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The Committee for Justice
and Recognition of Myalgic Encephalomyelitis
The polio like illness Myalgic Encephalomyelitis is introduced. Evaluated in context with discussion of some early epidemics.
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THE LANCET LEADING ARTICLES
A New Clinical Entity ?
In 1917 von Economo 1
reported a small outbreak of an illness in which the main features were fever,
stupor, and ophthalmoplegia: 2 of his 13 patients died and at necropsy there
was evidence of inflammation of the brain substance. During the next two years
a great many similar outbreaks were recorded and by 1921 the disease had
reached epidemic proportions in almost every country in Europe.2 In
spite of perplexing variations in the clinical picture from case to case,
locality to locality, and even from season to season,2 it soon
became clear for practical purposes a new clinical entity had appeared.3 In 1924, 5039 cases of encephalitis
lethargica were notified in England and Wales alone,4 but by the beginning
of the next decade confirmed cases of this dangerous disease had become
sporadic and by 1939 they were extremely rare.5 By the end of the late war, the centre of
interest had shifted to poliomyelitis as by far the most prevalent and
disabling infection of the nervous system. The work of Ritchie Russell and others 6 focused attention on
the importance of diagnosis in the pre-paralytic stage; and from other sources 7
8 there was evidence of a change in the epidemiology of poliomyelitis.
Against this background of intense interest in poliomyelitis and
its problems came reports of outbreaks 9-11 and isolated cases 12
13 which, for one reason or another, led to difficulties in
diagnosis. Broadly these can be divided
into those in which the cerebrospinal fluid (c.s.f.)
is abnormal and those in which it is normal.
Of the abnormal group (group I), only in the series described by Laurent 10 was no convincing
causative organism isolated. The conditions described by Kelleher 12 and Jennings 13 proved to be
aberrant poliomyelitis, and there was presumptive evidence of the same disease
in the outbreak reported by Barrett
9 from Cambridgeshire in 1949.
The Coxsackie group of viruses has also been implicated in this group 11
14 : in such cases pleocytosis is the rule and signs of parenchymal
damage to the nervous system are very uncommon. Much more perplexing outbreaks are those in which no changes are
found in the c.s.f. (group II).
Since we discussed these illnesses in 1954 under the non committal title, Not
Poliomyelitis,15 another epidemic with similar features has been
reported from Durban 16 17 and two further outbreaks are described
in this issue by Dr. Sumner and
by Dr. Ramsay and Dr. O’Sullivan. Only a brief description
18 has so far been published of the alarming outbreak at the Royal Free
Hospital last year, but there are arguments for including it in this
group. In none of these cases has it
been possible to incriminate the poliomyelitis or Coxsackie virus, nor indeed
has any other known infective agent been isolated.
There seem good reasons, in our present state of ignorance, for
placing in a third and intermediate group the epidemic which took place in
Akureyri, Iceland, in the winter of 1948-49,19 and about which Dr. Sigurdsson and Dr. Gudmundsson write on p. 766. In all 8 cases examined the c.s.f. was abnormal ; on the other
hand, the protracted course and mental symptoms described by Dr. Sigurdsson are prominent symptoms in
group II. The outlook in the nurses
training school in the University of Pennsylvania in 1945 20 is also
difficult to classify since it happened before the isolation of the Coxsackie
viruses : there was pleocytosis in 2 out of 5 cases. The unusual epidemic reported by Wallis from Cumberland in 1955 21 has many features
of group II – notably vertigo, diplopia, myalgia, cervical lymphadenopathy, and
protracted convalescence with mental symptoms.
