Autosomal recessive spastic paraplegia type 9B

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ORPHA:447760OMIM:616586G11.4
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Overview

Autosomal recessive spastic paraplegia type 9B (also called SPG9B or AR-SPG9) is a rare inherited neurological condition that belongs to a large group of disorders known as hereditary spastic paraplegias (HSPs). These conditions share a common feature: progressive stiffness and weakness in the legs caused by damage to the long nerve fibers that run from the brain down the spinal cord. In SPG9B specifically, mutations in the ALDH18A1 gene disrupt the normal function of an enzyme involved in making certain amino acids, which leads to nerve cell damage over time. People with SPG9B typically experience gradually worsening leg stiffness (spasticity), difficulty walking, and muscle weakness. Some individuals also develop intellectual disability, cataracts (clouding of the eye lens), and other neurological features, making this a more complex or 'complicated' form of hereditary spastic paraplegia. Symptoms often begin in childhood or early adulthood, though the age of onset can vary. There is currently no cure for SPG9B. Treatment focuses on managing symptoms, improving quality of life, and maintaining mobility for as long as possible. Physical therapy, muscle relaxant medications, and assistive devices are the main tools used today. Research into this condition is ongoing, and genetic diagnosis is increasingly important for families.

Also known as:

Key symptoms:

Progressive leg stiffness (spasticity)Leg muscle weaknessDifficulty walking or abnormal gaitIntellectual disability or learning difficultiesCataracts (cloudy lenses in the eyes)Muscle wasting (atrophy)Exaggerated reflexes in the legsBladder control problemsFoot deformities such as high arches (pes cavus)Delayed developmental milestones in childhoodSpeech difficulties in some individualsBalance and coordination problems

Clinical phenotype terms (29)— hover any for plain English
TetraplegiaHP:0002445Impaired continenceHP:0031064Loss of speechHP:0002371Primitive reflexHP:0002476
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 9B.

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No actively recruiting trials found for Autosomal recessive spastic paraplegia type 9B at this time.

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No specialists are currently listed for Autosomal recessive spastic paraplegia type 9B.

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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 9B.

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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.What specific mutation was found in the ALDH18A1 gene, and what does it mean for my (or my child's) prognosis?,Should other family members be tested, and what are the chances of siblings being affected?,What physical therapy program do you recommend, and how often should we attend?,Are there any clinical trials or research studies we could participate in?,What signs of progression should prompt us to contact you urgently?,How should we manage spasticity, and what are the options if current medications stop working?,Are there any specialists — such as an ophthalmologist or urologist — we should see regularly alongside you?

Common questions about Autosomal recessive spastic paraplegia type 9B

What is Autosomal recessive spastic paraplegia type 9B?

Autosomal recessive spastic paraplegia type 9B (also called SPG9B or AR-SPG9) is a rare inherited neurological condition that belongs to a large group of disorders known as hereditary spastic paraplegias (HSPs). These conditions share a common feature: progressive stiffness and weakness in the legs caused by damage to the long nerve fibers that run from the brain down the spinal cord. In SPG9B specifically, mutations in the ALDH18A1 gene disrupt the normal function of an enzyme involved in making certain amino acids, which leads to nerve cell damage over time. People with SPG9B typically expe

How is Autosomal recessive spastic paraplegia type 9B inherited?

Autosomal recessive spastic paraplegia type 9B follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.