| Dentatorubral pallidoluysian atrophy (DRPLA) is an | | | | experience - their once healthy child, or young adult, |
| autosomal predominant neurodegenerative disease. It | | | | will begin to have seizures, involuntary movements, |
| is characterized by respective combinations of ataxia, | | | | loss of control over voluntary movement, and |
| choreoathetosis, myoclonus, epilepsy, and dementia | | | | delusions. Dentatorubral pallidoluysian atrophy as well |
| as easily as a broad scope of ages at onslaught. In | | | | as other genetic conditions, exhibits a phenomenon |
| patients under the age of 20, dentatorubral | | | | known as anticipation. Anticipation means that the |
| pallidoluysian atrophy presents as seizures, ataxia, | | | | disease increases in severity and presents at a |
| myoclonus, as well as progressive mental | | | | younger age of onset with each successive |
| deterioration. In patients over the age of 20, | | | | generation. |
| dentatorubral pallidoluysian atrophy is suspected when | | | | Patients with Dentatorubral pallidoluysian atrophy |
| a person develops ataxia, choreoathetosis, dementia, | | | | have liberal disease, which means symptoms get |
| and psychiatric disturbances. A positive family history | | | | worse over moment. Children with symptoms, |
| confirms the diagnosis. The size of the repeat | | | | however, normally gain from having a diagnosis |
| transmitted to the next generation depends upon the | | | | established. The cardinal features of dentatorubral |
| size of the parent's repeat and the sex of the | | | | pallidoluysian atrophy are involuntary movements and |
| transmitting parent. Therefore, it is always important | | | | dementia (inability to clearly think, confusion, poor |
| to evaluate both parents of an affected individual | | | | judgement, failure to recognize people, places, and |
| even if they appear to have no symptoms of | | | | things, personality changes) regardless of the age of |
| DRPLA. | | | | onset. A history of ataxia, epilepsy, and mental |
| Dentatorubral pallidoluysian atrophy is sometimes | | | | retardation in children, combined with a positive family |
| initially thought to be Huntington disease. Patients with | | | | history, are often the presenting signs of this |
| the liberal myoclonus epilepsy phenotype had larger | | | | condition. Adult onset dentatorubral pallidoluysian |
| expansions and an early age at onslaught. | | | | atrophy presents with ataxia, choreoathetosis, |
| Furthermore, most of the patients with the | | | | dementia, and psychiatric disturbances. There is |
| progressive myoclonus epilepsy phenotype inherited | | | | currently no cure for dentatorubral pallidoluysian |
| their expanded alleles from their affected fathers. A | | | | atrophy. Epilepsy is treated with anti-seizure |
| possible diagnosis of dentatorubral pallidoluysian | | | | medication. |
| atrophy can be devastating for a family to | | | | |