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Postpoliomyelitis Syndrome

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Description: Post-poliomyelitis syndrome is a condition that affects polio survivors years after recovery, characterized by new muscle weakness, fatigue, and pain.
Type: Post-poliomyelitis syndrome is not a genetic disorder. Instead, it is a condition that affects polio survivors years after recovery from the initial poliovirus infection. It is characterized by muscle weakness, fatigue, and pain. Since it's not caused by genetic factors, there is no type of genetic transmission associated with it.
Signs And Symptoms: After a period of prolonged stability, individuals who had been infected and recovered from polio begin to experience new signs and symptoms, characterised by muscular atrophy (decreased muscle mass), weakness, pain, and fatigue in limbs that were originally affected or in limbs that did not seem to have been affected at the time of the initial polio illness. PPS is a very slowly progressing condition marked by periods of stability followed by new declines in the ability to carry out usual daily activities. Most patients become aware of their decreased capacity to carry out daily routines due to significant changes in mobility and decreasing upper limb function and lung capability. Fatigue is often the most disabling symptom; even slight exertion often produces disabling fatigue and can also intensify other symptoms. Problems breathing or swallowing, sleep-related breathing disorders, such as sleep apnea, and decreased tolerance for cold temperatures are other notable symptoms.Increased activity during healthy years between the original infection and onset of PPS can amplify the symptoms. Thus, contracting polio at a young age can result in particularly disabling PPS symptoms.A possible early occurring and long-lasting sign is a slight jitter exhibited in handwriting.
Prognosis: In general, PPS is not life-threatening. The major exception is patients left with severe residual respiratory difficulties, who may experience new severe respiratory impairment. Compared to control populations, PPS patients lack any elevation of antibodies against the poliovirus, and because no poliovirus is excreted in the feces, it is not considered a recurrence of the original polio. Further, no evidence has shown that the poliovirus can cause a persistent infection in humans. PPS has been confused with amyotrophic lateral sclerosis (ALS), which progressively weakens muscles. PPS patients do not have an elevated risk of ALS.No sufficient longitudinal studies have been conducted on the prognosis of PPS, but speculations have been made by several physicians based on experience. Fatigue and mobility usually return to normal over a long period of time. The prognosis also differs depending upon different causes and factors affecting the individual. An overall mortality rate of 25% exists due to possible respiratory paralysis of persons with PPS; otherwise, it is usually not lethal.Prognosis can be abruptly changed for the worse by the use of anesthesia, such as during surgery.
Onset: Post-polio syndrome typically manifests 15 to 40 years after the initial polio infection.
Prevalence: The prevalence of post-poliomyelitis syndrome (PPS) varies, but it is estimated to affect 25-50% of individuals who have previously recovered from polio. This condition typically occurs 15-40 years after the initial infection.
Epidemiology: Old data show PPS occurs in roughly 25 to 50% of people who survive a polio infection. However, newer data from countries that have contacted their polio survivors have shown 85% of their polio survivors to have symptoms of post polio syndrome. Typically, it occurs 30–35 years afterwards, but delays between 8 and 71 years have been recorded. The disease occurs sooner in persons with more severe initial infections. Other factors that increase the risk of PPS include increasing length of time since acute poliovirus infection, presence of permanent residual impairment after recovery from the acute illness, and being female. PPS is documented to occur in cases of nonparalytic polio (NPP). One review states late-onset weakness and fatigue occur in 14–42% of NPP patients.
Intractability: Post-poliomyelitis syndrome (PPS) is generally considered intractable, meaning there is no cure. The symptoms, which include new muscle weakness, fatigue, and pain, can be managed but not eliminated. Treatment focuses on symptom relief, improving quality of life, and preventing further complications.
Disease Severity: Postpoliomyelitis syndrome can vary greatly in severity. It typically involves a gradual worsening of symptoms such as muscle weakness, fatigue, and pain years after the initial polio infection. The progression is usually slow, and while it can significantly affect the quality of life, it is not typically life-threatening. Severity depends on the extent of the initial polio infection and the degree of recovery from it.
Healthcare Professionals: Disease Ontology ID - DOID:4952
Pathophysiology: ### Pathophysiology of Post-Poliomyelitis Syndrome (PPS)

Post-Poliomyelitis Syndrome (PPS) occurs in individuals who have previously had poliomyelitis, a viral infection caused by the poliovirus. The pathophysiology of PPS is not completely understood, but it is thought to involve several key mechanisms:

1. **Degeneration of Motor Neurons**:
- During the acute poliovirus infection, motor neurons in the spinal cord and brainstem are damaged or destroyed.
- To compensate, surviving motor neurons sprout new nerve endings to reinnervate muscle fibers, leading to enlarged motor units.
- Over time, these enlarged motor units can become overworked and begin to deteriorate, leading to muscle weakness and atrophy.

