dosing. When possible, sleep providers should consider other agents with less risk of augmentation, such as alpha-2-delta ligands. Finally, this case emphasizes the clinical impact of iron supplementation given significant improvements in symptoms after optimizing ferritin storage. Support (if any)
Roark et al. (Fri,) studied this question.