Dr. E.G. Dowsett MBChB, Dip
Honorary Consultant Microbiologist
Basildon and Thurrock Hospitals NHS Trust.
Originally a Presentation to the All
Party Group of MPs on ME/PPS on 31st January 2001.
This Lincolnshire Post-Polio Library Publication February 2001.
Few people would dispute that ME (Myalgic encephalomyelitis), an illness which blights the hopes and aspirations of all sufferers, especially the young, is denied equal treatment in respect of diagnostic facilities, medical coverage and welfare provision. Comparable chronic and unpredictably disabling neurological conditions, for example Multiple Sclerosis, which was formerly ascribed to "hysteria" and similarly neglected, now receive government recognition, facilities within the NHS, and more generous research funding - though the potential cost of effective treatment can still arouse bitter debate.
WHAT IS ME?[1,2,3]
It is a syndrome (a group of linked symptoms) initiated by one or more of a related group of enteroviruses which circulate annually in the community in summer and autumn in temperate climates, but all the year round in tropical areas.
b. MINOR ILLNESS:
The majority of encounters with these viruses are asymptomatic but some subjects, more commonly teenagers and adults, suffer a seemingly trivial minor illness, usually described as a non specific summer 'flu accompanied by gastrointestinal upset, sore throat and occasionally by generalised glandular enlargment.
c. SECONDARY PHASE:
The minor illness is self limiting in 90% of adults. However, some 5-10% of all age groups exposed, may progress to a more significant episode with severe headaches and vertigo, a stiff neck and back and generalised muscle pain, signifying that the central nervous system has now become involved with a possible progression to viral meningitis and encephalitis. Clinical recovery at this stage is normally possible, but does not preclude further effects of the illness in later years. It has to he remembered that ME is a life-long disability where relapse is always possible.
d. FINAL STAGE[1,2]:
After a variable interval, a multi-system syndrome may develop, involving permanent damage to skeletal or cardiac muscle and to other "end organs" such as the liver, pancreas, endocrine glands and lymphoid tissues, signifying the further development of a lengthy chronic, mainly neurological condition with evidence of metabolic dysfunction in the brain stem. Yet, stabilisation, albeit at a low level, can still be achieved by appropriate management and support. The death rate of 10% occurs almost entirely from end-organ damage within this group (mainly from cardiac or pancreatic failure). It has to be said that suicide in younger patients and in earlier stages of the disability is related to the current climate of disbelief, rejection of welfare support and loss of educational and employment prospects. It is an additional and potentially avoidable factor.
WHAT ARE THE LATE EFFECTS OF ME?
Most doctors with substantial experience of examining these patients would agree that the outlook for any individual is unpredictable. Case records need to be kept up to date for prolonged periods because patients who have remained clinically stable over 40 years or more and have worked normally for most of their lives are still subject to significant late effects. These include: overwhelming fatigue both physical and mental; cognitive disturbances; muscular and joint pain; muscular weakness and wasting; difficulty with breathing; episodes of hypothermia and low blood pressure; problems with swallowing and voice production as well as sudden attacks of breathlessness while sleeping. The similarities of these symptoms to those complained of by sufferers from the Post-polio syndrome, is striking and requires further explanation.
WHICH GROUP OF ME SUFFERERS ARE CHIEFLY AT RISK OF THE LATE EFFECTS?
The majority of ME patients contract their illness in the 3rd and 4th decade (50%) with secondary peak at puberty (18%). The incidence at the extremes of age (below 10 years and above 50 years) has, until recently, been low (about 10% in each group). Epidemiological surveys made between 1988 and 1998, in 2 Essex hospital clinics dedicated to ME, indicate that the percentage of patients over 50 years of age attending with new illness has risen from 6% in 1988 to 16% in 1995 and 18% in 1998. Some patients indeed, present with paralysis. Others have a vague past history of illness in childhood. Some years of "weakness" or "growing pains"; recovery, but always poor at sport; possibly a stable work record for 25 years or more, followed by a decline in walking ability; unusual fatigue after simple tasks; problems with climbing stairs, dressing and with short term memory. The current age range of these patients is from 40-92 years, so it is not easy to dismiss their symptoms as due solely to "ageing". Their social and medical problems are especially severe, as they share all the difficulties of access to remedial and support services complained of by other disabled people in the same age group. However, in the UK, there is almost nowhere to refer patients with suspected post-polio symptoms as the medical profession has largely forgotten or never experienced the many manifestations of that disease. Successful immunisation against only 3 polio viruses among some 69 enteroviruses currently in circulation is deemed to have solved all problems!
