_files/transparent.gif) |
|
| Post-Polio Syndrome (PPS) |
| Disease
Name |
Post-Polio Syndrome
(PPS) |
| SearchTerm
|
Post-polio muscular
atrophy (PPMA), Post-polio muscle dysfunction, Post-polio
sequelae, Postpoliomyelitis syndrome |
| Inheritance Type |
sporadic |
| Protein |
disease probably
unrelated to a missing protein |
| Clinical
Onset |
at least 15 years after
poliomyelitis |
| Clinical
Findings |
Post-polio syndrome
(PPS) is characterized by a further weakening of muscles that
were previously injured by polio infection or new weakness in
unaffected muscles. Symptoms include fatigue, slowly
progressive muscle weakness, muscle and joint pain, and
muscular atrophy. The term post-polio muscular atrophy refers
to new muscle weakness or atrophy, which is reported by over
half of the post-polio population. Other less frequently
reported symptoms of PPS include respiratory insufficiency
from progressive muscular weakness, dysarthria (speech
disturbances), dysphagia (difficulty swallowing), cold
intolerance, muscular twitches, and new or progressive joint
deformities.
Helpful links:
Medical
Dictionary.
Resource
Guide on Disability.
|
| Diagnostic
Workup |
Diagnostic criteria for
the neuromuscular symptoms of PPS, referred to as post-polio
muscle dysfunction include history of paralytic polio; new
muscle dysfunction after a stable period following infection
of 15 years; neurological exam compatible with prior
poliomyelitis; muscle weakness and atrophy compatible with a
lower motor neuron lesion; and no sensory loss. However,
limited sensory loss due to nerve compression does not exclude
the diagnosis.
Laboratory signs: There is no
diagnostic test for PPS. It is diagnosed by excluding all
other possible causes for new symptoms, including abnormal
breathing and muscle twitching that commonly disturb polio
survivors' sleep, a slow thyroid and anemia. Other
neurological or muscle diseases are almost never the cause of
PPS symptoms. Neurophysiological findings are compatible with
prior poliomyelitis. EMG is of some use, any may show signs of
reinnervation but no polyneuropathy. Magnetic resonance
imaging and muscle biopsies or spinal fluid analysis may add
information that supports a diagnosis, but they are not
required.
Differential Diagnosis: Rule out amyotrophic
lateral sclerosis (ALS). Many symptoms of PPS overlap with
those of other diseases such as osteoarthritis, fibromyalgia,
hypothyroidism, depression, rheumatoid arthritis, polymyalgia
rheumatica, and a number of neurologic conditions. PPS may be
difficult to diagnose in some cases because it is hard to
determine what component of a neuromuscular deficit is old and
what is new. Signs of generalized neuropathy exclude the
diagnosis.
See Diagnostic Criteria for Neuromuscular
Diseases http://www.enmc.org/nmd/diagnostic.html.
|
| Treatment
Notes |
Scientists are
conducting studies on the drugs pyridostigmine and selegiline
to reduce fatigue and improve strength in patients with
post-polio syndrome. A NINDS multicenter, controlled trial on
insulin-like growth factor (IGF-1) is also underway to see if
it can enhance the ability of motor neurons to sprout new
branches, maintain existing branches, and rejuvenate synapses.
Progression: PPS is a slowly progressive denervation
of muscle marked by long periods of stability. Overuse may
exacerbate rate of progression. Risk factors for developing
these late effects of polio were severity of paralysis and
hospitalization during the acute infection. There does not
appear to be a genetic contribution. PPS is usually not life
threatening, except in patients with severe respiratory
impairment.
|
| Abstract |
Post-polio syndrome
(PPS) is characterized by a history of poliomyelitis and
partial recovery of function, followed by development of
progressive weakness in new or previously affected muscles for
which there is no other definite cause. PPS can manifest as
general flu-like exhaustion and as muscle fatigability
(perceived by patients as a lack of endurance). PPS is caused
by the death of individual nerve terminals in the motor units
that remain after the initial attack of polio. This
deterioration of individual nerve terminals might be an
outcome of the recovery process from the acute polio attack.
During this recovery process, in an effort to compensate for
the loss of nerve cells (neurons), surviving motor neurons
sprout new endings to restore function to muscles. This
results in large motor units that may add stress to the body.
As a result of this rejuvenation, the individual may have
normal-functioning muscles for some time.
After a
number of years, the motor neurons with excessive sprouting
may not be able to maintain the metabolic demands of all the
new sprouts, and a slow deterioration of the individual
terminals may result. Restoration of nerve function may occur
in some fibers a second time, but eventually nerve terminals
are destroyed and permanent weakness occurs. This hypothesis
is consistent with the slow, stepwise, unpredictable course of
PPS. | |
|
| |
_files/transparent20.gif){kind=small}
NIDRR Rehabilitation Research
and Training Center in Neuromuscular Diseases (RRTC/NMD)_files/rehabTitle.gif)
_files/blueCurveLeft.gif)
_files/home.gif){kind=small}
_files/siteIndex.gif){kind=small}
_files/help.gif){kind=small}
_files/accessibility.gif){kind=small}
_files/star.gif){kind=small}