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									                                                                                          Thomas M, Allen SC    l   Guillain Barre Syndrome

GUILLAIN                     BARRE               SYNDROME           IN ELDERLY                            PATIENTS

Dr. M. Thomas,             MBBS MRCP                               J HK Geriatr Soc 1995; 6: 11- 13
Senior   Registrar
Dr. S.C. Alien, BSc MD FRCP
Consultant     Physician
Deportment  of Medicine        for the Elderly
The Royal Bournemouth         Hospital                             Received in revised form 11 January 1995
Castle   Lane East, Bournemouth        BH7 7DW    United Kingdom   Address correspondence   too: Dr. M. Thomas

Summary                                                            underestimate the incidence in old age. Most studies have
     The age specific incidence     of the Guillain     Barre      shown the incidence in females to be higher than males by
Syndrome (GBS) increases throughout adult life         though      a ratio of approximately 2:1, and the age-specific incidence
there have been no reported studies specifically devoted to        rises approximately four fold between the under 20 age
the GBS in elderly patients. However, there are important          group and the over 60 age group 1,2. The GBS is
aspects of GBS in the olderpatient that affect the diagnosis,      encountered in all regions of the world.
treatment and prognosis when compared to younger
patients. This review explores some of these differences and         Features required       for Diagnosis
conveys the message that clinicians should be aware ofthe
need for early recognition and intervention in GBS in                   ‘Progressive weakness in both arms and both legs.
elderly patients so as to obtain the best possible results of           l Areflexia
                                                                     Features strongly       supporting     the diagnosis