Unfortunately there is no information about the c.s.f. The recorded
atypical outbreaks can be grouped as follows:
group Laurrent 10 (1947) Unknown
ü
I Kelleher et al 12 (1949) Poliomyelitis ú usually
Curnen et al 11 (1949) Coxsackie ú abnormal
Jennings
et al 13 (1949) Poliomyelitis
ý
Barrett
et al 9 (1952) Poliomyelitis
ú
Galpine
and Macrae 14 Coxsackie
ú
(1953) and others þ
group Adelaide
22 (1949) Unknown
ü
II New York State 23
(1950) Unknown ú
Middlesex
Hospital 24 (1952) Uncertain ú Normal in
Coventry
25 (1953) Unknown
ú nearly all
Berlin
(1954) Sumner Unknown
ý cases
Durban
17 (1955) Unknown ú
Royal
Free Hospital 18 (1955) Unknown ú
Hampstead
(1955) Unknown
ú
(Ramsay and O’Sullivan) þ
group Pennsylvania
20 (1945) Unknown
Abnormal 2/5
III Akureyri, Iceland 19 (1948) Unknown Abnormal 8/8
Cumberland
21 (1955) Unknown
Unknown
Of the 8 outbreaks in group II, all except that
at the Royal Free were initially confused with poliomyelitis, and all occurred
during or shortly after the seasonal period of prevalence of
poliomyelitis. The three British
outbreaks 18 24 25 were in late summer, in contrast to the former
peak incidence of encephalitis lethargica in the first three months of the
year.2 Five outbreaks took
the form of dramatic localized epidemics, four of which were in nurses’
homes. Dr. Ramsay and Dr. O’Sullivan
describe cases in the neighborhood of one of these outbreaks, and Hill 17 had a similar
experience in Durban. The attack rate
in closed communities is high. The
onset in this group is usually acute with systemic prodromata such as are
common in poliomyelitis. In contrast,
fever is usually low and may be absent.14 18 25 The course is
generally two to eight weeks but occasionally symptoms may last for
months. Relapses are frequent. Usually the outcome is favourable but in a
few cases paresis or mental sequelae may be incapacitating for many months.22
23 26 Depression, emotional
lability, or irritability in convalescence have been a constant feature in all
group II outbreaks. Although previous
experience has shown that a long period of observation will be necessary before
the harmlessness of the disorder is assured, it can at least be said that the
immediate mortality-rate of nil is in striking contrast to the epidemic
infections of the nervous system previously described 4 ; and this
in itself is very encouraging.
Among the more characteristic features of group
II are the severe muscular pains, often accompanied by exquisite tenderness,
which often dominate the clinical picture.22-25 As White and Burtch 23 have pointed out, these pains differ
from those of poliomyelitis in that they are not simply a short-lived precursor
of paresis but may last for weeks. Most
commonly they affect the neck, back, or limbs but there may also be
Bornholm-like chest and abdominal pains.17 18 23 25 Continuous or intermittent painful muscular
spasms were noted in the outbreaks at the Middlesex and Royal Free Hospitals,
and they are also reported by Dr. Ramsay
and Dr. O’Sullivan. In nearly every patient there are symptoms
or signs of disease of the central nervous system, but the weight and site of
the damage vary considerably from outbreak to outbreak. The Hampstead and Berlin epidemics
illustrate this variation. The
innervation of the eye muscles (diplopia and nystagmus) and the seventh and
eighth cranial nerves (deafness, hyperacusis, vertigo, and facial weakness)
suffer most commonly. Sensory symptoms
and signs are common and pyramidal signs have also been observed. Some patients in Adelaide and Durban and at
the Middelsex Hospital had retention of urine.
The paresis, usually short-lived but occasionally persisting for weeks
or months, is in itself an interesting problem, for in many cases it is not
accompanied by the classical disturbances of tone and reflexes which would
point to damage in the anterior horn or pyramidal tract.23 25 26 Pain, muscular spasm, and involuntary
movements often make the degree of palsy difficult to assess. In this connection the striking
electromyographic records obtained by Dr. Ramsay
and Dr. O’Sullivan are of great
interest. Although they do not yet
point to the exact nature of the lesions, they may provide evidence of organic
paresis in patients who might otherwise be suspected of hysteria, and in a
disease at present so bereft of positive laboratory findings they may be a help
in diagnosis in the future.
The outbreaks mainly differ in the severity and
site of the damage to the nervous system; but the lymph glands are another
point of difference. Enlarged lymph
glands, particularly in the posterior cervical triangle, were prominent in the
Hampstead and Royal Free cases, and they were also noted in 4 cases by White and Burtch 23 and in the more doubtful
Cumberland outbreak.21 In
retrospect lymphadenopathy was found to have been present in 2 of the 14 cases
reported from the Middelsex.27
Hepatitis and splenomegaly may also turn out to be part of the
picture. It is doubtful whether the
absence of these features in the other reports can be attributed entirely to
observer error 27 and it must be accepted as a real
discrepancy.