WHAT IS THE POST-POLIO SYNDROME?[5,6,7]
Poliomyelitis is an acute enteroviral infection with a wide range of clinical manifestations and multi-organ involvement (a fact which was frequently overlooked by physicians dealing with large numbers of dangerously paralysed patients, between 1940 and 1950). 95% of people who contract the infection remain symptom free or suffer only a trivial non-specific respiratory or gastrointestinal illness as in ME.
Some 5% of those contracting the minor illness develop muscle weakness or paralysis before more serious or fatal complications supervene. The diagnostic distinction between "paralytic" and "non-paralytic polio" was entirely arbitrary in the days of the big epidemics. In fact, the category of "non-paralytic polio" contained many patients with mild or temporary paralysis and with encephalitis, which occurs in patients reaching the later stages of this illness. Modern studies indicate that overt paralysis in these patients depends entirely on the percentage of spinal nerve cells destroyed. For damage to be visible as weakness or paralysis at least 50%-60% of the nerves controlling muscular action must be damaged or destroyed. Thus, patients with less damage who may only have had a minor illness, and some who were asymptornatic can still present many years later with a classic Post-polio syndrome.
Recent publication[6,8] of this information (originally derived from studies made in 1955) has resulted in a re-definition of the post-polio syndrome and will certainly include many patients currently seen in ME clinics.
SUGGESTED NEW CRITERIA FOR THE DIAGNOSIS AND ASSESSMENT OF THE POST-POLIO SYNDROME.
|a.||A history of remote paralytic or
non-paralytic polio, or findings on history, physical
examination or laboratory and other technical studies compatible with damage to
the central nervous system in earlier life.
|b.||A period of recovery.
|c.||A period of stable functioning for 10-50
|d.||New symptoms for which no other
explanation can be found. Many patients and research workers point out that the
assessment of sufferers will now have to become more holistic, that standard
electrical tests of muscle function (EMG) will have to be more widespread (and
repeated), and that manual muscle testing must refer to repetitive activity and
daily tasks rather than a single examination on the couch.
IS IT POSSIBLE THAT MANY PATIENTS DIAGNOSED AS HAVING ME ARE SUFFERERS FROM AN ILLNESS CLINICALLY IDENTICAL TO "NON-PARALYTIC" POLIO?[6,8]
Yes, undoubtedly! This is an important question with fundamental implications for further research into the diagnosis, treatment and prevention of both disabilities.
Modern research published currently in a dedicated supplement of the American Journal of Physical and Medical Rehabilitation by the Editor and 3 leading research teams[6,8], indicates that part of the current difficulty in obtaining a clear diagnosis of the post-polio syndrome lies in the error of dividing acute poliomyelitis into "paralytic", "non-paralytic", "abortive" and "sub clinical" categories. It has to be recognised that there is a wide range of nerve damage in every patient. The Post-polio syndrome may therefore include:
|a.||Patients whose nervous system damage was
not clinically obvious at the time of diagnosis.
|b.||Those who had minimal paralysis for a
short period and were misdiagnosed as non-paralytic polio.
|c.||Those patients suffering from infection
due to non polio enteroviruses with potential to cause nervous
system damage and the "Post polio" syndrome, equal to that of polio viruses
e.g. Coxsackie viruses A9, A7; Coxsackie B viruses 1-6; ECHO virus 9;
Enteroviruses 70, 71 - all of which have been implicated in outbreaks of ME or
epidemics clinically identical to paralytic poliomyelitis.
|d.||Patients with symptoms clinically
identical to the Post-polio syndrome whose nerve damage arises from some other
cause, for example, local muscle problems due to metabolic dysfunction, the
effects of persistent virus infection, immune reaction to fragments of viral
genetic material etc.
It is essential that patients with clinical symptoms suggestive of Post-polio syndrome should be referred to a Physician to exclude other nervous diseases (eg, Motor Neurone Disease), and especially those which are treatable.