2. **Metabolic Stress**:
- The increased metabolic demand on the remaining healthy motor neurons can lead to metabolic stress and subsequent motor neuron dysfunction or death.

3. **Inflammation**:
- Chronic inflammatory changes might also play a role, contributing to ongoing neuromuscular deterioration.

4. **Muscle Overuse and Disuse**:
- Muscle overuse and disuse are believed to exacerbate symptoms. Overuse can damage muscle fibers and joints, while disuse can lead to atrophy and functional decline.

5. **Axonal Transport Defects**:
- Some theories suggest defects in axonal transport mechanisms within the motor neurons contribute to their dysfunction.

Understanding of PPS continues to evolve, but these mechanisms collectively contribute to the progressive weakness, fatigue, and pain characteristic of the syndrome.
Carrier Status: Post-poliomyelitis syndrome (PPS) is not associated with carrier status because it is a condition that affects polio survivors years after recovery from an initial acute poliovirus infection. PPS involves symptoms such as muscle weakness, fatigue, and pain. It is not associated with a genetic carrier state or infectious carrier status.
Mechanism: Numerous theories have been proposed to explain post-polio syndrome. Despite this, no absolutely defined causes of PPS are known. The most widely accepted theory of the mechanism behind the disorder is "neural fatigue". A motor unit is a nerve cell (or neuron) and the muscle fibers it activates. Poliovirus attacks specific neurons in the brainstem and the anterior horn cells of the spinal cord, generally resulting in the death of a substantial fraction of the motor neurons controlling skeletal muscles. In an effort to compensate for the loss of these neurons, surviving motor neurons sprout new nerve terminals to the orphaned muscle fibers. The result is some recovery of movement and the development of enlarged motor units.The neural fatigue theory proposes that the enlargement of the motor neuron fibers places added metabolic stress on the nerve cell body to nourish the additional fibers. After years of use, this stress may be more than the neuron can handle, leading to the gradual deterioration of the sprouted fibers, and eventually, the neuron itself. This causes muscle weakness and paralysis. Restoration of nerve function may occur in some fibers a second time, but eventually, nerve terminals malfunction and permanent weakness occurs. When these neurons no longer carry on sprouting, fatigue occurs due to the increasing metabolic demand of the nervous system. The normal aging process also may play a role. Denervation and reinnervation are going on, but the reinnervation process has an upper limit where the reinnervation cannot compensate for the ongoing denervation, and loss of motor units takes place. What disturbs the denervation-reinnervation equilibrium and causes peripheral denervation, though, is still unclear. With age, most people experience a decrease in the number of spinal motor neurons. Because polio survivors have already lost a considerable number of motor neurons, further age-related loss of neurons may contribute substantially to new muscle weakness. The overuse and underuse of muscles also may contribute to muscle weakness.Another theory is that people who have recovered from polio lose remaining healthy neurons at a faster rate than normal. However, little evidence exists to support this idea. Finally, the initial polio infection is thought to cause an autoimmune reaction, in which the body's immune system attacks normal cells as if they were foreign substances. Again, compared to neural fatigue, the evidence supporting this theory is quite limited.
Treatment: Postpoliomyelitis syndrome (PPS) is a condition that affects polio survivors years after recovery. Treatment options focus on managing symptoms and improving quality of life. These may include:

1. **Physical Therapy:** Helps maintain muscle strength and function.
2. **Occupational Therapy:** Aids in adapting to physical limitations and conserving energy.
3. **Medications:** To manage pain and reduce fatigue, such as pain relievers and anti-inflammatory drugs.
4. **Assistive Devices:** Braces, canes, or wheelchairs can improve mobility.
5. **Lifestyle Changes:** Regular but moderate exercise, a healthy diet, and adequate rest can help manage symptoms.
6. **Speech Therapy:** For those with difficulties in speech or swallowing.
Compassionate Use Treatment: Post-poliomyelitis syndrome (PPS) is a condition that affects polio survivors years after recovery. There are limited treatment options, and research into effective therapies is ongoing. Here are some details regarding compassionate use, off-label, and experimental treatments for PPS:

1. **Compassionate Use Treatment**:
- Compassionate use, also known as expanded access, may provide patients access to investigational drugs or therapies not yet approved by regulatory authorities. Patients with PPS generally need to work with their healthcare provider to explore this option and may require approval from the relevant regulatory body, such as the FDA in the United States.

