Post-Polio Syndrome

Original Editor - Peter Thwaites Top Contributors - Natalie Patterson, Reem Ramadan, Nupur Smit Shah, Admin, Peter Thwaites, David Olukayode, Kim Jackson and Tony Lowe

What is Post Polio Syndrome?

Post Polio Syndrome (PPS) is a poorly understood condition that can affect people who have previously had polio. Polio is a viral infection that used to be common in the UK, Polio cases have decreased by over 99% since 1988, from an estimated 350 000 cases to 175 reported cases in 2019. 

Most people who had Polio would have fought off the infection without even realising they were infected. However, some people with polio would have had paralysis, muscle weakness and shrinking of the muscles. But usually, these problems would have resolved over the following weeks or months, or remained the same for years afterwards.

​PPS is where some of these symptoms return or get worse many years or decades after the original polio infection.It affects between 25% and 40% of poliomyelitis survivors and mimics motor neuron disorders (MNDs), such as amyotrophic lateral sclerosis (ALS).

Epidemiology

Typically, new symptoms appear 35 years after the first polio episode; the age at which they first appear might range from 8 to 71 years.Patients with a more serious initial polio illness are more likely to develop PPS.

Pathogenesis

It is uncertain what causes PPS's progressive neurologic decline. The primary theories of pathogenesis include reinnervated motor units' progressive degeneration, their increased metabolic demand leading to neuromuscular junction dysfunction, the persistence of the poliovirus in neural tissue, and induction of autoimmunity, which results in the destruction of neural structures.

Clinical features

Fatigue

Fatigue is the most common symptom of post-polio syndrome and it occurs in about 80% of patients.It can take many forms, including:

• muscle fatigue – where your muscles feel very tired and heavy, particularly after physical activity
• general fatigue – where you feel an overwhelming sense of physical exhaustion, as if you've not slept for days
• mental fatigue – where you find it increasingly difficult to concentrate, have problems remembering things and make mistakes you wouldn't usually make

Organising your activities so that you don't over-exert yourself and taking regular rests will help reduce your fatigue.

Muscle weakness

Increasing muscle weakness is another common symptom of post-polio syndrome.

Muscle weakness is where you are finding it difficult to use the affected muscles, whether you feel tired or not. Weakness can occur in the muscles that were previously affected by the polio infection. It can also affect those that weren't previously substantially affected. There may also be associated shrinking of affected muscles, known as atrophy. Increasing muscle weakness is another common symptom of post-polio syndrome. It can be easy to confuse muscle weakness with muscle fatigue, but they are different.

Muscle and joint pain

Muscle and joint pain are also common in post-polio syndrome. Muscle pain is usually felt deep like an ache in the muscles or muscle cramps and spasms. The pain is often worse in the evenings and is similar to arthritis and consists of soreness, stiffness and a reduced range of movement.Muscle and joint pain are also common in post-polio syndrome. Muscle pain is usually felt as a deep ache in the muscles or muscle cramps and spasms.

The pain is often worse after you've used the affected muscles. It can be particularly troublesome during the evening after a day's activities.

Associated symptoms

As well as the common symptoms of post-polio syndrome, a number of associated symptoms can also develop from the combination of fatigue, muscle weakness, and muscle and joint pain.

Weight gain

Because of the above symptoms, most people with post-polio syndrome become less physically active than they used to be. This can often lead to weight gain and in some cases, obesity. Because of the symptoms mentioned above, most people with post-polio syndrome become less physically active than they used to be. This in turn can make any fatigue, muscle weakness and pain worse.

Mobility difficulties

As well as weight gain, the combination of fatigue, weakness and pain can also make walking difficult, leading to increasing mobility problems. Often people with post-polio syndrome will need a walking aid, such as crutches or a stick at some stage, and some people may eventually need to use a wheelchair. As well as weight gain, the combination of fatigue, weakness and pain can also make walking difficult, leading to increasing mobility problems.

Breathing difficulties

Some people with post-polio syndrome find breathing can be difficult because the breathing muscles become weaker. This can cause problems such as respiratory insufficiency, shortness of breath during sleep, sleep apnea, which has a prevalence of 65%. There is also an increased risk of chest infections due to the muscle weakness.

If you have post-polio syndrome, it's important to get medical advice as soon as possible if you experience symptoms of a possible chest infection, such as coughing up discoloured phlegm or blood, chest pain and wheezing.

