Treatment of Paroxysmal Nonkinesigenic Dyskinesia

  • Zain Guduru
  • Kapil D. SethiEmail author
Part of the Current Clinical Neurology book series (CCNEU)


Paroxysmal nonkinesigenic dyskinesia (PNKD) is a rare movement disorder characterized by intermittent attacks of hyperkinetic involuntary movements without loss of consciousness. It may be classified into primary (genetic or sporadic) or secondary. The attacks usually occur at rest and may be precipitated by alcohol, coffee, cola, tobacco, fatigue, and emotion. Eighty percent of the patients are of autosomal dominant inheritance with a MR-1 gene mutation on chromosome 2q. Management is the avoidance of precipitating factors. Usually, medical treatment is less rewarding. Medications which have shown benefit in some include carbamazepine, benztropine, or clonazepam. Deep Brain Stimulation (ViM, or GPi target) might be considered in severe and medically refractory cases.


Paroxysmal nonkinesigenic dyskinesia PNKD Dystonia Sleep benefit Myofibrillogenesis regulator 1 gene Mitochondrial dysfunction 

Suggested Reading

  1. Bhatia KP. The paroxysmal dyskinesias. J Neurol. 1999;246:149–55.CrossRefGoogle Scholar
  2. Bhatia KP. Familial (idiopathic) paroxysmal dyskinesias: an update. Semin Neurol. 2001;21:69–74.CrossRefGoogle Scholar
  3. Demirkiran M, Jankovic J. Paroxysmal dyskinesias: clinical features and classification. Ann Neurol. 1995;38:571–9.CrossRefGoogle Scholar
  4. Fink JK, et al. Myofibrillogenesis regulator 1 gene mutations cause paroxysmal dystonic choreoathetosis. Arch Neurol. 2004;61:1025.CrossRefGoogle Scholar
  5. Klein C, et al. Evolving spectrum of PRRT2-associated paroxysmal disorders. Brain. 2015;138:3476–95.CrossRefGoogle Scholar
  6. Nardocci N, et al. A family with paroxysmal nonkinesigenic dyskinesias: evidence of mitochondrial dysfunction. Eur J Paediatr Neurol. 2015;19:64–8.CrossRefGoogle Scholar
  7. Ptacek L, et al. Genotype-phenotype correlation of paroxysmal nonkinesigenic dyskinesia. Neurology. 2007;68:1782–9.CrossRefGoogle Scholar
  8. Sethi KD, Mehta SH. Therapeutics of paroxysmal dyskinesias.Google Scholar
  9. Sethi KD, Hess DC, Huffangle VH, Adams RJ. Acetazolamide treatment of paroxysmal dystonia in central demyelinating disease. Neurology. 1992;42:919–21.CrossRefGoogle Scholar
  10. Van Coller R, et al. Successful treatment of disabling paroxysmal nonkinesigenic dyskinesia with deep brain stimulation of the globus pallidus interna. Stereotact Funct Neurosurg. 2014;92:388–92.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Neurology, Movement Disorders SectionUniversity of KentuckyLexingtonUSA
  2. 2.Augusta UniversityAugustaUSA

Personalised recommendations