A study of the available material in group II
shows sufficient common ground to
suggest that this is a new clinical entity which may be expected to appear
again here or elsewhere in the late summer and autumn. From a purely practical standpoint it would
be useful to have a name for this syndrome.
As the most helpful single feature in the recognition of this syndrome
in the past has been the predominantly normal cerebrospinal fluid, the names
that have already been suggested, “Iceland disease” 16 23 and
“Akureyri disease,” are not really appropriate. The objections to any but a purely descriptive name for a
disorder without a known cause or established pathology are obvious. For this reason the term “benign myalgic
encephalomyelitis” may be acceptable.
It in no way prejudices the arguments for or against a single or a
related group of causal agents ; and it does describe some of the striking
features of a syndrome characterized by (1) symptoms and signs of damage to the brain and spinal cord, in
a lesser or greater degree; (2) protracted muscle pain with paresis and cramp; (3) emotional disturbances in convalescence; (4) normal c.s.f.;
(5) involvement, in some variants, of
the reticuloendothelial system; (6) a
protracted course with relapses in severe cases; (7) a relatively benign outcome.
It remains to identify this syndrome more precisely; but we believe that
its characteristics are now sufficiently clear to differentiate it from
poliomyelitis, epidemic myalgia, glandular fever, the forms of epidemic
encephalitis already described, and need it be said, hysteria.
1.
von
Economo, C. Wein. Klin. Wschr. 1917, 30, 581
2.
Hall,
A.J. Epidemic Encephalitis. Bristol, 1924
3.
Kinnier
Wilson, S.A. Lancet, 1918, ii, 7.
4.
MacNalty,
A.S. Epidemic Diseases of the Central
Nervous System. London, 1927.
5.
Epidemic
Encephalitis. 3rd Report by
the Matheson Committee. New York, 1939.
6.
Russell,
W.R. Poliomyelitis. London, 1956.
7.
Dauer,
C.C. Amer. J. Hyg. 1948 48, 133.
8.
MacLean,
F.S. New Zealand Department of Health,
Annual Report of the Director General, 1945, p. 90
9.
Barrett,
A.M., Gairdner, D., McFarland, A.M. Brit.
Med. J. 1952, i, 1317.
10.
Laurent,
L.J.M. Proc R. Soc. Med. 1947, 40, 927.
11.
Curnen,
E.C., Shaw, E.W., Melnick, J.L. J. Amer. Med. Ass. 1949, 141, 894.
12.
Kelleher,
W.H., Bratton, A.B., MacCallum, F.O. Brit.
Med. J. 1949, ii, 213.
13.
Jennings,
G.H., Hamilton-Paterson, J.L., MacCallum, F.O.
Ibid, p. 210.
14.
Galpine,
J.F., Macrae, A.D. Lancet, 1953, I, 372.
15.
See
leading article, Ibid, ii, 1060.
16.
Editorial, S. Afr. Med. J. 1955, I, 331.
17.
Hill,
R.W., Ibid. p. 314.
18.
See
Lancet,
1955, ii, 351.
19.
Sigurdsson,
B., Sigurjonsson, J., Sigurdsson, J., Thorkelsson, J.. Gudmunsson, K.R. Amer. J. Hyg. 1950, 52, 222.
20.
McConnell,
J. Amer. J. Med. Sci. 1945, 209, 41.
21.
Wallis,
A.I. 1955, Lancet, ii, 290; Ibid. p.
1091.
22.
Pellew,
R.A.A. Med. J. Aust. 1952, i,
944.
23.
White,
D.N., Burtch, R.B. Neurology, 1954, 4,
506.
24.
Acheson,
E.D., Lancet, 1954, ii, 1044.
25.
Macrae,
A.D., Galpine, J.F. Ibid,
p. 350.
26.
Agnams,
J.N., personal communication.
27. Acheson,
E.D. Lancet, 1955, ii,
394.
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In depth reports on some of the epidemics
of the 1970’s, 80’s and 90’s can be seen here; http://www.oocities.org/tcjrme/CurrentTopics3.html
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