IS IT NECESSARY TO DIFFERENTIATE BETWEEN THE LATE EFFECTS OF ME AND THE POST-POLIO SYNDROME?[8,9,10]
Not really, even if it were useful or practicable to do so at present, as the two conditions are clinically identical and similar in respect of neuroanatomical, neuroendocrine, neuropsychological electroencephalographic and other techniques, including brain imaging and molecular biology, as indicated by a remarkable series of research papers published by Bruno and colleagues over the past 20 years.
WHAT IS THE EVIDENCE THAT THE LATE EFFECTS OF ME AND THE POST-POLIO SYNDROME CAN BE CAUSED BY ENTEROVIRUSES OTHER THAN POLIO VIRUSES 1-3?
1948, the year in which polio viruses were first cultured, specimens from 2
children with clinical poliomyelitis, yielded a non-polio enterovirus,
(eponymously called Coxsackie after the neighbourhood in which they lived).
This finding opened a Pandora's box of some 70 previously undiscovered
enteroviruses of which 14 strains were later found to have neurogenic potential
equal to that of polio viruses.
the late 1940s, studies in the USA indicated that outbreaks of major or minor
enteroviral illness (eg. Paralytic or non-paralytic and non specific "summer
'flu") could be caused by varying proportions of virulent and non virulent
polio viruses combined with other neurogenic enteroviruses, for example in
Akron and Cincinnati [Table 1], Ohio (1947) Delaware and
the UK, an outbreak of poliomyelitis affecting an Edinburgh housing estate from
August 1961-February 1962 (a period when polio immunisation with the Salk
(injectable) vaccine had recently been introduced) provided evidence that a
"mosaic" of enteroviruses, including Polio type 3, Coxsackie viruses B2 and B4,
Echo viruses 5 and 15 could act in combination to enhance virulence in
individual patients, to block the spread of polio virus type 3 and to interfere
with vaccine efficiency. Each virus type appeared sequentially until the
arrival of Echo virus 5 in November which ended the outbreak by the following
February (as indicated by serial sampling of the local school sewer). It has to
be remembered that a sudden change in the virulence and spread of enteroviruses
in the 20th century has been due to alterations in human hygienic behaviour
rather than to viral mutations.
|TABLE 1. 1947 OUTBREAK OF
SUMMER 'FLU, CINCINNATI, USA 
|3 LABORATORY TESTS
FOR COXSACKI VIRUS
(NON POLIO ENTEROVIRUS)
|1||0||Polio HV||-||-||-||High virulence
polio virus only
polio virus &
infection of CSF
|3||3||"||+||+||+||Low virulence polio
virus & coxsackie
|4||150*||"||+||+||+||Infection of CSF &
low virulence polio
virus & Coxsackie
|5||734*||Polio HV||-||-||-||Only high virulence
polio virus &
infection of CSF
|6||27||Polio HV||-||-||-||Only high virulence
virus & infection
|8||107*||Polio HV||-||-||-||Only high virulence
polio virus and
infection of CSF
|KEY * Raised cell count in
cerebro-spinal fluid indicates infection in the central nervous system
Polio HV - Polio type one, virulent.
Polio LV - Possibly polio type two, low virulence (non paralytic)
Coxsackie is a neurovirulent, non polio enterovirus
HOW MAY SYMPTOMS OF THE LATE EFFECTS OF ME AND POLIO BE EXPLAINED?[9,10]
It has to be accepted that some degree of encephalitis has occurred in all these cases and that the areas chiefly affected include the upper spinal motor and sensory nerve roots and the spinal nerve networks traversing the adjacent brain stem (a nerve centre controlling all vital bodily functions which is always damaged). The most troublesome symptoms of both conditions are progressive muscle weakness, fatigue and pain, and the commonest cause of relapse over use of repaired nerve networks and an inappropriate response to physical or mental stress in combination with the increasing effect of normal aging.
This is almost always central and due to damage affecting the Reticular activating system (which keeps the brain awake and alert as well as maintaining some control over muscular activity). Fatigue is characteristically intermittent, but profound and incapacitating and related even to minor activity.
MUSCLE WEAKNESS AND WASTING:
This may have a central cause (as above) or a local origin due to loss of motor units controlling individual muscles (including the breakdown of repair to these over time). Metabolic, immune or ongoing viral injury to muscle fibres, are other possibilities where infection persists.
