Myoclonus-dystonia syndrome

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ORPHA:36899OMIM:616398G24.1
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3Specialists8Treatment centers

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Overview

Myoclonus-dystonia syndrome (also called M-D syndrome, DYT11, or epsilon-sarcoglycan-related dystonia) is a rare movement disorder that affects the nervous system. It causes two main types of involuntary movements: myoclonus (sudden, brief muscle jerks that look like electric shocks) and dystonia (sustained muscle contractions that twist or hold the body in abnormal positions). These movements most often affect the upper body, including the arms, neck, and trunk, while the legs are usually less involved. Symptoms typically begin in childhood or early adolescence, often before age 20. The muscle jerks can be mild or severe enough to interfere with everyday tasks like writing, eating, or holding objects. Many people with this condition also experience psychiatric symptoms such as anxiety, depression, obsessive-compulsive disorder (OCD), or alcohol use disorder. Interestingly, alcohol often temporarily reduces the movement symptoms, which can lead some people to use it as a way to cope. The good news is that myoclonus-dystonia is generally not life-shortening, and many people live full lives with proper treatment. Medications can help reduce the muscle jerks and dystonia. For people who do not respond well to medication, a surgical treatment called deep brain stimulation (DBS) has shown very promising results. With the right care team and support, many people with this condition are able to manage their symptoms and maintain a good quality of life.

Also known as:

Key symptoms:

Sudden, brief muscle jerks (myoclonus), especially in the arms and upper bodyInvoluntary muscle twisting or abnormal postures (dystonia)Shaky or jerky handwritingDifficulty holding objects or performing fine hand movementsNeck twisting or abnormal head positionTremor-like shaking of the armsAnxiety or panic attacksDepressionObsessive-compulsive behaviorsAlcohol use disorder (often linked to self-medicating symptoms)Symptoms that temporarily improve with alcoholMild or absent leg involvement compared to armsSymptoms that worsen with stress or fatigue

Clinical phenotype terms (11)— hover any for plain English
Spinal myoclonusHP:0010531Limb myoclonusHP:0045084Writer's crampHP:0002356Personality disorderHP:0012075Panic attackHP:0025269
Inheritance

Autosomal dominant

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

Age of Onset

Juvenile

Begins in the teen years

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Myoclonus-dystonia syndrome.

View clinical trials →

No actively recruiting trials found for Myoclonus-dystonia syndrome at this time.

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Specialists

3 foundView all specialists →
ER
Emmanuel Roze
Specialist
PI on 1 active trial
EP
Emmanuel Roze, Md, PhD
Specialist
PI on 1 active trial

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 Myoclonus-dystonia syndrome.

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Community

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Latest news about Myoclonus-dystonia syndrome

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.Which gene mutation do I have, and what does that mean for my family members?,Should my children or siblings be tested for this condition?,What medications would you recommend first, and what side effects should I watch for?,Am I a candidate for deep brain stimulation, and when should we consider it?,How should I address the anxiety or depression that comes with this condition?,Are there any clinical trials I should know about?,What specialists should be part of my care team?

Common questions about Myoclonus-dystonia syndrome

What is Myoclonus-dystonia syndrome?

Myoclonus-dystonia syndrome (also called M-D syndrome, DYT11, or epsilon-sarcoglycan-related dystonia) is a rare movement disorder that affects the nervous system. It causes two main types of involuntary movements: myoclonus (sudden, brief muscle jerks that look like electric shocks) and dystonia (sustained muscle contractions that twist or hold the body in abnormal positions). These movements most often affect the upper body, including the arms, neck, and trunk, while the legs are usually less involved. Symptoms typically begin in childhood or early adolescence, often before age 20. The musc

How is Myoclonus-dystonia syndrome inherited?

Myoclonus-dystonia syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Myoclonus-dystonia syndrome typically begin?

Typical onset of Myoclonus-dystonia syndrome is juvenile. Age of onset can vary across affected individuals.

Which specialists treat Myoclonus-dystonia syndrome?

3 specialists and care centers treating Myoclonus-dystonia syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.