Objective: Report the effectiveness of various medications in the treatment of paroxysmal kinesigenic dyskinesia (PKD).

Background: PKD is an episodic movement disorder characterized by attacks of dyskinesias precipitated by a sudden movement or startle. Low dose carbamazepine is well established as the therapy of choice for PKD. However, if there is a contraindication or adverse reaction to carbamazepine, there is limited information regarding which medications are suitable alternatives.

Method: We retrospectively reviewed the electronic medical records of patients with a diagnosis of pediatric onset PKD that were evaluated at our center between 2001 and 2019. We collected demographic information, medications trialed, daily dosage of medication, and treatment response.

Results: We identified sixteen patients with pediatric onset PKD. Carbamazepine (mean dose 280mg, range 100-800mg/day) was the initial treatment of choice in the majority of patients (12), and produced complete remission of PKD in all patients. However, two patients had to discontinue Carbamazepine due to adverse events (leukopenia and drug rash with eosinophilia and systemic symptoms, respectively). Three patients used lamotrigine at a mean dose of 75mg/day (range 25-100mg/day); all of these patients had complete remission (range of remission at last follow-up: 3-7 years). Oxcarbazepine was used in 3 patients at a mean dose of 600mg/day (range 300-900mg/day) and all 3 patients had complete remission (range of remission at last follow-up: 1-9 years). Levetiracetam was used in 2 patients (1000mg/day and 1500mg/day) and was discontinued in both of these patients due to ineffectiveness. Phenytoin (150mg/day) was used in one patient and they were episode free for 6 years at last follow-up. One patient was on 1500mg/day of Valproate, but this was discontinued due to ineffectiveness.

Conclusion: In addition to carbamazepine, low doses of lamotrigine, oxcarbazepine, and phenytoin appear to be effective in PKD. However, levetiracetam and valproate may be ineffective options for PKD.

Previously presented at the AAN 2021 meeting

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MDS Abstracts - https://www.mdsabstracts.org/abstract/alternative-medications-for-paroxysmal-kinesigenic-dyskinesia/