Paroxysmal Nonkinesigenic Dyskinesia

Paroxysmal Nonkinesigenic Dyskinesia

Paroxysmal Nonkinesigenic Dyskinesia (PNKD) is an episodic movement disorder first described by Mount and Reback in 1940 under the name "Familial paroxysmal choreoathetosis" [Mount, L. A.; Reback, S. : Familial paroxysmal choreoathetosis: preliminary report on a hitherto undescribed clinical syndrome. Arch. Neurol. Psychiat. 44: 841-847, 1940.] . It is a rare hereditary disease that affects various muscular and nervous systems in the body, passing to roughly fifty percent of the offspring. It has been mapped to chromosome 2q31-36 [http://archneur.ama-assn.org/cgi/content/full/56/6/721 Archives of Neurology - Familial Paroxysmal Dystonic Choreoathetosis] ] .

Alternate names

While actually being a separate entity, PNKD is sometimes used as a synonym for Huntington's disease [ [http://www.whonamedit.com/synd.cfm/951.html Who Named it: Huntington's Chorea] ] . The condition can and has been referred to by a number of different names since it was first described, including the following:
* Paroxysmal nonkinesigenic dyskinesia
* PNKD
* Paroxysmal dystonic choreoathetosis
* PDC
* Mount-Reback syndrome (in honor of the two doctors that classified it)
* Nonkinesigenic choreoathetosis
* Familial paroxysmal choreoathetosis
* Choreoathetosis familial paroxysmal
* FPD1
* DYT8
* Dystonia 8

ymptoms

The condition manifests itself as attacks lasting from a few minutes to several hours. Episodes only happen when the individual is awake, and they remain conscious throughout the attack. Symptoms are most severe in youth and lessen with age. Sufferers can have multiple attacks on a daily basis or may have periods of weeks or months between attacks. Symptoms experienced during attacks can vary and include dystonia, chorea, athetosis, ballismus, or a combination. [ [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=pnknd NCBI Bookshelf -- GeneReviews -- Familial Paroxysmal Nonkinesigenic Dyskinesia] ]

Triggers

While not the same in all people, there are several common triggers that can precipitate an attack: [ [http://www.wrongdiagnosis.com/medical/mount_reback_syndrome.htm WrongDiagnosis.com - Mount-Reback syndrome] ]
* Moderate to high consumption of stimulants, such as alcohol, caffeine, or nicotine.
* Low amounts of energy due to hunger, lack of sleep, illness, or physical fatigue.
* Moderate to high presence of stress.
* Menstruation and ovulation.

Treatment

Most pharmacological treatments work poorly, but the best treatment is a low dosage of clonazepam, a muscle relaxant. Affected individuals have reported garlic to be effective for softening the attacks, but no studies have been done on this.

References


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