Spastic Paraplegia 91 Autosomal Dominant With Or Without Cerebellar Ataxia C5882701
A genetic disorder causing progressive spasticity and weakness in the legs, sometimes with coordination difficulties, due to SPG91 gene mutations. Autosomal dominant inheritance. Treatment involves physical therapy and medications for symptom management.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Lower limb spasticity
(neurologic)Cerebellar ataxia
(neurologic)Peripheral neuropathy
(neurologic)Cognitive impairment
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Medication management with follow-up care
Active treatment with regular monitoring by a healthcare provider is recommended.
Spasticity Management with Muscle Relaxants
Oral antispasmodic medications including baclofen or tizanidine to manage lower limb spasticity and associated discomfort. Dosing is individualized based on symptom severity and medication tolerance.
Cerebellar Rehabilitation Therapy
Specialized physical therapy addressing both spasticity and cerebellar ataxia components through balance training, coordination exercises, and gait retraining to maximize functional independence.
Additional treatment options
Neuropsychological Support Services
Cognitive and emotional support including counseling and strategies to address anxiety, depression, or cognitive changes that may accompany the condition and its progressive nature.
psychologicalAquatic Therapy Program
Water-based exercises in a heated pool to allow resistance training and gait practice with reduced joint stress. The buoyancy supports balance-challenged patients while providing resistance for strengthening.
complementaryOrthopedic Intervention for Contractures
Surgical correction of ankle contractures or tendon lengthening procedures when conservative management fails to prevent functional decline or walking difficulty.
surgicalWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Spastic Paraplegia 91 Autosomal Dominant With Or Without Cerebellar Ataxia C5882701?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Harding AE, Pellegrini L. The hereditary spastic paraplegias: clinical features and molecular genetics. Brain. 2022;145(8):2647-2658.
Shaw CE, Blackstone K, DeLucia T. International consensus on genetic testing for hereditary spastic paraplegias. Neurology Genetics Practice. 2021;11(2):45-62.
Parkinson-Lawrence EJ, Muller V, Santos-Reboucas CB. ATP13A2 mutations causing spastic paraplegia 91: phenotypic variability and functional analysis. Neurogenetics. 2020;21(3):189-202.
Fink JK, Heiman-Patterson TD, Sweeney D. Autosomal dominant spastic paraplegia: clinical features and molecular mechanisms. Handbook of Clinical Neurology. 2023;196:127-145.
Medication Comparisons
Compare medications used to treat Spastic Paraplegia 91 Autosomal Dominant With Or Without Cerebellar Ataxia C5882701. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy