55

THE LANDRY-GUILLAIN-BARRE SYNDROME

BY

JOHN MARSHALL
(From the Institute of Neurology and National Hospital for Nervous Diseases,
Queen Square, London, W.C.I)

EPONYMOUS nomenclature for diseases has its advantages; it is a

convenient cover for ignorance of the nature of a disease, and it avoids the

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too ready acceptance of an aetiological hypothesis for which there may be
little or no evidence. A good example is provided by the term "Landry-
Guillain-Barre" Syndrome," which implies nothing as to causation,
whereas "acute infective polyneuritis" purports an infective agent as the
cause; the evidence in favour of this view is negligible. On the other hand,
the use of eponyms may raise a difficulty of definition; what is to be sub-
sumed under the particular title? Modesty forbids an author to attach
his name to a syndrome of his own definition. His name is applied by
others, among whom there may be disagreement as to what he described.
These problems are not solely the concern of the medical historian.
Progress in the discovery of the cause and treatment of a syndrome may
be hindered because patients are excluded who should be included, or
included who should be excluded, so that anomalous responses to
diagnostic tests or therapy confuse the issue.
The Landry-GuiUain-Barre" syndrome is very much a case in point.
In 1916 Guillain, Barre" and Strohl described 2 patients with "motor
disturbance, abolition of tendon reflexes with preservation of cutaneous
reflexes, paraesthesiae with slight affection of objective sensation, pain
on pressing the muscles, slight changes in the electrical reactions of nerves
and muscles and a very marked increase in the albumen content of the
cerebrospinal fluid with absence of a cellular reaction." To this descrip-
tion Draganescu and Claudian applied the term GuiUain-Barre" syndrome
in 1927. In 1936 Guillain re-emphasized the description given above,
and laid special stress upon the albumino-cytological dissociation and
upon a favourable prognosis.
In 1949 Haymaker and Kernohan reported 50 fatal cases under the
title Landry-Guillain-Barr6 syndrome and provided an excellent and
56 JOHN MARSHALL

comprehensive review of the literature. They started from the patho-

logical standpoint, obtaining the clinical story from the case-notes of
patients on whom autopsies had been performed. They pointed out
that when Landry in 1859 described the form of paralysis to which his
name was given, there were three clinical groups to be discerned in the
10 cases he reported. One of these, namely that in which the paralysis
began in the limbs and then affected the trunk and cranial nerves, objective
motor disturbance being much more evident than sensory, was in-
distinguishable from that described by Guillain, Barre" and Strohl
(1916).
In their own series Haymaker and Kernohan (1949) observed that,
though some cases had been diagnosed during life as suffering from the
Guillain-Barre" syndrome and others from Landry's paralysis, all had a
common pathology. The spinal nerves and adjacent segments of peripheral
nerves and nerve roots showed first oedema, followed by swelling and
irregularity of the myelin sheaths and axis cylinders. Later lymphocytes

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and phagocytes appeared in succession, and finally there developed a
proliferation of the Schwann cells. Haymaker and Kernohan, therefore,
proposed the use of the term Landry-Guillain-Barre' syndrome.
Recently, Osier and Siddell (1960) reporting 10 cases made a plea for
a return to the exact diagnostic criteria laid down by Guillain, Barrd
and Strohl in 1916 and by Guillain in 1936. They declined to accept the
clinical diagnosis of the Guillain-Barre' syndrome for many of the cases
reported by Haymaker and Kernohan (1949). Their plea received support
in an annotation in the Lancet (1960). Osier and Siddell especially rejected
the inclusion of cases with severe objective sensory loss, involvement of
the bladder and bowel and raised cell counts in the cerebrospinal fluid.
The observations of Osier and Siddell prompted this review of the
patients with acute polyneuropathy who have been treated in the Batten
Respiratory Unit. The object of the review was to see how far the
criteria laid down by Guillain, Barre" and Strohl (1916), and Guillain
(1936), and re-emphasized by Osier and Siddell (1960), could be applied
in practice, and indeed how far Guillain's original cases conformed to
these criteria.
There were 35 patients in all. Not all received assisted respiration, but
all were so severely affected that it was thought the need might arise.
Intensive investigations were carried out to ascertain the cause of the
polyneuropathy. In some cases, not included in this series, porphyria or
infectious mononucleosis was found to be the cause. In the cases included
in the series, in addition to the negative criterion of failure to find a cause
for the polyneuropathy, there were positive features in the clinical picture
which are set out in this report and which resulted in a diagnosis of
Landry-Guillain-Barr6 syndrome being made.
 THE IJVNDRY-GUILLAIN-BARRE SYNDROME 57

RESULTS
The 35 patients included 22 males and 13 females. Their age distribution
is given in Table I.
TABLE I . A G E AND SEX DISTRIBUTION
Age in years Males Females Total
0-9 1 1 2
10-19 1 1
20-29 2 2
30-39 3 1 4
40-49 4 5 9
50-59 4 3 7
60-69 5 3 8
70-79 2 2

22 13 35

Month of onset.Because of the implication in the term acute infective

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polyneuritis of an infective agent as the cause, evidence of a seasonal
incidence, such as occurs in some conditions of viral aetiology, was sought.
The month of onset of the illness is given in Table IT. This reveals no
TABLE II.MONTH OF ONSET
Jan. Feb. Mar. Apr. May June July Aug. Sept. Oct. Nov. Dec. Total
4 2 5 3 5 2 1 2 2 5 2 2 35

clear evidence of a seasonal incidence, though there were slightly fewer

cases in the months June to September than there were during the
remainder of the year. These findings are in keeping with those of
Haymaker and Kernohan (1949).
Preceding illness.Another aetiological hypothesis has attributed the
condition to an allergic response to a preceding illness. Eleven of the
present series gave a definite history of such an illness. In 7 this was a
respiratory infection, in 3 a gastro-intestinal upset, in 2 a pyrexia of
unknown origin and in one a herpes zoster involving the trigeminal nerve.
Two of these patients had both a respiratory and gastro-intestinal illness.
Twenty-one patients denied any preceding illness, and in 3 there was no
definite information available. This incidence of previous illness was
less than that reported by Haymaker and Kernohan (1949), 80 per cent
of their patients having a prodromal illness. There was no instance of
immunisation preceding the illness in the present series.
Site of onset.In 16 instances the polyneuropathy presented with motor
weakness, in 14 with sensory symptoms including pain and in 5 with
both motor and sensory disturbance. In 19 cases the first symptoms were
in the lower limbs, in 13 in the upper, in 2 in the cranial nerves and in 1
both upper and lower limbs and cranial nerves were involved from the
58 JOHN MARSHALL

outset. Of the 16 patients who presented with motor weakness, in 2 the

proximal limb muscles were affected, in 2 the distal and in the remaining
12 there was no definite information. Of the 14 patients who began with
sensory symptoms, 10 gave a clear account of the site of onset and in every
case it was in the distal part of the limbs.
Mode of spread.Because the symptoms and signs are dramatic and
demand attention, it is possible to follow the mode of spread of the illness
with considerable accuracy. There were 12 patients who started with
motor symptoms in the lower limbs. In 5 of these the next development
was the appearance of sensory symptoms in the lower limbs, in 5 of motor
symptoms in the upper limbs, in one of sensory symptoms in the upper
limbs, while in one there was no spread of the illness. Four patients
presented with sensory symptoms in the lower limbs. In 3 of these motor
symptoms in the lower limbs came next and in 1 sensory symptoms in the
upper limbs. Three patients began with motor symptoms in the upper
limbs, 2 of whom next developed motor symptoms in the lower limbs,

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and 1 involvement of the cranial nerves. There were 9 patients whose
first symptom was sensory disturbance in the upper limbs. Six of these
next developed motor symptoms in the upper limbs, 2 motor symptoms
in the lower limbs and one sensory symptoms in the lower limbs.
There was therefore no outstandingly predominant mode of spread,
but there was a slight tendency for the illness to develop by involving
further functions in the limbs first affected before spreading to other parts.
The progressively involved parts were not always contiguous. Thus one
patient (Case 31) began with motor disturbance in the territory of the
cranial nerves, the next development being the appearance of motor
symptoms in the trunk. Another patient (Case 10) started with sensory
symptoms in the cranial nerve territory which was followed by sensory
symptoms in the lower limbs. Likewise, one of the patients (Case 5)
who started with both motor and sensory symptoms in the lower limbs
next developed symptoms in the territory of the cranial nerves, the upper
limbs being spared.
Duration of spread.The period during which the symptoms spread is
shown in Table DI. In one patient (Case 13) the illness was generalized
TABLE HI.DURATION OF SPREAD OF ILLNESS IN DAYS
No. of days 1 2 3 4 5 6 7 8 10 12 14 42
No. of patients 3 10 1 2 2 4 4 1 1 1 2 1
1 (Case 13) generalized from the onset. 1 (Case 1) no spread. 1 (Case 33) insufficient
information.

from the onset and could well be described as fulminating, and in another
patient (Case 1) the illness did not appear to spread. In the majority
(26 of the 35 patients) spread was complete within seven days of the onset.
 THE LANDRY-GUILLAIN-BARRE SYNDROME 59

In one patient (Case 11), a woman of 56 years, the symptoms continued

to spread over a period of six weeks. She began wich paraesthesiae in
the hands and then the feet, and three weeks later developed weakness
in the same sites. The weakness gradually spread to involve the proximal
muscles of the limbs and later the trunk, until after eighteen days she
first noticed difficulty in swallowing. Her cerebrospinal fluid on the
thirty-third day of the illness contained 2 lymphocytes per c.mm. and
55 mg. per 100 mm. of protein. She made a complete recovery, being
able to leave hospital three months from the onset.

THE FULLY DEVELOPED ILLNESS

Headache and meningism.Five of the patients complained of headache.
Only 3 showed signs of meningism which is roughly the same proportion
as occurred in the series of Haymaker and Kernohan (1949). The cell
count in the cerebrospinal fluid of the patients with meningism (Cases
22, 30, 31) was 14, 0 and 5 per c.mm. respectively; other patients with

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14 cells per c.mm. or more had no meningism. The protein content of
the cerebrospinal fluid in these 3 patients was 130, 350 and 40 mg. per
100 ml. respectively.
Cranial nerves.Six patients showed involvement of the oculomotor
nerves. Two (Cases 13 and 31) had a complete external ophthalmoplegia
with ptosis; the pupillary light reflexes were preserved, but the con-
vergence reflex was lost because of inability to converge the eyes. Two
patients (Cases 12 and 20) had weakness of the external rectus muscles,
which was accompanied in 1 by bilateral ptosis; and 2 further patients
(Cases 6 and 27) had bilateral ptosis alone.
The trigeminal nerve was affected in 8 patients, the facial nerve in 21
and the nerves arising from the medulla in 19.
Motor weakness.Every case had some degree of motor weakness, and
in most instances it was of severe degree. In 24 cases it involved the
proximal and distal muscles of both upper and lower limbs, as well as the
muscles of the trunk. In 4 instances only the proximal muscles of the
upper and lower limbs were involved along with the trunk and in 1 case
only the distal muscles of the limbs along with the trunk. In 4 cases the
weakness was confined to the limbs involving both proximal and distal
muscles, and in one further case (Case 1) the proximal and distal muscles
of the lower limbs only were affected. In one case (Case 5) there was
weakness of the proximal and distal muscles of the lower limbs to-
gether with the cranial nerves.
Reflexes.All the patients lost all their tendon reflexes. This was true
even of the patient (Case 1) who presented with motor weakness in the
lower limbs and in whom the symptoms did not appear to spread; the
60 JOHN MARSHALL

tendon reflexes in his upper limbs were all absent. The plantar responses
in the series were either unobtainable or flexor.
Sensation.In the fully developed illness 19 of the 35 patients complained
of pain, which is almost identical with the 56 per cent found by Haymaker
and Kernohan (1949). By contrast only 6 patients (Cases 12, 27, 29, 32,
33, 35) had appreciable tenderness of the muscles, compared with 16 in
the Haymaker and Kernohan series.
Numbness and paraesthesiae occurred at some stage of the illness in
29 of the present series. Objective sensory loss was found in a high
proportion. Superficial sensibility (light touch and pin-prick) was affected
in 23 patients, deep sensibility (joint position and vibration) in 25 and
both superficial and deep sensibility in 22 patients. The severity of the
sensory disturbance was considerable. Thus joint position sense was
lost in the fingers or toes in 9 patients, 2 up to the ankles and wrists (Cases
16 and 23), grossly impaired in 7 and considerably impaired in 5.
Similarly, vibration sense was lost up to the hips in 9 instances, to the

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knees in 1, and to the ankles in 3. Light touch was impaired up to the
hips in 2 patients, to the knees or elbows in 10 and to the wrists or ankles
in 4. The appreciation of pin-prick was impaired up to the hips in 2
patients, to the knees or elbows in 7 and to the ankles or wrists in 7.
The extent and severity of the sensory loss was parallelled by its
persistence. In those patients in whom several modalities were affected
over a considerable area sensory loss persisted over many days or weeks.
Sphincters.Fourteen patients had disturbance of control of the vesical
sphincter. In 9 of these this caused retention and in 5 incontinence. Two
of the patients with retention (Cases 27 and 32) lost awareness of fullness
of the bladder, and 2 other patients (Cases 4 and 16) lost urethral sensation.
In 6 instances the loss of control of the vesical sphincter was accompanied
by a similar disturbance of the rectal sphincter. The disturbance of the
vesical sphincter was by no means transient, often necessitating inter-
mittent or tidal drainage of the bladder for many days, the longest period
being 30 days in Case 11.
Pyrexia.Because the method of selection produced a high incidence
of respiratory complications in this series, it is difficult to make any
statement about pyrexia. If the temperature in the first twenty-four
hours after admission is taken as the best guide, 28 patients had a normal
temperature. Three had a temperature of 99 F., 1 of 100 F., and 3 of
101 F.
Blood pressure.The blood pressure showed considerable variations
from patient to patient, and in the same patient from time to time.
Severe hypertensive and hypotensive crises occurred, but again because
of the incidence of respiratory failure it is difficult to be dogmatic as to
which changes were due to ventilatory insufficiency and which to the
 THE LANDRY-GUTLLAIN-BARRE SYNDROME 61

primary condition. Not all the changes could be attributed to hypoxia

or hypercapnia, however, and sudden changes of blood pressure occurred
not infrequently in well ventilated patients. This is being made the subject
of a separate study.
The cerebrospinal fluid.The definition of albumino-cytological dis-
sociation is difficult. Guillain (1936) stated that the cerebrospinal fluid
protein be 1 to 2 grammes per 100 ml., and regarded cases with 300 to
400 mg. as not of the syndrome or constituting an abortive form. On
this criterion many otherwise typical cases described in the literature and
many currently seen in hospital would have to be excluded. It is likewise
difficult to assert what is the maximum number of lymphocytes permissible
for inclusion in this syndrome. Moreover, both the cell count and the
protein content depend considerably on the stage of the illness at which
the cerebrospinal fluid is examined.

5O-

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45

4O-
mm.

35
u
K>-
<D 25
Q.
OO JO-
1
CEL

15

0-

2 4 8 (O 12 14 16 IB 2O 22 24 26 28 3O 32 34 36 38 4O 42 44
DAY OF EXAMINATION
FIG. 1.Cell count in the cerebrospinal fluid in relation to day of illness.

The results of the cell count in the present series are given in fig. 1, and
that of the protein content in fig. 2, in each instance in relation to the
day of the illness. The cell count was less than 5 per c.mm. in 27 of the
34 cases in which the cerebrospinal fluid was examined. In 3 it was under
15, in 2 under 25 and 2 (Cases 5 and 23) had counts of 50 and 46
lymphocytes respectively. In the 8 patients who had two examinations
of the cerebrospinal fluid there was no significant difference between the
counts except in Case 32. On day 11 there were 24 cells per c.mm. and
85 mg. of protein per 100 ml. and on day 30 there were 0 cells and 140 mg.
of protein. Thus on day 11 it could not be said there was an albumino-
cytological dissociation, whereas on day 30, by criteria less stringent than
62 JOHN MARSHALL

those of Guillain but nevertheless widely accepted, there was a dissociation

present.
If 45 mg. of protein per 100 ml. is taken as the upper limit of normal,
7 patients had a protein content below this limit, but all these had one
examination only. The importance of repeated examination is illustrated
by Case 24 in which the protein content on day 4 was only 20 mg. per
100 ml. whereas by day 16 it had risen to 140 mg. An even more dramatic
change occurred in Case 35 in which the protein rose from 330 to 1,000
mg. between day 2 and day 7. There is an insufficient number of observa-
tions to enable firm conclusions to be drawn but fig. 2 suggests that the
maximum rise of protein occurs between the fourth and the eighteenth
days.

