Professional Documents
Culture Documents
Clinical Review: Poliomyelitis and The Postpolio Syndrome
Clinical Review: Poliomyelitis and The Postpolio Syndrome
Clinical Review: Poliomyelitis and The Postpolio Syndrome
Lane-Fox Unit and In the first half of the 20th century, poliomyelitis was
Department of
Neurology, St
widely feared. It often struck without warning, was Summary points
Thomas’ Hospital, highly contagious, and affected large, young popula-
Guy’s and St tions, causing prolonged or permanent flaccid
Thomas’ NHS Despite the success of the World Health
Foundation Trust, paralysis or death. There are arresting and disturbing
Organization’s policy for global eradication of
London SE1 7EH accounts of the explosive nature of polio epidemics
poliomyelitis, poliomyelitis is still endemic in
Robin S Howard and the response of communities to these outbreaks.1
consultant neurologist some countries and vaccine associated
The effective control of poliomyelitis throughout most
robin.howard@
poliomyelitis continues to occur
of the world has been a remarkable story of scientific
gstt.sthames.nhs.uk
and social progress. However, “wild” poliomyelitis is The eradication policy involves intensive mass
BMJ 2005;330:1314–9
still endemic in parts of sub-Saharan Africa and the immunisation, but concerns about vaccine
Indian subcontinent, and it continues to occur sporadi- associated poliomyelitis have led to increased use
cally elsewhere. In addition, there is a small incidence of inactivated polio vaccine, which is injected
of vaccine induced polio in infants and adults. Global rather than given orally, in immunisation
eradication remains a goal of the World Health strategies
Organization and of public health policies throughout
the world, with the eventual discontinuation of routine Other causes of acute flaccid paralysis, such as
immunisation.2–4 Western Nile encephalitis, may mimic acute
Acute poliomyelitis is now rarely encountered in poliomyelitis
the United Kingdom, but “imported” poliomyelitis still
occurs and it is necessary to distinguish acute poliomy- Postpolio syndrome is characterised by fatigue,
elitis from other causes of acute flaccid paralysis. weakness, joint and muscle pain, and worsening
Despite the obvious success of preventive policies, functional abilities
many patients who had poliomyelitis experience late
functional deterioration after periods of prolonged Development of postpolio syndrome does not
stability—the so called postpolio syndrome. The inevitably imply progressive deterioration, but
patterns of disability and their management present management requires a multidisciplinary
unique challenges to the multidisciplinary rehabilita- approach with provision of appropriate exercise
tion team. regimens and suitable medical or orthotic
support
Methods
This review is based on personal experience and the local immune response or a viraemic phase occurs.
advice of numerous physicians and surgeons, in The virus continues to be excreted in the saliva for two
particular the combined expertise of my colleagues at or three days and in the faeces for two to three weeks.
the Lane Fox Unit at St Thomas’ Hospital. In prepar- The infection rate is extremely high, but it is probable
ing the article, I accessed archive literature, depart- that 95% of all infections are either asymptomatic or
mental review papers,5 6 the library, Medline searches characterised by an abortive flu-like illness. In the tem-
via Ovid, and websites of patient groups involved in perate climates of the northern hemisphere, epidemics
the care of people with polio and postpolio functional of polio occurred most commonly during the summer
deterioration. months, and the incidence was greatest where children
bathed together.
The disease Acute illness
Poliomyelitis is caused by an enterovirus of high infec- After the minor, flu-like illness, patients develop a
tivity whose main route of infection is via the human meningitic phase as the virus reaches the central nerv-
gastrointestinal tract. There are three subtypes of ous system. This is characterised by high fever with
poliovirus, but, before the introduction of vaccine, type pharyngitis, myalgia, anorexia, nausea, vomiting, head-
1 accounted for 85% of paralytic disease. Infection is
oral, and the virus multiplies in the pharynx and intes- Extra references to relevant articles are on bmj.com
tine for one to three weeks before it is contained by a
ache, and neck stiffness. The factors favouring the Immunisation and eradication
development of spinal poliomyelitis are unclear, but it
Prevention
has been suggested that physical activity and intramus-
The demonstration, in 1949, that poliovirus could be
cular injections during the minor illness may be
successfully propagated in non-neuronal cell cultures
important. The onset of spinal poliomyelitis is
led to the award of the Nobel Prize to Enders, Robbins,
associated with myalgia and severe muscle spasms,
and Weller. This was the catalyst for the development
with the subsequent development of an asymmetrical,
of effective immunisation against poliomyelitis, initially
predominantly lower limb, flaccid weakness that
by Jonas Salk and subsequently by Albert Sabin. The
becomes maximal after 48 hours.
introduction of the Salk trivalent inactivated polio vac-
A purely bulbar form with minimal limb involve-
cine (IPV) in 1956 for routine immunisation reduced
ment also occurs, particularly in children, and was
the incidence of polio in the United States by more
more common in those whose tonsils and adenoids than 90%. It is given by injection and stimulates serum
had been removed. This form of polio has a IgM, IgG, and IgA, but not secretory IgA, immunity
particularly high mortality because of vasomotor being induced by antibody transuding into the
disturbances such as hypertension, hypotension and oropharynx.
