Inherited congenital spastic tetraplegia

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ORPHA:210141OMIM:617008G82.4
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Overview

Inherited congenital spastic tetraplegia is a rare genetic condition that affects the nervous system, specifically the parts of the brain and spinal cord that control movement. In this condition, all four limbs (both arms and both legs) are affected by spasticity, which means the muscles are abnormally stiff and tight. This stiffness makes voluntary movement very difficult. The condition is present from birth or becomes apparent very early in life. Children with this condition typically show significant delays in reaching motor milestones such as sitting, crawling, and walking. Many individuals may never achieve independent walking. In addition to movement problems, some people may experience intellectual disability, difficulty with speech, seizures, and problems with swallowing or feeding. The severity can vary from person to person, even within the same family. Treatment is mainly supportive and focuses on managing symptoms. Physical therapy, occupational therapy, and speech therapy are cornerstones of care. Medications to reduce muscle stiffness, such as baclofen or botulinum toxin injections, may be used. Orthopedic surgery may be needed in some cases to correct joint or bone problems caused by chronic spasticity. There is currently no cure for this condition, but early and consistent therapy can help maximize function and quality of life.

Also known as:

Key symptoms:

Stiff and tight muscles in all four limbsDifficulty with voluntary movementsDelayed motor milestones such as sitting and walkingInability to walk independently in severe casesIntellectual disabilitySpeech and language difficultiesSeizures or epilepsyFeeding and swallowing difficultiesPoor head controlAbnormal postureJoint contractures from chronic muscle tightnessDroolingVision problemsScoliosis or other spine curvature

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

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 Inherited congenital spastic tetraplegia.

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No actively recruiting trials found for Inherited congenital spastic tetraplegia at this time.

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No specialists are currently listed for Inherited congenital spastic tetraplegia.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

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 Inherited congenital spastic tetraplegia.

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Community

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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.What specific genetic testing should be done to confirm the diagnosis and identify the cause?,How severe is my child's condition, and what level of function can we expect over time?,What therapies should we start right away, and how often should they occur?,Are there medications that could help reduce muscle stiffness, and what are the side effects?,Should we be concerned about seizures, and how would we recognize them?,What feeding or nutritional support might be needed?,Are there any clinical trials or emerging treatments we should know about?

Common questions about Inherited congenital spastic tetraplegia

What is Inherited congenital spastic tetraplegia?

Inherited congenital spastic tetraplegia is a rare genetic condition that affects the nervous system, specifically the parts of the brain and spinal cord that control movement. In this condition, all four limbs (both arms and both legs) are affected by spasticity, which means the muscles are abnormally stiff and tight. This stiffness makes voluntary movement very difficult. The condition is present from birth or becomes apparent very early in life. Children with this condition typically show significant delays in reaching motor milestones such as sitting, crawling, and walking. Many individua

At what age does Inherited congenital spastic tetraplegia typically begin?

Typical onset of Inherited congenital spastic tetraplegia is neonatal. Age of onset can vary across affected individuals.