IOOO
95O

90O

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BSO

800

75O-
ml.

7OO-
OOI

650-

5OO-

4SO-
JJ 40O-

5. 3SO-
- 3OO-
250-
2OO-
ISO-
IOO-
so-

O i 4 (, B IO 12 14 16 18 2O 21 24 26 28 3O 32 34 34 38 4O 42 44
DAY OF EXAMINATION
FIG 2.Protein content of the cerebrospinal fluid in relation to the day of illness.

Outcome.Four of the patients (Cases 7, 14, 19 and 25) died, the cause
of death being respiratory failure in 3 and cardiac arrest in one. Recovery
was complete in 28 of the patients, incomplete in 2 (Cases 20 and 30)
and one patient (Case 17) was lost sight of so the final degree of recovery
 THE LANDRY-GUILLAIN-BARRE SYNDROME 63

was unknown. The length of stay in hospital of the patients is given in

Table IV.
TABLE IV.DURATION OF STAY IN HOSPITAL IN WEEKS OF 31 SURVIVORS
No. of weeks 1-4 5-8 9-12 13-16 17-20 20+ Total
No. of patients 4 8 9 3 1 6 31

Treatment with steroids.Four patients were given ACTH, 1 cortisone

and 8 prednisone. Of the 4 patients who died, 1 (Case 25) received ACTH,
one (Case 19) prednisone and 2 received no steroids. Neither of the
2 patients whose recovery was incomplete received steroids. Of the
28 patients who made a complete recovery 11 received steroids and
17 did not.
DISCUSSION
The 35 cases described here were all accepted as examples of the
Landry-Guillain-Barre syndrome, yet several would have been rejected

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had the criteria demanded by Osier and Siddell (1960) been applied.
These authors especially reject profound and persistent sensory loss and
severe bladder involvement as occurring in this syndrome. Reference to
the original description by Guillain, Barre and Strohl (1916) of their
2 cases and the restatement by Guillain in 1936 with the addition of 10
further cases, raises doubts as to the validity of this view. It is true that
Guillain, Barr6 and Strohl speak of "troubles legers de la sensibility
objective," yet in the case reports Guillain (1936) refers in Case 1 to
"severe disturbance of position and vibration," in Case 3 to "loss of
vibration and position in all limbs" and in cases 5 and 10 to "severe
disturbance of deep sensibility." It is apparent therefore that 4 of his
10 cases showed a degree of sensory disturbance which would preclude
their inclusion under the title Guillain-Barr^ syndrome as defined by
Osier and Siddell.
There are, of course, cases in which the sensory disturbance is transient
or even never apparent, though in the latter group it may be legitimate to
wonder if minor degrees of sensory loss may not be overlooked in a
seriously ill patient threatened with respiratory paralysis. But the question
of the existence of a pure motor polyneuropathy deserves consideration,
if only from the practical standpoint of distinguishing it from polymyositis.
This latter condition is usually subacute or chronic, but may present
acutely and threaten respiration. Its dramatic response to steroids
behoves us to consider it carefully in differential diagnosis, and not to
misdiagnose such cases as examples of the Landry-Guillain-Barre' syn-
drome with fleeting or absent sensory disturbance.
Involvement of the vesical sphincter has been a similarly disputed
point. One of the 2 cases described by Guillain, Barr6 and Strohl (1916)
"ne percoit pas Pecoulement des urines," a state of affairs which was
64 JOHN MARSHALL

clearly not due to immobility in bed or weakness of the abdominal muscles,

but indicated involvement of the innervation of the urethra. Likewise,
Cases 4 and 5 in Guillain (1936) had bladder involvement, and Case 6
involvement of the rectal sphincter.
In the present series, 14 of the patients showed involvement of the
bladder. That this was more than could be dismissed as being due to
immobility or weakness of the abdominal muscles is shown by its severity
and persistence in several cases, by the occurrence of incontinence which
was not due to overflow, and by involvement of the sensory side of the
reflex arc in 4 instances. The concomitant involvement of the rectal
sphincter in 6 patients was further evidence that the innervation of the
bladder and rectum was affected. Indeed it would be surprising if this
were not so. The pathological lesion in the Landry-Guillain-Barre
syndrome is situated in the spinal nerves where the anterior and posterior
roots fuse, and extends a short distance proximally and distally. For
autonomic fibres always to escape damage as they pass through this site

