Ask the Expert

UCLA Neurologist and PPS expert Dr. Susan Perlman answers your questions.

Dr. Perlman,
Can you tell us what the likelihood is for patients to develop breathing problems as a result of their PPS? What are the contributing factors and how can assistive devices help?

– Mauricio Minotta

Ask the Expert

January 2010

May 2010

Dear Mauricio,
The same rules apply to post polio-related breathing symptoms as to other post polio symptoms.
No. 1: One-third of new breathing symptoms will be due to post polio (true new weakening of the muscles of respiration).
One-third will represent worsening of prior polio breathing residua due to non-neurologic complications (recurrent infection, smoking or environmental exposure).
One-third will be a cause unrelated to post polio (other lung disease).

No. 2: The likelihood of a polio survivor developing PPS is about 60%. A survivor of bulbar polio or someone with a history of significant involvement of muscles of respiration acutely will also have a greater than 50% risk of developing post polio symptoms affecting breathing (or swallowing). Someone without an acute history of muscle weakness in limbs or breathing (non-paralytic polio) may have a 14% risk of developing post polio weakness in those areas.

No. 3: Factors that may increase the risk of developing post polio breathing problems are severity of original breathing issues and amount of recovery (i.e. greater number of remodeled motor units); presence of scoliosis, gastroesophageal reflux, or sleep apnea; and presence of other lung issues (smoking or environmental lung disease, asthma, recurrent infections). Assistive devices can be life saving and energy conserving—e.g. BIPAP for sleep apnea, intermittent positive pressure ventilation for chronic hypoventilation, supplemental oxygen for chronic low oxygen levels. Use of these should be discussed with a knowledgeable pulmonologist.


Dear Dr. Perlman,
I am a caregiver for a bulbar polio survivor. He is having increased problems with breathing. I was just reading about diaphragm pacers. No one in this area understands PPS and I am struggling. I am afraid I am going to lose him due to this ignorance. Would a diaphragm pacer be helpful for him? How do I pursue this?

– Anne Marie

Dear Anne Marie,
Diaphragmatic pacing (either direct or via the phrenic nerve) is primarily used for individuals who have lost central brain control of diaphragm function. For those with peripheral weakness (as in polio), there may not be enough nerve-muscle function left to stimulate or the nerve-muscle complex could be overworked and fatigued by the stimulation or a weakness of the throat muscles could be made worse by increasing diaphragm pull. Less invasive means, such as nasal bi-level positive airway pressure or intermittent positive pressure ventilation, might be more appropriate choices after a careful evaluation by a skilled pulmonologist.


Dear Dr. Perlman,
I have heard of patients with multiple sclerosis using a device called Bioness to assist in walking. Is this a viable product for people suffering from PPS? If so, is there any research available? If not, what other devices or aids may be used for those suffering from this condition?

Thanks
– Kim Wiley

Dear Kim,
Bioness only works to improve foot drop if the peripheral motor nerve and muscle (foot dorsiflexor) are intact. In polio survivors with foot drop, these nerves and muscles are usually atrophied and unlikely to respond to the Bioness stimulator. If a free trial is offered by the vendor, it might be interesting to try out.


Dear Dr. Perlman,
I became ill with polio at age 12 in 1959. I was diagnosed with myasthenia gravis several years ago as well as central & obstructive sleep apnea. Is there research that shows connection for all those maladies?

– John Bryan

Dear John,
Both post polio and myasthenia can increase your risk of obstructive sleep apnea. Post polio can also increase your risk of central apneas.

Recent research suggests an immune system component in post polio syndrome (chronic low grade inflammation, triggered by polio virus debris). Myasthenia gravis is known to be caused by the immune system making antibodies to the nerve-muscle connection, possibly triggered by the thymus gland. So both post polio and myasthenia are immuno-neurologic disorders and may respond to similar treatments (IVIG, pyridostigmine).

As a polio survivor, you have a 60% risk of developing post polio symptoms. The prevalence of myasthenia gravis is quite rare, about five in 100,000 people. There is no direct relationship that would make polio survivors more likely to develop myasthenia.


Dear Dr. Perlman,
What are the similarities between Parkinson’s and post-polio syndrome? And how can the doctor make the distinction when the patient had bulbar polio as a child?

– Frances Powell

Dear Frances,
The only similarity between these two disorders is that both can cause trouble walking or weakness in using muscles. However, in Parkinson’s, those problems come from the central nervous system, while in post polio they come from the peripheral motor system. The neurologic examination would look very different. A neurologist could easily pick out the changes related to Parkinson’s and the changes related to post polio, even in someone suffering from both conditions.


Dear Dr. Perlman,
I was born in 1949 and became sick during the winter of 1953. I think I had polio, although the doctor was not certain. That winter my tongue became stiff, and so did my legs, although not completely. Breathing was also difficult.

Every 10 years since my youth, I have had a kind of breakdown, with hyperventilation and extreme loss of energy and strength in muscles etc. In between, I am very often getting very tired, so my question is: Do you think I have a light case of post polio syndrome?

Thanks,

– Niels Windfeld Lund
Norway

Dear Niels,
You could certainly be a polio survivor who is experiencing post polio symptoms. The best way to confirm this would be to have a thorough neurologic examination and an electromyogram, to look for the signs of post polio and to rule out other neuromuscular conditions.