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ICD Hub / Diseases of the nervous system (G00–G99) / 8A00.10 - Progressive supranuclear palsy
ICD-11

ICD-11 Codes
for 8A00.10 - Progressive supranuclear palsy
Teodor Jurukovski January 1, 1970
Fact checked by: Teodor Jurukovski
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Understanding Progressive Supranuclear Palsy (PSP)

Progressive supranuclear palsy (PSP) is a rare, late-onset neurodegenerative disorder that affects specific areas of the brain responsible for controlling movement, balance, eye movements, and cognitive functions. It is classified as a type of atypical parkinsonism, meaning it shares some symptoms with Parkinson's disease but has distinct characteristics and a typically more rapid progression. The condition is progressive, with symptoms worsening over time due to the gradual deterioration and death of nerve cells.

Alternative Names for Progressive Supranuclear Palsy

Progressive supranuclear palsy is also known by several other terms and abbreviations, which are important for accurate medical documentation and patient understanding. These include:
  • PSP
  • Steele-Richardson-Olszewski syndrome
  • Atypical parkinsonism
  • Parkinson-plus disorder
Understanding these synonyms is crucial for comprehensive medical coding and communication.

Key Symptoms of Progressive Supranuclear Palsy

The presentation of PSP can vary, but common symptoms typically emerge in mid- to late-adulthood and include:
  • Balance Problems and Falls: Frequent, often unexplained falls, particularly backward, are a hallmark symptom.
  • Eye Movement Difficulties: Trouble looking up or down, slow eye movements, and difficulty controlling eyelids.
  • Motor Impairments: Stiffness (rigidity), slow movement (bradykinesia), and problems with coordination.
  • Speech and Swallowing Issues: Slurred speech, quiet voice, and difficulty swallowing (dysphagia).
  • Cognitive and Behavioral Changes: Mood swings, apathy, irritability, changes in judgment, difficulty with problem-solving, and sometimes dementia.
These symptoms often progress over several years, leading to significant disability.

Causes and Risk Factors for PSP

The exact cause of progressive supranuclear palsy remains unknown. However, research indicates that it involves the abnormal accumulation and clumping of a protein called tau within nerve cells in specific brain regions. This tau pathology is thought to disrupt normal brain function and lead to cell death. While the condition is usually sporadic, meaning it occurs randomly, age is the primary risk factor, with most diagnoses occurring in individuals over 60. It is slightly more common in males.

Diagnosing Progressive Supranuclear Palsy

Diagnosing PSP can be challenging, especially in the early stages, as symptoms often overlap with other neurological conditions like Parkinson's disease. There is no single definitive test. Diagnosis typically relies on a thorough medical history, a comprehensive neurological examination focusing on motor skills, eye movements, speech, and swallowing. Imaging techniques such as brain MRI may be used to rule out other conditions and can sometimes show characteristic changes, such as shrinkage in the midbrain. Specialists in movement disorders are often involved in the diagnostic process.

Managing Progressive Supranuclear Palsy Symptoms

Currently, there is no cure for PSP, nor are there treatments that can halt its progression. Management focuses on alleviating symptoms and improving the patient's quality of life. This may include:
  • Medications: Some medications used for Parkinson's disease may offer temporary relief for stiffness and slowness, though responses are often limited. Antidepressants may help with mood symptoms.
  • Therapies: Physical therapy can help with mobility and balance, occupational therapy with daily tasks, and speech therapy with communication and swallowing difficulties.
  • Eye Care: Special glasses or eye drops may address vision and eye movement issues.
  • Nutritional Support: For severe swallowing problems, a feeding tube (PEG) may be necessary.
  • Palliative Care: Provides comprehensive support for symptom relief and quality of life.
Participation in clinical trials may also be an option for some individuals.

Frequently asked questions

What is the ICD-11 classification for Progressive supranuclear palsy (code 8A00.10)?
Progressive supranuclear palsy (8A00.10) is classified under Parkinsonism, specifically as an atypical Parkinsonism.
How is Progressive supranuclear palsy (8A00.10) described in ICD-11?
Progressive supranuclear palsy (8A00.10) is described as a neurodegenerative disease characterized by supranuclear gaze palsy, postural instability, progressive rigidity, and mild dementia. It includes classical and four atypical variants.
What are the synonyms for Progressive supranuclear palsy (8A00.10) recognized in ICD-11?
Synonyms for Progressive supranuclear palsy (8A00.10) include Steele-Richardson-Olszewski syndrome, Atypical progressive supranuclear palsy, PSP-Parkinsonism, and Classical progressive supranuclear palsy.
Can additional details be added to the ICD-11 code 8A00.10 for Progressive supranuclear palsy?
Yes, post-coordination is applicable to code 8A00.10 to add detail, such as manifestations like dementia due to diseases classified elsewhere.
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