Paroxysmal dystonic choreathetosis with episodic ataxia and spasticity

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ORPHA:53583OMIM:601042G24.8
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Overview

Paroxysmal dystonic choreathetosis with episodic ataxia and spasticity is a very rare neurological condition that causes sudden, unpredictable episodes of abnormal movements and coordination problems. It is also sometimes called by its initials or grouped under the broader category of paroxysmal movement disorders. The condition affects the nervous system in a way that causes the body to produce involuntary, twisting, or writhing movements (called dystonia and choreathetosis) that come and go in episodes. During these episodes, a person may also experience poor balance and coordination (ataxia), making it hard to walk or perform everyday tasks. Between episodes, some people have ongoing muscle stiffness (spasticity). Episodes can be triggered by things like stress, fatigue, caffeine, or sudden movement, and they can last from minutes to hours. The condition typically begins in childhood or early adulthood. While it is not life-threatening in most cases, it can significantly affect quality of life, school performance, and independence. Treatment focuses on reducing the frequency and severity of episodes, often using medications such as anticonvulsants or other drugs that calm abnormal nerve signals. With the right management, many people are able to reduce how often episodes occur and live more comfortably.

Also known as:

Key symptoms:

Sudden episodes of involuntary twisting or writhing movementsUncontrolled, dance-like movements of the arms and legsLoss of balance and coordination during episodesMuscle stiffness (spasticity) that may be present between episodesEpisodes that can last from a few minutes to several hoursDifficulty walking or standing during an episodeSlurred speech during episodesEpisodes triggered by stress, fatigue, or sudden movementNormal or near-normal neurological function between episodes in some patients

Clinical phenotype terms (13)— hover any for plain English
Paroxysmal dyskinesiaHP:0007166Episodic ataxiaHP:0002131
Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Paroxysmal dystonic choreathetosis with episodic ataxia and spasticity.

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No actively recruiting trials found for Paroxysmal dystonic choreathetosis with episodic ataxia and spasticity at this time.

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No specialists are currently listed for Paroxysmal dystonic choreathetosis with episodic ataxia and spasticity.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Paroxysmal dystonic choreathetosis with episodic ataxia and spasticity.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.Which gene or genes should be tested to confirm my diagnosis, and what does the result mean for my family?,What medications are most likely to help reduce my episodes, and what are the side effects?,Should I try a ketogenic diet, and is GLUT1 deficiency suspected in my case?,What triggers should I avoid, and how can I identify my personal triggers?,Are there any clinical trials or research studies I could participate in?,What should I do — or what should my family do — if I have a severe or prolonged episode?,How is this condition likely to change over time, and what can I do now to protect my long-term health?

Common questions about Paroxysmal dystonic choreathetosis with episodic ataxia and spasticity

What is Paroxysmal dystonic choreathetosis with episodic ataxia and spasticity?

Paroxysmal dystonic choreathetosis with episodic ataxia and spasticity is a very rare neurological condition that causes sudden, unpredictable episodes of abnormal movements and coordination problems. It is also sometimes called by its initials or grouped under the broader category of paroxysmal movement disorders. The condition affects the nervous system in a way that causes the body to produce involuntary, twisting, or writhing movements (called dystonia and choreathetosis) that come and go in episodes. During these episodes, a person may also experience poor balance and coordination (ataxia

How is Paroxysmal dystonic choreathetosis with episodic ataxia and spasticity inherited?

Paroxysmal dystonic choreathetosis with episodic ataxia and spasticity follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Paroxysmal dystonic choreathetosis with episodic ataxia and spasticity typically begin?

Typical onset of Paroxysmal dystonic choreathetosis with episodic ataxia and spasticity is childhood. Age of onset can vary across affected individuals.