NOTE: This Message was originally addressed to Tom Mckeever
      from Dempt@eskimo.com and was forwarded to you by Tom Mckeever
                           --------------------
Date:         Thu, 13 Apr 1995 13:15:53 -0800
From:         Tom Dempsey 
Subject:      PPS Pamphlet
To:           Multiple recipients of list POLIO 
The following is a pamphlet that was developed by the Washington
Easter Seals aimed at medical professionals.  You may want to give
this to your doctor.
I have also placed this on the Polio Survivors Page in HTML, Text,
Word for Windows 6.0, and WordPerfect 4.2 formats.  Since I use WFW
6.0, I'd appreciate it if someone would tell me how the Wordperfect
file works.
                                Tom
Tom Dempsey               |     dempt@eskimo.com
Seattle, Washington USA   |     http://www.eskimo.com/~dempt/
----
Polio Survivors Page  http://www.eskimo.com/~dempt/polio.html
-------------- Enclosure number 1 ----------------
AN APPROACH TO THE PATIENT WITH SUSPECTED
POST POLIO SYNDROME
Originally written by Dr. Warren Anderson and the Medical Advisory Board of 
the
 Post Polio Program Easter Seal Society of Oregon
MEDICAL ADVISORY BOARD of the POLIO OUTREACH ADVISORY COUNCIL A Working 
Advisor
 Council to the Easter Seal Society of Washington
Joshua Benditt, MD, Pulmonologist
Evan Cantini, MD, Physiatrist
Dianna Chamblin, MD, Physiatrist
Margarette Forgette, MD, Physiatrist
Bill Kelly, Physical Therapist
Dennis Lang, RN, MPH, Polio Survivor
Lawrence R. Robinson, MD, Physiatrist
Mark Sumi, MD, Neurologist
EASTER SEAL SOCIETY OF WASHINGTON
521 Second Avenue
Seattle, Washington 98119
March 1995
TABLE OF CONTENTS
I.  Introduction - 1
A.  Definition of Post Polio Syndrome - 1
B.  Scope of the Problem - 1
C.  Diagnostic Criteria - 1
II.  Pathology: Physiologic and Clinical Consequences - 2
A.  Extensive Neuronal Involvement in the Average Case - 2
B.  Motor Unit Remodeling - 2
C.  Decompensation Then Produces Post Polio Syndrome - 2
III.  Patient Presentation - 3
A.  Prime Symptoms - 3
B.  Additional Presenting Symptoms - 3
C.  Past History - 4
IV.  Evaluation Process - 5
A.  Identify Areas of Dysfunction - 5
B.  Formalize Treatment Goals - 5
C.  Prognosis - 7
V.  Resources in Patient Management - 8
A.  Neurology Consultation - 8
B.  Physiatry (Physical Medicine and Rehabilitation) - 8
C.  Physical Therapy - 8
D.  Occupational Therapy - 8
E.  Speech Pathology - 8
F.  Pulmonology - 8
G.  Psychology - 8
H.  Support Groups - 8
I.  Other - 9
VI.  Bibliography - 9
AN APPROACH TO THE PATIENT WITH SUSPECTED POST POLIO SYNDROME
Polio survivors are at risk for the occurrence of certain physiologic changes 
i
 the nervous system which result in a characteristic set of symptoms now 
nown
 as Post Polio Syndrome.  In addition to these unexpected physiological 
changes
 there are antici
pated complications such as arthritis, scoliosis, and entrapment syndromes 
that
 frequently accompany paralytic conditions.  These anticipated complications 
ar
 not the problems that distinguish PPS from other diseases of the nervous
 system.  Post Polio S
yndrome (PPS) is a major chronic illness and one which poses unique problems 
to
 its survivors and their physicians.
No Diagnostic test exists for PPS, so clinical criteria must be used to
 establish the diagnosis.  Many Physicians lack training in the diagnosis and
 management of a syndrome only recently acknowledged as existing.  Patients 
are
 often uncomfortable with p
hysicians they feel do not understand their problems. They also fear 
ncreased
 disability, often at the same time they are coping with limitations of 
ging.