This is a severe symptom which is difficult to treat and is usually due to dysfunction of the thalamus, an important sensory relay station in the brain stem. Failure to produce natural painkillers (e.g. endorphins and encephalins), may be an additional factor.
INAPPROPRIATE REACTION TO PHYSICAL OR MENTAL STRESS:
This also arises from injury to the brain stem which normally controls the production of cortisol (a steroid required for stress control) via the hypothalamus, pituitary and adrenal glands . In the absence of an efficient response, even minor stress can cause catastrophic collapse in these patients. NB. Because of the many and varied symptoms arising from encephalitic damage to the brain, all symptoms reported, however bizarre they may seem, must be taken as possible evidence of organic disease.
Despite promising reports from the USA of anti-enteroviral agents, and of Dopamine receptor agonists (to correct some deficiencies in neurotransmission) no specific medical treatment is yet available in the UK and the main principles of management rely upon conservation of energy, reduction of stress, and simplification of manual tasks at home or at work. These objectives cannot possibly be achieved without financial and social support, aids to mobility, house conversions and suitable rehabilitation facilities. In the USA it is claimed that (with counselling, if necessary, for those who find such adjustments to life style difficult) 91% of patients will stabilise in view of the fact that, at this stage, the disability is only slowly progressive. Patients have to be cautious about drugs, especially those acting on the central nervous system including psycho-active preparations and alcohol. In general, these patients need less anaesthetic but higher doses of pain killers than usual and more time to convalesce from surgery. There are now many new options for muscle problems including modern orthoses and corrective surgery.
|a.||There has been little government
interest or support for patients suffering from the late effects of ME or from
the post-polio syndrome. It is generally expected that survivors of
polio will gradually disappear because of successful immunisation of the UK
population 40 years ago. However the fact that "Post-polio", by any other name,
can arise from currently circulating enteroviruses has not
been taken into account. The Chief Medical Officer's Working Party on ME (set
up in 1999 and funded privately by the Linbury Trust) has made it clear that
its remit is only with management, and that all discussion
about the cause, epidemiology and social benefit requirements of these patients
is ruled out. It seems that it will be difficult to advise on rational
management in the absence of such vital information.
|b.||The potential size and cost of
the problem. This is impossible to assess in the UK because no
official epidemiological surveys have been made. However,
increasing numbers of patient support groups and individual research workers
have been making their own calculations. In the case of ME, prevalence appears
to range from 300/100,000 to 500/100,000 in occupations at high risk of
infection, but no information is yet available
about the number likely to suffer late effects (except that it may have trebled
in the last 10 years).
The number likely to be affected by the post-polio syndrome has been calculated as between 200-270/100,000 currently, but no account has been taken of survivors from non-paralytic polio which could easily double that figure. Possible costing for ME support has been based on 3 times the cost of maintenance for multiple sclerosis on the supposition that ME is 3 times as common. The only costs that we can be sure of are those derived from the failure of appropriate management, and of inappropriate assessments which waste vast sums of money and medical time while allowing patients to deteriorate unnecessarily.
|c.||Some Immediate Steps that Could
Be Taken[7,17,18,19,20]. These
patients could be referred to NHS rehabilitation clinics and welfare facilities
as for any other chronic neurological disease but physiotherapy must include
exercise suitable for patients with some damaged muscle fibres which have been
overused while others are normal and liable to deconditioning. Separate "ME" and "Post-polio Clinics" are more
expensive and often inaccessible. We should be educating doctors and paramedics
now about the very common and seriously disabling effects of
neglect. Rapid diagnostic tests for
enteroviruses, anti-enteroviral drugs and possible vaccines are already in
preparation here, or in use (in the USA) to deal with the tremendous burden of
circulating enteroviral infections, (for example, leading to febrile
respiratory infections, viral meningitis and myocarditis, let alone unnecessary
admissions to hospital and inappropriate prescription of antibiotics in
methods could well be employed for the benefit of young people in the UK and to
prevent the rising tide of ME in schools - the commonest cause of long term
absence and subsequent educational deficit![19,20]
|d.||Research workers must be
encouraged and appropriately funded to work in this field. However
they should first be directed to papers published before 1988, the time at
which all specialised experience about poliomyelitis and associated infections
seem to have vanished mysteriously![11,12,13]
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© Copyright E.G. Dowsett 2001
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Created: 19th February 2001
Last Minor Modification: 21st February 2001.