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Treatment of paroxysmal dyskinesias in children
1Department of Neurology (Child Neurology), University of Rochester Medical Center, 601 Elmwood Ave., Box 631, Rochester, NY, 14642, USA, Jonathan_Mink@urmc.rochester.edu.
Insights
Paroxysmal dyskinesias are uncommon movement disorders with episodic abnormal movements. Treatment strategies focus on triggers, with anticonvulsants for PKD and benzodiazepines for PNKD, while dietary changes may help PED.
Area of Science:
- Neurology
- Genetics
- Movement Disorders
Background:
- Paroxysmal dyskinesias are rare movement disorders characterized by intermittent abnormal movements.
- Genetic research is advancing classification and understanding, but treatment remains phenotype-driven.
- Triggers for episodes vary among subtypes, influencing therapeutic approaches.
Purpose of the Study:
- To review current understanding and treatment strategies for paroxysmal dyskinesias.
- To highlight the phenotype-based approach to managing these movement disorders.
- To discuss emerging treatment options and future directions.
Main Methods:
- Literature review of paroxysmal dyskinesias.
- Analysis of treatment efficacy based on disorder subtype and precipitating factors.
- Discussion of genetic advances and their clinical implications.
Main Results:
- Paroxysmal kinesigenic dyskinesia (PKD) responds well to anticonvulsants targeting sodium channels.
- Paroxysmal nonkinesigenic dyskinesia (PNKD) often benefits from benzodiazepines and lifestyle modifications.
- Paroxysmal exertion-induced dyskinesia (PED) may improve with ketogenic or modified Atkins diets.
Conclusions:
- Treatment for paroxysmal dyskinesias is tailored to specific phenotypes and triggers.
- Anticonvulsants, benzodiazepines, and dietary interventions show efficacy in different subtypes.
- Further research into the underlying biology may reveal novel therapeutic targets.
Opinion Statement:
Paroxysmal dyskinesia represents a group of uncommon movement disorders that are characterized by episodes of abnormal movements arising from a baseline of normal or nearly normal movement. Recent advances in the genetics of these disorders have helped provide some unification of classification schemes and better understanding. However, the approach to treatment continues to be based on the phenotype more than the genotype. The treatment approach is primarily based on the factors that precipitate the episodes of abnormal movements. For paroxysmal kinesigenic dyskinesia (PKD) in which the spells are triggered by sudden movement, treatment with anticonvulsants that target voltage-sensitive sodium channels (e.g., carbamazepine or phenytoin) is highly effective. For paroxysmal nonkinesigenic dyskinesia (PNKD), treatment with benzodiazepines is effective in many patients. PNKD episodes are often precipitated by caffeine, ethanol, or sleep deprivation, and lifestyle modifications are often helpful. Paroxysmal exertion-induced dyskinesia (PED) is less likely to respond to medications, but the ketogenic diet or modified Atkins diet may provide benefit. As more knowledge is gained about the underlying biology of these disorders, additional treatments may emerge.
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