 Patients are often trapped in a "conquer the disease" mentality derived from
 the experience of r
ecovering from the acute episode an average of 25 years earlier. This is
 incompatible with the lifestyle adjustments necessary for optimal results in
 PPS rehabilitation.
I.  INTRODUCTION
A.  DEFINITION OF POST POLIO SYNDROME
An otherwise unexplained constellation of symptoms which may include 
eakness,
 fatigue, pain, heat or cold intolerance, and swallowing, breathing, or sleep
 disturbance developing in a patient who had paralytic polio.  Post Polio 
Muscl
 Atrophy (PPMA) has
 been used as the label for the above symptoms when they include progressive
 muscle atrophy.
B.  SCOPE OF THE PROBLEM
1987 National Health Interview Survey estimated 1.63 million American polio
 survivors (=0.625% of population), 50% with some Post Polio Syndrome 
symptoms.
C.  DIAGNOSTIC CRITERIA
1.  PPS is a diagnosis of exclusion and should be based on a thorough history
 and physical exam.
2.  Evidence of prior paralytic polio: via EMG, an appropriate history, or
 characteristic residual atrophy.
3.  Period of apparent stability before any new symptoms.  New symptoms may
 often be seen after an illness or injury.
4.  Exclusion of other conditions (especially motor neuron diseases and 
overuse
 syndromes).
II.  PATHOLOGY:  PHYSIOLOGIC AND CLINICAL CONSEQUENCES
A.  EXTENSIVE NEURONAL INVOLVEMENT IN THE ACUTE POLIO INFECTION
1.  The extent of central nervous system infection by polio virus is not well
 appreciated.  Infection is far more widespread than anterior horn cells 
alone.
 Often anterior horn cell infection is largely subclinical due to residual
 capacity of uninfected
and surviving neurons.  Infection outside the anterior horns is likely to be
 largely subclinical also, but may help to explain the disabling symptoms of
 fatigue and pain which are subjective and controversial (because the
 physiologic basis is uncertain).
2.  Ninety-five percent (95%) of motor neurons are infected in an average 
acute
 infection, with a 50% neuronal fatality rate.
3.  There is frequent segmental involvement, accounting for the lack of 
symmetr
 of weakness.
4.  In addition to the anterior horns in the spinal cord, infection involves
 intermediolateral horns and dorsal root ganglia.
5.  Infection also involves motor cortex, hypothalamus, and globus pallidus,
 brainstem nuclei, reticular formation, cerebellar roof nuclei, and vermis.
B.  MOTOR UNIT REMODELING IN THE POST RECOVERY PHASE
1.  A normal quadriceps has, on average, 200 muscle fibers/anterior horn cell
 and a normal anterior horn cell can adopt as many as 1,000 orphaned muscle
 fibers.
2.  Over 50 % of motor units may be lost without symptoms. (Normal walking 
uses
 only 15-20% of maximum muscle strength.)
3.  Clinical improvement occurs acutely through recovery of mildly affected
 neurons, collateral sprouting, and strengthening (hypertrophy) of intact
 musculature.
4.  Increased demand on surviving motor units results in increased firing
 frequency which in turn produces a change in fiber type to predominantly
 aerobic "slow twitch" fibers with increased vascularity.
C.  DECOMPENSATION THEN PRODUCES POST POLIO SYNDROME
While a single underlying etiology for PPS has not been proven, several 
theorie
 exist:
1.  There is an increased metabolic burden on surviving anterior horn cells
 (even in asymptomatic muscles) as they direct more muscle fibers to 
ontract,
 more often, to achieve the same force of contraction.  This leads to 
nterior
 horn cell fatigue and
can lead to premature metabolic injury, perhaps even cell loss.  Fatigued
 neurons may be unable to continue to trophically support as many muscle 
fibers
  The collateral sprouts to some muscle fibers will degenerate.  The strength 
o
 these muscle fibers
will be lost to the motor unit, and a spiral of decline may set in.  This 
ode
 of decompensation augured by fatigue, may be anterior horn cell based.  This
 appears not to be a static process and there may be dynamic denervation and
 reinervation.
