Learn About Dentatorubral-Pallidoluysian Atrophy

What is the definition of Dentatorubral-Pallidoluysian Atrophy?

Dentatorubral-pallidoluysian atrophy, commonly known as DRPLA, is a progressive brain disorder that causes involuntary movements, mental and emotional problems, and a decline in thinking ability. The average age of onset of DRPLA is 30 years, but this condition can appear anytime from infancy to mid-adulthood.

Save information for later
Sign Up
What are the causes of Dentatorubral-Pallidoluysian Atrophy?

DRPLA is caused by a mutation in the ATN1 gene. This gene provides instructions for making a protein called atrophin 1. Although the function of atrophin 1 is unclear, it likely plays an important role in nerve cells (neurons) in many areas of the brain.

How prevalent is Dentatorubral-Pallidoluysian Atrophy?

DRPLA is most common in the Japanese population, where it has an estimated incidence of 2 to 7 per million people. This condition has also been seen in families from North America and Europe.

Is Dentatorubral-Pallidoluysian Atrophy an inherited disorder?

This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. In most cases, an affected person has one parent with the condition.

Who are the top Dentatorubral-Pallidoluysian Atrophy Local Doctors?
Elite
Highly rated in
25
conditions
Pediatrics
Neurology

Childrens Health

Childrens Medical Center Dallas

1935 Medical District Dr 
Dallas, TX 75235

Berge Minassian is a Pediatrics specialist and a Neurologist in Dallas, Texas. Dr. Minassian has been practicing medicine for over 30 years and is rated as an Elite doctor by MediFind in the treatment of Dentatorubral-Pallidoluysian Atrophy. He is also highly rated in 25 other conditions, according to our data. His top areas of expertise are Dentatorubral-Pallidoluysian Atrophy, Lafora Disease, Epilepsy with Myoclonic-Atonic Seizures, and Myoclonus Epilepsy Partial Seizure. He is board certified in Pediatric Medicine and Neurology and licensed to treat patients in Texas. Dr. Minassian is currently accepting new patients.

Elite
Highly rated in
10
conditions

The Hospital For Sick Children

Program In Genetics And Genome Biology, 555 University Avenue 
Toronto, ON, CA M5G1X

Saija Ahonen is in Toronto, Canada. Ahonen is rated as an Elite expert by MediFind in the treatment of Dentatorubral-Pallidoluysian Atrophy. She is also highly rated in 10 other conditions, according to our data. Her top areas of expertise are Lafora Disease, Dentatorubral-Pallidoluysian Atrophy, Progressive Myoclonic Epilepsy, and Epilepsy with Myoclonic-Atonic Seizures.

 
 
 
 
Learn about our expert tiers
Learn more
Elite
Highly rated in
23
conditions

Bellaria Hospital

Bellaria Hospital 
Bologna, IT 

Roberto Michelucci is in Bologna, Italy. Michelucci is rated as an Elite expert by MediFind in the treatment of Dentatorubral-Pallidoluysian Atrophy. He is also highly rated in 23 other conditions, according to our data. His top areas of expertise are Lafora Disease, Dentatorubral-Pallidoluysian Atrophy, Autosomal Dominant Partial Epilepsy with Auditory Features, and Partial Familial Epilepsy.

What are the latest Dentatorubral-Pallidoluysian Atrophy Clinical Trials?
Prospective, Longitudinal, Observational Study of the Natural History and Functional Status of Patients With Lafora Disease
Match to trials
Find the right clinical trials for you in under a minute
Get started
An Open-Label, Safety Study for Previously Treated Vatiquinone (PTC743) Participants With Inherited Mitochondrial Disease
Who are the sources who wrote this article ?

Published Date:updated Last, November

Published By: National Institutes of Health

What are the Latest Advances for Dentatorubral-Pallidoluysian Atrophy?
Sodium voltage-gated channel alpha subunit 9 mutation in epilepsy.
Neuronal ceroid lipofuscinosis. Type 6 late infantile variant in two compound heterozygous siblings with novel mutations.
Tired of the same old research?
Check Latest Advances
Clinical and molecular characterization of Unverricht-Lundborg disease among Egyptian patients.