Exploring the Link Between Motor Neuron Degeneration and Post-Polio Syndrome

The relationship between motor neuron degeneration and post-polio syndrome?

Dear Dr.
Jian,
I have the following questions and would appreciate your guidance:
At the age of three, I experienced atrophy and weakness in my left lower limb due to poliomyelitis and have never received rehabilitation or assistive devices.
In 2005, I suddenly lost the ability to walk due to an unknown reason affecting my right foot.
Following my doctor's recommendation, I underwent cervical spine surgery, and the report is as follows:
1.
Cervical stenosis at C3-C6 with cord compression was noted.
After laminectomy, the thecal sac was fully decompressed.

2.
Autologous bone graft from C4-6 spinous processes and miniplate.

Postoperatively, there was no improvement, and I was referred to a neurologist and participated in hydrotherapy rehabilitation.
I applied for major illness classification at the hospital, which included complications from poliomyelitis and cerebral palsy affecting the nervous system, muscles, bones, and lungs, with diagnosis code 04510 (ICD-9-CM: 045.10).

I have been hospitalized multiple times at National Taiwan University Hospital for diagnosis and rehabilitation, with discharge summaries indicating:
1.
Post-poliomyelitis syndrome with motor neuron degeneration.

2.
Dysphagia, suspected to be related to motor neuron degeneration.

3.
Involuntary leg movements during sleep, cause to be determined.

4.
Peripheral arterial occlusive disease in the left leg.

The Chinese diagnosis certificate states:
1.
Motor neuron degeneration
2.
Residual effects of poliomyelitis.

Now, nearly five years later, following my physician's instructions, my right leg has almost no support, and I am also experiencing increasing weakness in my left hand.
In recent years, I have frequently had difficulty swallowing, often choking on liquids or during swallowing.
Additionally, I have experienced episodes of losing my voice while speaking over the past six months.
The doctor has only mentioned some deterioration and increased medication dosage, but there seems to be no effective strategy.
Earlier, the doctor indicated that I am a less prominent case of MND, leaning more towards post-polio syndrome.
I am unclear about the connection between the two conditions—whether post-polio syndrome implies the presence of MND or ALS, or if there is no absolute relationship at all.
Is the aforementioned condition considered a rare disease?
On another note regarding medication, the prescriptions for DEPAKINE 500mg and NEUQUINON 10mg were previously covered by health insurance, but for the past six months, they have inexplicably become out-of-pocket expenses.
The doctor has also suggested increasing the dosage of Neuquion to 600-900mg, which adds another financial burden.
Thank you for your attention to these matters.
Best regards!

Y.Y. Lin, 60~69 year old female. Ask Date: 2010/04/04

Dr. Jian Yingxiu reply Rare Disease

MND or ALS should be discussed with a neurologist.
Currently, certain diseases can be diagnosed through genetic testing, so you may want to consult your neurologist about this.
However, your previous cervical spine issues may also contribute to these symptoms, so it is essential for your physician to consider your medical history and examination results comprehensively.

Reply Date: 2010/04/04

More Info

Post-Polio Syndrome (PPS) and motor neuron degeneration are two interconnected but distinct medical conditions that can arise following an initial poliovirus infection. Your history of polio at a young age, followed by the development of symptoms consistent with PPS and motor neuron degeneration, raises important questions about the relationship between these conditions.

Post-Polio Syndrome is a condition that affects polio survivors, typically occurring decades after the initial infection. It is characterized by new muscle weakness, fatigue, and pain, which can arise in muscles that were previously affected by polio as well as in muscles that were not. The underlying mechanism is believed to involve the degeneration of motor neurons that were previously damaged by the poliovirus, leading to a gradual decline in muscle function. This degeneration can manifest as weakness, muscle atrophy, and other neurological symptoms.

Motor neuron degeneration, on the other hand, refers to the progressive loss of motor neurons in the spinal cord and brain, which can lead to conditions such as Amyotrophic Lateral Sclerosis (ALS) or other forms of motor neuron disease (MND). While PPS is specifically related to the history of polio, motor neuron degeneration can occur independently or as a part of other neurodegenerative diseases.