2.  Another mode of decompensation is muscle fiber based:  Rapidly firing 
muscl
 fibers produce more lactic acid which may not be adequately dissipated.  
his
 is especially true with any degree of isometric contraction. Muscle fiber
 fatigue may, lead to
muscle fiber injury, lost function, and a spiral of decline.
3.  Any increase in mechanical load (such as would result from increased 
weight
 or increased physical activity) or decrease in force generating capacity 
(such
 as would result from inactivity following illness or injury) may trigger
 metabolic failure in m
otor units or in muscle fibers functioning close to their capacity.
4.  The resulting relative weakness may lead to joint and muscle misuse and
 overuse. This may lead in turn to both arthritis and overuse syndromes.
5.  In addition to anterior horn cell and muscle fiber modes of fatigue, 
centra
 fatigue may also be a factor. Polio virus infection of the motor strip and 
the
 reticular activating system is well described.  A working definition for
 central fatigue is:
"Increased mental effort necessary to perform a fixed amount of muscle
 contraction".  This is very much how Post Polio Syndrome patients describe
 their feelings of fatigue, many report hitting a "post polio wall".
III.  PATIENT PRESENTATION
A.  PRIME SYMPTOMS
A common presentation is a polio survivor who previously had lower extremity
 involvement in a well defined polio episode.  The patient may have 
estricted
 ambulation from hiking or jogging, lived a sedentary life, and did not feel
 disabled.  After a peri
od of relatively stability he or she may begin to notice unusual fatigue and
 discomfort and may further restrict activity.  Denial of decreased 
unctional
 capacity may lead to a crisis as the patient can no longer can meet
 occupational, social, and famil
y commitments.  Persistence and attempts to continue at a previous activity
 level may lead to a downward spiral of decreasing functional capacity with
 resulting depression and despair.  On examination, relative obesity may be
 present and weakness is easi
ly demonstrated, often in the "good" leg; limbs considered unaffected are 
often
 subclinically affected with polio and may present with "new" polio.  A
 statistical summary of the clinical characteristics of several series of PPS
 patients is as follows:
1.  Fatigue, Pain, and Weakness are almost always present. Fatigue (89%); 
ain
 in Muscle or Joint (86%); New weakness (83%) in previously symptomatic (69%) 
o
 asymptomatic (50%) muscles.
2.  New Atrophy (28%);  This equates to Post Polio Muscular Atrophy (PPMA).
3.  Activities of daily living difficulties (78%) = functional loss. Walking
 (64%); Climbing Stairs (61%); Dressing (17%).
B.  ADDITIONAL PRESENTING PROBLEMS
1.  Pulmonary dysfunction:
Patients with Post Polio Syndrome may suffer from weakness of the breathing
 muscles, namely the diaphragm and ribcage.  Occasionally, this can be severe
 enough to cause symptoms of dyspnea on exertion and even at rest, poor
 clearance of respiratory secre
tions increasing the risk of pneumonia, and elevations in the resting 
rterial
 CO2 level.  Measurement of pulmonary function tests in these patients 
sually
 shows a significant restrictive pattern (small lung volumes) on the basis on
 neuromuscular weakne
ss.
If respiratory muscle weakness is severe enough mechanical ventilation may be
 required.  Small mechanical ventilators have been developed which deliver
 breaths through a comfortable plastic nose mask.  This is often performed 
whil
 the patient is asleep
at night and results in improved daytime function.