2. **Off-label Treatments**:
- Some medications approved for other conditions may be used off-label to manage PPS symptoms. Examples include:
- Pain management medications like gabapentin or pregabalin for neuropathic pain.
- Antidepressants such as amitriptyline, which can also help manage pain and improve sleep quality.
- Intravenous immunoglobulin (IVIG) has been used in some cases to address inflammation and immune-related issues.

3. **Experimental Treatments**:
- Clinical trials are ongoing to investigate new treatments for PPS. Some avenues explored include:
- Stem cell therapy to promote nerve and muscle regeneration.
- Treatments targeting muscle metabolism and mitochondrial function to enhance muscle endurance and strength.
- Various rehabilitative therapies focusing on improving physical function and reducing fatigue.

Patients interested in these treatments should consult with their healthcare provider to understand potential benefits and risks and to discuss participation in clinical trials if available.
Lifestyle Recommendations: For post-poliomyelitis syndrome (PPS), here are some lifestyle recommendations:

1. **Physical Activity**: Engage in a balanced exercise program that includes gentle, low-impact activities like swimming or walking to maintain mobility without causing fatigue. Work with a physical therapist to create a tailored plan.

2. **Rest and Energy Conservation**: Balance activity with adequate rest. Practice energy conservation techniques, such as pacing activities, taking frequent breaks, and prioritizing tasks.

3. **Healthy Diet**: Maintain a nutritious diet to support overall health. Include plenty of fruits, vegetables, whole grains, and lean proteins.

4. **Weight Management**: Keep a healthy weight to reduce stress on muscles and joints.

5. **Assistive Devices**: Use assistive devices like braces, wheelchairs, or canes as needed to improve mobility and reduce fatigue.

6. **Pain Management**: Implement strategies for managing pain, which can include physical therapy, heat/cold therapy, and medications as recommended by a healthcare provider.

7. **Emotional Support**: Seek support for mental and emotional well-being through counseling, support groups, or other resources.

8. **Regular Medical Check-ups**: Schedule regular appointments with a healthcare provider who is knowledgeable about PPS to monitor progression and adjust care plans accordingly.

Consult a healthcare professional to develop a personalized plan that addresses individual needs and limitations.
Medication: There's no specific medication to cure post-poliomyelitis syndrome (PPS). Management typically focuses on alleviating symptoms. Medications may include pain relievers like acetaminophen or ibuprofen for muscle pain, or prescription medications for more severe pain. Antidepressants or sleep aids might be prescribed to address fatigue or sleep disturbances. In each case, treatment should be tailored to an individual’s specific symptoms and needs by a healthcare provider.
Repurposable Drugs: Currently, there are no specific drugs explicitly approved for repurposing to treat post-poliomyelitis syndrome (PPS). Treatment mainly focuses on managing symptoms and improving quality of life through physical therapy, pain management, and sometimes using medications for pain or fatigue as deemed appropriate by healthcare providers. If you have further inquiries or need guidance, consulting with a medical professional is recommended.
Metabolites: Post-poliomyelitis syndrome (PPS) is not primarily characterized by specific metabolites currently recognized for diagnostic or therapeutic purposes. The syndrome is a condition that affects polio survivors years after recovery, manifesting as new muscle weakness, fatigue, and pain. As of now, there are no well-defined metabolic biomarkers specifically associated with PPS. Management focuses on symptomatic treatment, physical therapy, and lifestyle modifications. Further research is needed to identify any potential metabolic profiles unique to PPS.
Nutraceuticals: Currently, there are no specific nutraceuticals that have been proven to treat or alleviate the symptoms of post-poliomyelitis syndrome (PPS). Management typically focuses on supportive care, such as physical therapy, occupational therapy, and lifestyle modifications to conserve energy. It's essential for patients to consult healthcare providers before starting any new supplement regimen.
Peptides: In post-poliomyelitis syndrome (PPS), certain peptides may be researched for therapeutic purposes, although there is limited evidence on specific peptides used to treat PPS. The focus is generally on symptom management through physical therapy, adaptive devices, and medications to alleviate pain and fatigue. Research on peptide-based treatments is ongoing, but they are not yet a standard part of PPS management.