Levetiracetam as a Therapeutic Option in the Treatment of MR1-Associated Paroxysmal Nonkinesigenic Dyskinesias in Children: A Case Report

Background/Purpose: Paroxysmal nonkinesigenic dyskinesias are a rare movement disorder presenting with attacks of dyskinesias triggered by stress, fatigue, caffeine intake, or alcohol intake. Frequently a causative autosomal-dominant mutation in the MR1 gene can be detected, and symptoms often appear in childhood. Apart from avoidance of triggers, benzodiazepines have priority in treatment.

Methods: We present a female patient with inherited MR1-associated PNKD with the first symptoms appearing at the age of 20 months. Attacks with dyskinesias of hands and arms were mostly triggered by fatigue. When frequency and duration increased and oropharyngeal involvement was additionally observed, we started a therapy with levetiracetam, due to the known side effects of benzodiazepines and poor response to benzodiazepines to the mother who also suffers from the disease.

Results: Under levetiracetam, frequency and intensity of the attacks decreased after a short time.

Conclusion: Considering the side effects and in the literature repeatedly reported poor and unstable response to benzodiazepines in PNKD, especially in pediatric patients, it raises the question of alternative therapies. Following our experience in the reported patient, levetiracetam seems to be a recommendable option. The causal and exact mode of action is not clear, and a positive effect on the impaired calcium-related neurotransmission caused by MR1 mutations is suspected.

Publication History

Article published online:
28 October 2021