2.  Sleep Disorders:
Patients with Post Polio Syndrome have a high incidence of sleep disturbances
 with poor sleep quality and frequent awakenings which may be due to several
 factors.  However, the most important etiology to rule out is central,
 obstructive and mixed sleep a
pneas.  Nocturnal hypoxemia and hypercarbia can lead to worsening of daytime
 function of the breathing muscles.  Nocturnal non invasive ventilation can 
e
 used in these patients to improve sleep quality and reduce symptoms of 
daytime
 sleepiness, and perh
aps improve daytime respiratory muscle function.
3.  Dysphagia:
Many PPS patients reported some new difficulty with eating or swallowing more
 commonly in those with bulbar polio. Video fluoroscopy has been used for
 evaluation and has frequently revealed pharyngeal constrictor weakness.
 Laryngeal penetration and loss
of the cough reflex may occur without symptoms, suggesting an underestimation 
o
 the presence and severity of dysphagia in this population.  Many patients 
have
 already employed compensation such as altering diet, cutting solids into 
small
 pieces, chewing
 it thoroughly, taking small sips of liquids, eating slowly, and using 
postural
 maneuvers.  Most patients with dysphagia had also experienced some 
progressive
 speech difficulty such as increased hoarseness, weakness, or slurring.
4.  Cold intolerance (29%):
Limbs may be cold and cold exposure produces weakness.  This is thought to be
 due to intermediolateral column involvement resulting is vasoparesis, venous
 pooling, and excessive heat loss.
5.  Degenerative arthritis:
A joint that is biomechanically disadvantaged may develop degenerative
 arthritis.
6.  Social and psychological problems:
Long term disability and denial may result in social and psychological 
problems
C.  PAST HISTORY
1.  Average age of polio onset is 7 years.  Median time to maximum recovery 
is 
 years.  Median period of stable neurologic and functional status is 25 
ears.
 Median post polio symptom duration before patient presents for evaluation is 
5
 years.
2.  Variables associated with shorter interval to PPS: greater severity and
 greater age.
3.  Initial symptoms are most frequent in the lower limb most affected in the
 acute illness. (Upper extremity weakness is easier to compensate for without
 overuse resulting.)
4.  The onset is usually insidious but is frequently precipitated by injury,
 illness, bed rest, or weight gain.
IV.  EVALUATION PROCESS
A.  IDENTIFY AREAS OF DYSFUNCTION
1.  The history is especially useful in identifying fatigue, dysphagia, sleep
 disorders, and alteration in activities of daily living.
2.  The Neurologic exam will identify atrophy or weakness and verify that
 reflexes are not increased.  Pay special attention to the "good" limb as
 significant weakness may be present of which the patient has never been 
aware.
 With leg muscles, functiona
l tests must be used because manual testing may not detect quadriceps 
eakened
 to 30% of normal even though this is sufficient strength for routine daily
 activities.  Seek a mechanical advantage in manual muscle testing:  Test the
 triceps or quadriceps w
ith the elbow or knee flexed more than 90 degrees.  Test the psoas in the 
supin
 position.
3.  The general physical exam and biomechanical exam note obesity, joint
 deformity, overuse syndromes, and scoliosis.
4.  Electromyography may be requested when needed to document previous 
anterior
 horn cell disease (especially when the previous history of polio is in 
doubt).
 EMG can also be used to rule out other neuromuscular pathologies or to 
identif
 subclinically
involved muscles.
5.  CK elevation may be seen in patients but may not correlate with 
progressive
 weakness.
B.  FORMALIZE TREATMENT GOALS
After the diagnosis of PPS is established, a patient conference is a 
convenient
 way to formalize treatment goals and begin patient education.  These areas
 should be addressed:
1.  Lifestyle Modifications:
This item is the "sine qua non" of all attempts at successful management of 
PPS
  At the time of formal diagnosis, patients are often desperate, yet imbued 
wit
 a belief in their own ability to overcome their disability through the "no
 pain, no gain" app
roach.  This approach may have served them very well after their acute attack 
o
 polio many years ago but is now actually self destructive.  Persistence in 
thi
 approach of "overcoming" illness has led to a spiral of deteriorating 
function
 and frequently
 a p
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