circulatory collapse, autonomic dysfunction, dys- Sabin trivalent oral live attenuated polio vaccine
phagia, dysphonia, and respiratory failure. Poliovirus (OPV) replaced the Salk vaccine in 1962. This is com-
may also cause an acute encephalitis. posed of live attenuated strains of polioviruses 1, 2, and
3 grown in cell culture. The advantages over the Salk
Diagnosis
The cerebrospinal fluid shows increased protein Box 1: Causes of acute flaccid paralysis
content and pleocytosis with normal glucose concen- In countries where polio is currently, or was recently,
tration, but the virus is more commonly isolated from endemic every case of acute flaccid paralysis should be
the nasopharynx or stools. In the absence of a viral iso- notified regardless of the likely cause. Two stool
late, serological diagnosis can be established by specimens should be collected within 14 days of the
start of paralysis, and virus isolation should be
neutralisation of sera against paired antigens of the performed in a qualified laboratory.
three viral serotypes, which also allows differentiation
of wild type from vaccine induced disease. Molecular Infection
diagnosis with the polymerase chain reaction is now • Viral
Enterovirus—poliomyelitis (wild and vaccine
the technique of choice for identifying poliovirus sero-
associated), enterovirus 71, coxsackievirus A7
type and for differentiating between wild and vaccine Flavivirus—Japanese encephalitis, West Nile virus
strain poliomyelitis.7 Herpesvirus—cytomegalovirus, Epstein-Barr virus,
Differential diagnosis—Acute flaccid paralysis is asso- varicella-zoster virus
ciated with infection by other enteroviruses (including Tick borne encephalitis
coxsackievirus A7 and enterovirus 71), tickborne HIV related—associated with opportunistic
infections
encephalitis, and by flaviviruses (including Japanese
Other neurotropic viruses—rabies
encephalitis and, more recently, West Nile virus).8 The
• Borrelia
differential diagnosis of asymmetric motor flaccid
• Mycoplasma
paralysis includes Guillain-Barré syndrome,9 acute
• Diphtheria
intermittent porphyria, HIV neuropathy, diphtheria,
• Botulism
and Borrelia bergdorferi infections (Lyme disease), and
disorders at the neuromuscular junction such as Neuropathy
myasthenia and botulism (see box 1).10 • Acute inflammatory polyneuropathy
• Acute motor axonal neuropathy
• Critical illness neuropathy
• Lead poisoning
Treatment of acute attack
• Other heavy metal poisoning
All patients should be put on strict bed rest to prevent
Spinal cord
extension of the paralysis. Pain relief and frequent
• Acute transverse myelitis
passive movements prevent contractures and joint
• Acute spinal cord compression
ankylosis. Acute respiratory failure may develop
• Trauma
rapidly, requiring intubation and intermittent positive
• Infarction
pressure ventilation. If prolonged ventilation is
needed or there is coexisting bulbar weakness, trache- Neuromuscular junction
ostomy may be necessary to protect the airway. • Myasthenia gravis
During the first few weeks of mobilisation, the
Muscle
main aim of management is to prevent deformity by
• Polymyositis
stretching and splinting of affected limbs. Intensive
• Viral myositis
physiotherapy is directed towards retraining affected
• Post-infectious myositis
muscles to regain strength and function and providing
• Critical illness myopathy
appropriate orthoses to compensate for loss of
function, facilitate mobility, and prevent undue wear Functional
and tear.
School of Social Alice is 58 years old and is married with two sons, both she was 12 years old. At that time, she had spent a
Sciences, Cardiff
University, Cardiff
in their 20s. She works for several charities, is a magis- month in hospital and then, after several years of
CF10 3WT trate, and is out most days and evenings. She fits in a taxing physiotherapy and exercises, she recovered
Ruth Bridgens gentle game of tennis every week, but cannot walk completely, except for slight atrophy in one leg.
PhD candidate, more than a mile, even on the level. This was not always As she continued becoming weaker, she would
medical sociology
the case. A few years ago, she was jogging at least three suddenly find herself overwhelmed by exhaustion,
bridgensr@cardiff.ac.uk
miles every day and playing squash several times a which was relieved if she lay down for 15 minutes. She
week. Gradually, she began to notice that jogging remembered an article about something called
uphill became difficult. She tried to find more level postpolio syndrome, which she had made a mental
routes. Climbing stairs became difficult, and her legs note of at the time but had thought nothing
and back began to ache after exercise. This carried on moreabout. Her general practitioner referred her to a
for a couple of years. She realised that the aching was neurologist, who said, “Nothing wrong with you.” She
in three areas—her left arm, right leg, and back, the mentioned postpolio syndrome, and he replied,
three places that had been most affected by polio when “There’s no such thing as postpolio syndrome.”