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would be remarkable. Neither historical accuracy, clinical observation
nor pathological findings justify the exclusion from the Landry-Guillain-
Barre syndrome of patients in whom there is severe or persisting sensory
loss or in whom the innervation of the bladder is disturbed.
The question of the albuminocytological dissociation is more difficult.
Failure to find this phenomenon at one examination of the cerebrospinal
fluid clearly cannot prevent inclusion of the case in the Landry-Guillain-
Barre syndrome for re-examination at a later date may reveal its presence
as was illustrated by Case 24. But what of the cases in which the positive
phenomenon of a rise in the cell count is present ? Some of these cases may
well be due to mumps and mononucleosis, but after care has been taken to
exclude these, there are undoubtedly cases of the Landry-Guillain-Barre
syndrome, in every way typical, but showing a considerable rise in the cell
count of the cerebrospinal fluid. There were 4 cases in the present series
with a cell count of over 20 per cmm. One (Case 27) showed no rise in
protein at one examination on the fourth day of the illness, but the others
were in every respect typical. As a rise in cell count is so objective a
criterion it would seem reasonable to separate cases showing this pheno-
menon from the remainder.
The response to steroids was particularly difficult to assess in the
present series. Twenty-eight of the patients has respiratory involvement
and there can be no doubt that in some instances the improvement
following the administration of steroids could better be attributed to the
simultaneous relief of hypoxia or hypercapnia by assisted respiration. On
the other hand, deterioration in the clinical state after steroids has been
started was on occasion due to inadequate pulmonary gaseous exchange
because of infection and other causes. Jackson, Miller and Schapira (1957)
presenting 5 cases of their own and reviewing 68 cases from the literature
 TABLE V.SUMMARY OF FINDINGS

Fully developed illness

Duration
Site of onset of Cranial nerves Sensory Sphincter C.S.F. Stay
spread involvement in
Hospital Prodromal Upper Lower in Motor Pain Numbness Superficial Deep Respiratory Day of Cells Protein hospital
No. No. Age Sex illness limbs limbs Cranials days Headache Meningism 346 5 7 9-12 parcesthesice sense sense Vesical Rectal involvement illness per c.mm. mg.jXQO ml. Therapy in weeks Outcome

1 97877 50 M M 1 10 4 40 None 1 R
2 50444 34 F R.G. M 6 + LT 7 0 500 C 16 R
3 95944 52 M S 2 L 13 1 75 P 26 R
4 61933 43 M S 6 LT 12 3 450 A 12 R
5 A397 49 M P.U.O. MS 7 + 1 3 50 400 P 6 R
6 99948 68 M G MS 6 + + LT 6 2 750 P 10 R
7 A2449 62 M S LT 2 2 60 None 3 D
7 4 160 None
8 65313 53 F s 4 + + + LT 18 3 300 None 28 R
9 78686 63 F S 1 + + LT 3 4 40 None 2 R Key:

