X-linked parkinsonism-spasticity syndrome

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ORPHA:363654OMIM:300911G20
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Overview

X-linked parkinsonism-spasticity syndrome is an extremely rare genetic neurological condition that primarily affects males. It is caused by changes (mutations) in a gene located on the X chromosome, which means it follows an X-linked pattern of inheritance. The condition is characterized by a combination of parkinsonism — which includes tremor, slowness of movement, stiffness, and balance problems similar to Parkinson's disease — along with spasticity, which is an abnormal tightness or stiffness in the muscles, particularly in the legs. These symptoms together can significantly affect a person's ability to walk and perform daily activities. The disease typically begins in adulthood, and symptoms tend to worsen gradually over time. Affected individuals may first notice difficulty with movement, stiffness in their limbs, or tremor. Because the condition is X-linked, it mainly affects males, while females who carry one copy of the altered gene are usually unaffected or may have very mild symptoms. There is currently no cure for X-linked parkinsonism-spasticity syndrome. Treatment is focused on managing symptoms. Medications used for Parkinson's disease, such as levodopa, may be tried to help with parkinsonian symptoms, though the response can vary. Physical therapy and rehabilitation are important parts of care to help maintain mobility and reduce the impact of spasticity. Anti-spasticity medications such as baclofen or tizanidine may also be used. Research into this condition is ongoing, and management is best coordinated by a team of specialists familiar with movement disorders.

Also known as:

Key symptoms:

Tremor in the hands or limbsSlowness of movement (bradykinesia)Muscle stiffness or rigiditySpasticity (tight, stiff muscles especially in the legs)Difficulty walking or unsteady gaitBalance problemsDifficulty with fine motor tasks like writing or buttoning clothesMuscle weaknessIncreased muscle reflexesProgressive worsening of movement over timeDifficulty with speech or swallowing in advanced stages

Clinical phenotype terms (14)— hover any for plain English
Resting tremorHP:0002322Cogwheel rigidityHP:0002396Hyperactive deep tendon reflexesHP:0006801Diffuse cerebral atrophyHP:0002506Lateral ventricle dilatationHP:0006956Dilated third ventricleHP:0007082Ankle clonusHP:0011448Scissor gaitHP:0012407
Inheritance

X-linked recessive

Carried on the X chromosome; typically affects males more than females

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for X-linked parkinsonism-spasticity syndrome.

View clinical trials →

No actively recruiting trials found for X-linked parkinsonism-spasticity syndrome at this time.

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Search ClinicalTrials.gov ↗Join the X-linked parkinsonism-spasticity syndrome community →

No specialists are currently listed for X-linked parkinsonism-spasticity syndrome.

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 X-linked parkinsonism-spasticity syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.How certain is the diagnosis, and should genetic testing be done to confirm it?,Would levodopa or other Parkinson's medications be helpful in my case?,What physical therapy program would be most beneficial for me?,How quickly is this condition likely to progress in my case?,Should my family members be tested for this genetic change?,Are there any clinical trials or research studies I could participate in?,What should I do if I start having trouble swallowing or speaking?

Common questions about X-linked parkinsonism-spasticity syndrome

What is X-linked parkinsonism-spasticity syndrome?

X-linked parkinsonism-spasticity syndrome is an extremely rare genetic neurological condition that primarily affects males. It is caused by changes (mutations) in a gene located on the X chromosome, which means it follows an X-linked pattern of inheritance. The condition is characterized by a combination of parkinsonism — which includes tremor, slowness of movement, stiffness, and balance problems similar to Parkinson's disease — along with spasticity, which is an abnormal tightness or stiffness in the muscles, particularly in the legs. These symptoms together can significantly affect a person

How is X-linked parkinsonism-spasticity syndrome inherited?

X-linked parkinsonism-spasticity syndrome follows a x-linked recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does X-linked parkinsonism-spasticity syndrome typically begin?

Typical onset of X-linked parkinsonism-spasticity syndrome is adult. Age of onset can vary across affected individuals.