Spinocerebellar ataxia-dysmorphism syndrome

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ORPHA:1185OMIM:271270G11.8
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15Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Spinocerebellar ataxia-dysmorphism syndrome is an extremely rare genetic condition that affects the brain, physical development, and overall growth. The term 'spinocerebellar ataxia' refers to problems with the cerebellum, the part of the brain that controls balance and coordination. 'Dysmorphism' means there are unusual physical features present from birth. Children born with this syndrome typically show poor coordination (ataxia), intellectual disability, short stature, and distinctive facial features. The condition is present from birth or early infancy and affects multiple body systems. Key symptoms include difficulty with balance and walking, delayed developmental milestones, intellectual disability, and characteristic facial features such as a broad nasal bridge, low-set ears, or other subtle differences in facial structure. Growth may be slower than expected, and some children may have additional skeletal abnormalities. Because this condition is so rare, there is no specific cure or targeted treatment available. Management focuses on supportive care, including physical therapy to help with coordination and movement, occupational therapy for daily living skills, speech therapy if needed, and special education support. Regular follow-up with multiple specialists is important to address the various aspects of the condition. Research into this syndrome is very limited due to its extreme rarity, and much of what is known comes from a small number of reported cases in the medical literature.

Key symptoms:

Poor balance and coordination (ataxia)Intellectual disabilityShort stature or growth delayUnusual facial featuresDelayed developmental milestonesDifficulty walkingLow muscle toneSpeech and language delaysSkeletal abnormalitiesLearning difficultiesSmall head size (microcephaly)

Clinical phenotype terms (26)— hover any for plain English
Genu recurvatumHP:0002816Slender long boneHP:0003100
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Spinocerebellar ataxia-dysmorphism syndrome.

View clinical trials →

No actively recruiting trials found for Spinocerebellar ataxia-dysmorphism syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Spinocerebellar ataxia-dysmorphism syndrome community →

Specialists

15 foundView all specialists →
LM
Laurent Servais, MD
Specialist
PI on 6 active trials1 Spinocerebellar ataxia-dysmorphism syndrome publication
HN
Hilde L Nilsen
Specialist
PI on 1 active trial
AP
Alexander F. Karelin, PhD
Specialist
PI on 1 active trial
DF
David Coman, MBBS FRACP
Specialist
PI on 2 active trials
SD
Stefan Zielen, Prof. Dr.
Specialist
PI on 5 active trials
KF
Kathleen Friel
COLORADO SPRINGS, CO
Specialist
PI on 1 active trial
MP
Marie Claire VINCENT, PhD-PharmaD
Specialist
PI on 2 active trials
JM
John T. Sandlund, MD
MEMPHIS, TN
Specialist
PI on 2 active trials
LM
Luca Benatti, MD
Specialist
PI on 1 active trial1 Spinocerebellar ataxia-dysmorphism syndrome publication
DM
Dirk Thye, MD
SOUTH SAN FRANCISCO, CA
Specialist
PI on 2 active trials
MP
Michel Willemsen, Prof.
Specialist
PI on 1 active trial
TP
Todd Kirby, PhD
BOULDER, CO
Specialist
PI on 1 active trial
TM
Thomas O Crawford, M.D.
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 Spinocerebellar ataxia-dysmorphism syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Spinocerebellar ataxia-dysmorphism syndrome

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Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

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.What specific tests can confirm this diagnosis in my child?,Are there any new genetic tests that might identify the exact cause?,What therapies do you recommend to help with coordination and development?,How often should my child be seen by a neurologist and other specialists?,Are there any clinical trials or research studies we could participate in?,What should I watch for that would require urgent medical attention?,What resources are available to help with educational planning and support?

Common questions about Spinocerebellar ataxia-dysmorphism syndrome

What is Spinocerebellar ataxia-dysmorphism syndrome?

Spinocerebellar ataxia-dysmorphism syndrome is an extremely rare genetic condition that affects the brain, physical development, and overall growth. The term 'spinocerebellar ataxia' refers to problems with the cerebellum, the part of the brain that controls balance and coordination. 'Dysmorphism' means there are unusual physical features present from birth. Children born with this syndrome typically show poor coordination (ataxia), intellectual disability, short stature, and distinctive facial features. The condition is present from birth or early infancy and affects multiple body systems. K

How is Spinocerebellar ataxia-dysmorphism syndrome inherited?

Spinocerebellar ataxia-dysmorphism syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Spinocerebellar ataxia-dysmorphism syndrome typically begin?

Typical onset of Spinocerebellar ataxia-dysmorphism syndrome is neonatal. Age of onset can vary across affected individuals.

Which specialists treat Spinocerebellar ataxia-dysmorphism syndrome?

15 specialists and care centers treating Spinocerebellar ataxia-dysmorphism syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.