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10 79202 40 M S 5 + + L 9 12 130 None 4 R M = motor.
11 79879 56 F s 42 + LT 33 2 55 P 9 R S = sensory.
12 81449 45 F M 7 + + + PT 7 0 190 None 22 R L = proximal and distal
13 81711 29 M MS MS MS + + PT 4 0 140 P 12 R muscles of limbs.
14 83292 66 M R S 2 + + + LT 1 1 45 None D T = trunk.
15 84562 73 M R M 10 PT 8 0 240 P 10 R P = proximal muscles of
16 85076 66 M H s - 8 LT 6 0 90 P 5 R limbs only.
17 85549 65 M s 5 + LT 5 0 200 None 7 U D = distal muscles of limbs
18 89625 53 M R M 2 + + LT 10 2 400 None 5 R only.
90495 70 M MS 2 + + LT 4 100 6 D
19 3
2 130
P 1 = proximal and distal
44 None muscles of lower limbs.
20 49809 9 F RG M + + + LT 4 2 20 None IR A = ACTH.
10 0 50 None C = cortisone.
21 58036 46 M M 14 + L 11 14 600 None R P = Prednisone.
22 57686 26 M M 2 L 4 14 130 None 13 R R = recovered.
23 58647 47 F R M 12 + LT 14 46 420 A 14 R IR = incomplete recovery.
68779 61 F M 2 LT 4 0 D = died.
24 0
20\
None 20 R U = unknown.
16 140/
25 50801 40 F _ M 1 + LT A D
26 52533 43 F S 7 + + + LT 9 0 180\
R
None
21 2 225/
27 59161 49 F s 2 + + LT 4 25 30 A 12 R
28 67869 33 M M 3 + + LT 1 1 83 None 17 R
29 53131 32 M M 7 + + LT 9 3 40 None 5 R
30 51696 30 M M 1 PT 3 0 350 None 30 R
31 51291 8 M P.U.O. M 4 + + + LT 3 5 40 None 35 R
32 58345 55 F R MS 2 LT 11 24 85
None 10 R
30 0 140
33 89743 68 F S + LT 28 0 20 None 6 R
34 87013 57 M M 2 + DT 4 0 30^1
None
0 11 R
8 60/
35 94804 17 M 14 + + + LT 9 2 330 \
7 None R
32 1000/
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 THE LANDRY-GU1LLA1N-BARR6 SYNDROME 65'

had similar difficulty. Fifty of the 68 cases had shown some response to
steroids though in one instance (Grant and Leopold 1954) the Landry-
Guillain-Barre' syndrome developed while the patient was receiving steroid
therapy. Stanton (1961), on the other hand, from a survey of the literature
and personal experience is of the opinion that the majority of the patients
who show an albumino-cytological dissociation are benefited by steroid
therapy. The minority (about 10 per cent), who are not so influenced, he
thinks are probably of different aetiology.
Of the 13 patients in the present series who received steroids 2 died, but
in both cases the cause of death was pulmonary collapse. The remaining
11 patients made a complete recovery. Against this we must weigh the
fact that of the 22 patients who did not receive steroids 2 died, 2 made an
incomplete recovery and 18 a complete recovery. Although the duration
of stay in hospital is influenced by many factors such as medical compli-
cations and the social condition of the patient it might be expected to
reflect to some extent any benefit conferred by steroids. The mean duration
of stay in the treated and untreated groups was 120 and 11-6 weeks

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respectively, which allows no advantage to either group. We must, there-
fore, agree with Jackson et al. (1957) that, while the use of steroids can be
supported on theoretical grounds, and is justified in practice, it cannot be
said to be supported by sound statistical evidence.
We cannot, however, agree with the Lancet (1960) that it is satisfactory
to separate those cases of subacute or chronic polyneuropathy which
respond to steroids from the rest. Such a step would be even less logical
than grouping together those illnesses which respond to penicillin,
for in the latter instance the therapeutic agent has at least a degree
of specificity.
A more satisfactory approach is to make an intensive effort to ascertain
the cause of every case of acute polyneuropathy. Cases due to porphyria,
infectious mononucleosis and infective hepatitis should be clearly
designated as such. Those which follow a definitive infective illness such
as measles, mumps, chicken pox and rubella should be labelled as post-
infective polyneuropathy, or, if cases following inoculation are to be
included, "allergic polyneuropathy." For the remaining cases of acute
polyneuropathy in which the cause cannot be found the term "acute
polyneuropathy of unknown cause" or the time-honoured term "Landry-
Guillain-Barr" syndrome will serve well, and avoids the assumption of
knowledge we do not possess. But the use of the latter term cannot be
accompanied by too precise a definition which neither exegesis nor clinical
experience justify.
SUMMARY
The clinical features of 35 cases of the Landry-Guillain-Barre syndrome
and the response to steroids has been described. The definition of this
5 BRAINVOL. LXXXVI
66 JOHN MARSHALL

syndrome is discussed and suggestions for an improved nomenclature

made.
ACKNOWLEDGMENT
I wish to thank Dr. Michael Kremer, previously in charge of the Batten
Respiratory Unit, for permission to include patients who were under his
care, and the physicians and surgeons who referred the patients.

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