Your symptoms, including significant weakness in your right leg, left hand weakness, dysphagia (difficulty swallowing), and involuntary leg movements during sleep, suggest a complex interplay between PPS and potential motor neuron degeneration. The fact that you have been diagnosed with "Postpolimyelitis syndrome with motor neuron degeneration" indicates that your healthcare providers recognize the possibility of both conditions contributing to your current clinical picture.

It is crucial to understand that while PPS can lead to motor neuron degeneration, not all individuals with PPS will develop significant motor neuron degeneration or MND. The relationship between the two is not entirely understood, and ongoing research is needed to clarify the mechanisms involved. Some studies suggest that the stress on the remaining motor neurons due to the increased demand on weakened muscles may lead to further degeneration, but this does not imply that all PPS patients will progress to MND or ALS.

As for the classification of your condition as a rare disease, it is important to note that while PPS itself is not classified as a rare disease, the combination of PPS with significant motor neuron degeneration may be less common. The rarity often depends on the specific symptoms and their severity, as well as the individual’s medical history.

Regarding your medication concerns, it is not uncommon for medications to shift from being covered by insurance to being out-of-pocket expenses. This can be frustrating, especially when the recommended dosages increase. It is advisable to discuss these changes with your healthcare provider, who may be able to provide alternatives or assist in navigating insurance coverage.

In conclusion, the relationship between Post-Polio Syndrome and motor neuron degeneration is complex and multifaceted. It is essential to maintain open communication with your healthcare team, including neurologists, to monitor your symptoms and adjust your treatment plan as necessary. Regular follow-ups and possibly engaging in physical therapy may help manage symptoms and improve your quality of life.

Similar Q&A

Understanding Nerve Disorders: Is Your Condition Related to ALS?

About ten years ago, my father underwent surgery on his right arm due to nerve compression, which caused atrophy and weakness in the entire arm. After the surgery, he did not experience further atrophy or weakness. Recently, however, he suddenly started to feel weakness in his ri...

Dr. Jiang Junyi reply Neurology
Dear Ms. Hong, The condition you are concerned about, commonly referred to as "Lou Gehrig's disease," is known as amyotrophic lateral sclerosis (ALS), which is a type of motor neuron disease. Generally, the motor neurons in the brain are referred to as upper motor...

Early Signs of ALS: Understanding Symptoms and Prevention Strategies

Hello, doctor. Recently, when I stand on my left foot, I feel a constant urge to engage my calf muscles (similar to the feeling of trying to maintain balance). I am aware of this urge before it happens. Additionally, when lying in bed, I have the urge to shake my leg, and I have ...

Dr. Jiang Junyi reply Neurology
Hello Mr. Ju, You mentioned "the early reactions of amyotrophic lateral sclerosis (ALS)?" ALS primarily refers to issues with motor neurons, which control muscle movement. Generally, the motor neurons in the brain are referred to as upper motor neurons, while those in ...

Understanding Muscle Weakness in ALS: Fatigue vs. Strength Loss

In amyotrophic lateral sclerosis (ALS), the muscle weakness experienced is primarily due to muscle strength reduction rather than just a feeling of weakness. This condition leads to the degeneration of motor neurons, resulting in actual loss of muscle strength and function.

Dr. Jiang Junyi reply Neurology
Hello: The condition you mentioned, commonly known as amyotrophic lateral sclerosis (ALS), primarily involves issues with motor neurons. Motor neurons control muscle movement; generally, the motor neurons in the brain are referred to as upper motor neurons, while those in the bra...

Improving Mobility in Post-Polio Patients: Rehabilitation Options

Hello, my father contracted polio as an infant, resulting in atrophy and weakness in one of his legs. He seemed to have worn a metal brace for a period when he was around thirty, and at that time, he was still able to walk with the support of one leg. He is now 53 years old and h...

Dr. Li Weiqiang reply Rehabilitation
Dear Angel, Hello, my father contracted polio during infancy, resulting in atrophy and weakness in one leg. It seems that around the age of thirty, he wore a leg brace for a period, during which he was still able to walk with the support of one leg. He is now 53 years old and ha...