Swallowing problems

Weakness in the muscles you use for chewing and swallowing (pharyngeal and laryngeal muscles) may lead to problems swallowing (dysphagia), such as coughing, choking or gagging when swallowing. Voice and speech changes may occur, such as hoarseness, low volume or a nasal-sounding voice, particularly after you've been speaking for a while or when you're tired. This is usually due to facial weakness and vocal cord paralysis. A speech and language therapist may be able to help.

Sensitivity to cold

Some people with post-polio syndrome find they become very sensitive to cold temperatures or a sudden drop in temperature as a result of poor blood supply.Because of this intolerance to cold, people with post-polio syndrome may need to wear extra layers of clothing to try to stay comfortable.

Diagnosis of Post-polio syndrome

It is difficult to diagnose post-polio syndrome as there are no specific tests for it and symptoms can be mistaken for other conditions. However, diagnostic criteria for PPS exist and they include:

• Having had poliomyelitis in the past and still having motor neuron loss.
• At least 15 years of stable neurologic and functional conditions following the acute start of polio.
• A new weakness and aberrant muscle fatigability that appear gradually (or very rarely abruptly) and last for at least a year.
• Excluding other illnesses with symptoms that are comparable.

As the symptoms of post-polio syndrome can be similar to those of several other conditions, such as arthritis, some tests such as X-rays may be needed to rule out any other possible causes of your problems.

To rule out other conditions, or confirm whether you have post-polio syndrome, the following tests may be carried out:

• Electromyography (EMG) tests – to measure the electrical activity in your muscles and nerves and find out whether they're damaged.
• Sleep studies – if you're having problems sleeping, such as sleep apnea, or you're feeling unusually tired.
• Heart rate and function tests.
• A magnetic resonance imaging (MRI) scan or computerised tomography (CT).
• Lung function tests .
• Tests to investigate swallowing problems (dysphagia).

It's possible to have post-polio syndrome alongside other conditions, so not every health problem or symptom you experience may be related to the condition.

Differential diagnosis

• Amyotrophic lateral sclerosis
• Polymyalgia rheumatica
• Fibromyalgia
• Hypothyroid myopathy
• Myasthenia gravis
• Chronic inflammatory demyelinating polyneuropathy
• Cervical or lumbar spinal spondylosis with radiculopathies

Peter Thwaites: Living with Post-Polio Syndrome

When we left hospital after contracting Polio, (know as Infantile Paralysis many years ago), regardless of the disabilities bequeathed us by the Polio Virus, the last thing told us by our attending Physician, now burnt into our memory was "You must use it, or lose it".

How wrong was this statement, and since that fateful day we have been unwittingly destroying, or further damaging, already seriously damaged motor neurons, to such a degree that a large number of Polio Survivors are now experiencing one of the most devastating debilitating neurodegenerative condition in the world.

Non-motor symptoms of PPS have considerable quality of life implications and are notoriously challenging to manage, and yet very few Medical Professionals have even a slight knowledge of Polio, and even less of PPS.

Every person who was caught by the Polio virus had damage to their motor neurons. Many Polio victims were never diagnosed as their symptoms could have been only 'flu like' and would have gone unnoticed.

Our parents were told that our recovery was stable and once our muscle strength and physical abilities returned, they would remain stable for the rest of our lives, in fact we developed unexpected and disabling symptoms about 35 years after the acute polio virus attack.

Post-Polio Syndrome or Post-Polio Sequelae symptoms:

• Fatigue
• Muscle Weakness
• Muscle Pain
• Joint Pain
• Cold Intolerance
• Sleep Disorders
• Difficulty Swallowing
• Breathing Issues

At the time of the initial attack parents of Polio Survivors were told that they would die, stop breathing, or never walk again, but in the vast majority of cases this was not the case.

For some strange reason, we were told that:

• We would never go to college
• We would never get a job, and
• We would never get married.

To be accepted, we became 'Type A' personalities, with the feeling of loneliness, and have a lower sense of adequacy, worth, and value.

We were told to 'get normal', and so:

• We discarded assistive devices
• We have more years of education
• We work more overtime hours and take fewer sick days
• We married at a higher rate
• We are more sensitive to criticism and failure

Fatigue is the number 1 reported PPS, brain fatigue associated with:

• Reported difficulty with concentration, memory, attention, word finding and staying awake
• Abnormal attention, word finding and slowed thinking speed on neuropsychologic tests
• Brain wave slowing
• Other PPS "brain" symptoms evident: Abnormal breathing, and Twitching in sleep.