Autosomal recessive spastic paraplegia type 77

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ORPHA:466722OMIM:617046G11.4
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Overview

Autosomal recessive spastic paraplegia type 77 (SPG77) is an extremely rare inherited neurological condition that primarily affects the nervous system, leading to progressive stiffness and weakness in the legs. It is caused by mutations in the FARS2 gene, which provides instructions for making an enzyme important for energy production inside cells, particularly in the mitochondria (the energy factories of cells). When this enzyme does not work properly, nerve cells that control movement gradually become damaged. People with SPG77 typically develop progressive spasticity (stiffness) in the lower limbs, which makes walking increasingly difficult over time. Additional features may include problems with balance and coordination (cerebellar ataxia), and in some cases, cognitive difficulties or epilepsy. The severity of symptoms can vary between individuals, even within the same family. Because SPG77 is so rare, there is currently no specific cure or disease-modifying treatment available. Management focuses on relieving symptoms and maintaining quality of life. This includes physical therapy to manage spasticity, medications to reduce muscle stiffness, and supportive care from a team of specialists. Research into hereditary spastic paraplegias is ongoing, and understanding of this condition continues to grow.

Also known as:

Key symptoms:

Progressive leg stiffness (spasticity)Difficulty walkingLeg weaknessProblems with balance and coordinationIncreased muscle tone in the legsExaggerated reflexesDifficulty with fine movementsSeizures or epilepsy in some casesCognitive difficulties in some casesFatigueFoot deformities such as high archesBladder control problems

Clinical phenotype terms (37)— hover any for plain English
Weakness due to upper motor neuron dysfunctionHP:0010549Scissor gaitHP:0012407Lower limb amyotrophyHP:0007210Muscle abnormality related to mitochondrial dysfunctionHP:0003800Macrodontia of permanent maxillary central incisorHP:0000675
Inheritance

Autosomal recessive

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

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Autosomal recessive spastic paraplegia type 77.

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No specialists are currently listed for Autosomal recessive spastic paraplegia type 77.

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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 Autosomal recessive spastic paraplegia type 77.

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Community

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Questions for your doctor

Bring these to your next appointment

  • Q1.How quickly is this condition likely to progress in my case or my child's case?,What physical therapy program would be most helpful right now?,Are there medications that can help with the leg stiffness?,Should other family members be tested for FARS2 mutations?,Are there any clinical trials or research studies we could participate in?,What assistive devices or home modifications should we consider?,How often should we schedule follow-up neurological evaluations?

Common questions about Autosomal recessive spastic paraplegia type 77

What is Autosomal recessive spastic paraplegia type 77?

Autosomal recessive spastic paraplegia type 77 (SPG77) is an extremely rare inherited neurological condition that primarily affects the nervous system, leading to progressive stiffness and weakness in the legs. It is caused by mutations in the FARS2 gene, which provides instructions for making an enzyme important for energy production inside cells, particularly in the mitochondria (the energy factories of cells). When this enzyme does not work properly, nerve cells that control movement gradually become damaged. People with SPG77 typically develop progressive spasticity (stiffness) in the low

How is Autosomal recessive spastic paraplegia type 77 inherited?

Autosomal recessive spastic paraplegia type 77 follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.