Introduction                                                            *Progression of symptoms over days to four weeks.
      Until recently the incidence of GBS, also known as               ‘Relative symmetry of symptoms.
acute post-infective polyneuritis, was uncertain; there was            *Mild sensory symptoms or signs.
considerable discrepancy between different series and it               ‘Cranial nerve involvement, especially bilateral weakness
was not clear whether          there was an age specific                of facial muscles.
incidence’,‘.      Indeed, some publications   suggested a             *Recovery beginning two-four weeks after progression
bimodal incidence in adult life3.4.5,though these studies               ceases.
were either poorly defined geographically or relied on                 ‘Autonomic dysfunction.
surveillance rather than positive case-finding within a                *Absence of fever at onset.
defined population.       Furthermore, early studies relied            *Elevated concentration of protein in cerebrospinal fluid
upon rather loose criteria for the diagnosis thus leading to            with less than 10 cells per mm3.
either over-estimation     or under-estimation   of the true           ‘Typical electro-diagnostic features.
incidence. More recently, diagnostic criteria (Fig. 1) for the
GBS developed by the National Institute of Neurological              Features making        the Diagnosis    Doubtful
and Communicative Disorders and Stroke (NINCDS) 6have
allowed greater uniformity of diagnosis, and use of these              ‘Sensory level.
criteria in a study based on case-finding within a well                *Marked, persistent asymmetry of symptoms or signs.
defined population and geographical area has shown that                ‘Severe and persistent bladder or bowel dysfunction.
the incidence of GBS increases with age and shows no                   ¡D>50cells per mm3 in cerebrospinal fluid.
bimodal pattern 1 Large scale population meta-analyses
have shown an average incidence of GBS varying between               Features excluding       a diagnosis
0.4 and 1.7 cases per 100,000 population per year 2. The
true overall incidence may be higher than this as there is             *Diagnosis of botulism, myasthenia, poliomyelitis or toxic
some evidence that milder cases are not included in case-               neuropathy.
finding studies either because of diagnostic uncertainty or            *Abnormal porphyrin metabolism.
because they are missed completely when they occur                     ‘Recent diphtheria.
within the context of other neurological disease or general            *Purely sensory syndrome, without weakness.
     This almost certainly       leads to a tendency        to     Figure 1. Diagnostic     criteria for Guillain Barre Syndrome
Presentation                                                        course of the illness than their younger counterparts; this
       The main features of GBS in elderly patients are not         inevitably delays treatment and could affect the prognosis’.
  different to those in young adults, that is, in most cases the        Electrophysiological     studies are useful, though the
 disease presents as an acute sensorimotor neuropathy with         classical findings of GBS may be absent in up to 5% of
 areflexia, though it is worth noting that there are subtle        patients in the first few days of the illness, though they
 variations which can dominate the presentation in some            become almost universal in the later stages of the disease
 individuals. The differential diagnosis of GBS depends on         before recovery begins. This underlines the need for
 the pattern of weakness and the clinical setting. However,        repeated recordings if the first one is normal x. Similarly,
 since the elderly patients tend to present with more              the typical cerebrospinal fluid (CSF) findings of a raised
 concomitant disease than young patients, and especially as        protein and normal cell count may not occur in the first few
 they can often have co-existing neurological disease. there       days of the illness and can take up to a week to become
 can be delay or difficulty in making the diagnosis which          abnormal 8. These factors are particularly important in
 can lead to subsequent delay in treatment and thus affect         elderly people because an elderly patient with multiple
 the prognosis. Although the aetiology is unknown, many            pathology in whom there may be other explanations for
 factors have been implicated in the triggering of what is         weakness could have the diagnosis of GBS overlooked if
 essentially an immunological        disease. Preceding viral      too much reliance is put on electrophysiological      and CSF
 infections have frequently been noted (particularly the           studies early in the illness.
 Epstein-Barr virus and cytomegalovirus), and GBS has also             The clinician needs to retain an open mind and re-visit
 been seen in the wake of vaccination.               surgery and   the possibility of GBS as a diagnosis, particularly if the
treatment with certain drugs.                                      patient is not improving or if another explanation has not
      Some cases have been seen in association with                been found.
bacterial infection, and it has been contended that a
particularly      aggressive    form of GBS can follow             Treatment
Campylobacter jeiuni enteritis.. There is some evidence                 A patient with suspected or proven GBS should be
that preceding gastrointestinal infection is an uncommon            admitted to hospital. In all but the mildest cases, attention
mode of presentation in elderly people .                           should be paid to skin care and avoidance of pressure
      Probably, the most important           of variant form of     necrosis, and DVT prophylaxis with low dose heparin and
presentation in old age iS the very mild case, which is easily     graduated compression         stockings should be given.
eclipsed by the multiple problems often seen in a frail             Ventilator); function should be monitored by twice daily
elderly patient but which can immobilize an individual             measurement of forced vital capacity (FVC) and by
whose mobility is already marginal, Another important              estimation of arterial blood gas tension (or oxygen
variant      form     is the       Miller-Fisher       syndrome    saturation by pulse oximetry). A rapid fall in FVC and
(opthalmoplegia, ataxia and areflexia) though this appears         particularly when the FVC falls towards 1 litre (or 18 ml/kg
to be rare in old age. An uncommon but devastating                 body weight) with accompanying oxygen desaturation,
variant is acute pandysautonomia           which carries a very    should alert a clinician to the possibility that the patient
poor prognosis despite aggressive treatment.                       may require ventilatory support and Intensive Care Unit
      The possibility   of GBS should be included in the           staff need to be involved with the patient’s care. There is a
differential diagnosis of all elderly patients presenting with     high risk of aspiration pneumonia, particularly in patients
acute unexplained        weakness, sensory impairment or           with impaired swallowing and especially when ventilatory
autonomic dysfunction.                                             muscles are too weak to provide an effective cough.
                                                                   Antibiotics may be required for established respiratory
Diagnosis                                                          infection. The patient’s hydration needs to be attended to;
     Diagnosis of an individual case of GBS remains mainly         intravenous fluids may be required if oral hydration
clinical, though the introduction of the NINCDS criteria has       becomes hazardous or impossible.
helped clinicians to make a decision in difficult cases.                Pulse and blood pressure (BP) should be monitored; a
     In a typical case the patient will experience acute           mild tachycardia is common, particularly in patients who
sensorimotor      peripheral    neuropathy  with areflexia,        have mild autonomic        involvement     or have become
usually beginning in the lower limbs and ascending to              hypoxic. However, a tachycardia of more than 110/min or
involve the proximal muscles and the upper part of the             extreme lability of the BP should alert the clinician to the
body. Pain in the proximal muscle groups, particularly the         possibility that the patient has developed severe acute
thighs, is a common feature. The time course varies but            pandysautonomia which will require Intensive Care Unit
from onset to maximum severity is usually between 3 and 7          treatment.     Elderly patients,     who inevitably      have
days but can be up to 4 weeks. In elderly patients the             compromised physiological reserves in all systems, are
initial mild symptoms are more likely to be ignored or             particularly vulnerable during this acute phase of GBS and
dismissed, thus elderly patients often present later in the        are likely to develop the complications        of GBS more
 readily than their younger counterparts.                       References
     Except in very mild cases. or cases which have stopped     1. Winner SJand Grimley Evans J. Age specific incidence
 progressing before physiological      systems begin to fail,       of Guillain-Barre Syndrome in Oxfordshire.    Qart J
 plasmaphoresis should be considered. This has been                Med 1990;77(284):1297-1304.
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emerging that immunoglobulin        therapy may be squally      3. Kaplan JE, Schonberger LB, Hurwitz ES, Katona P.
effective 10and definitive studies on that treatment option        Guillain-Barre Syndrome in the United States 1978-
are likely to be published in the near future. Cortico-            1951: additional     observations   from the National
steriod therapy is not helpful 11.12. Ventilator-y failure         Surveillance System. Xeurology 1983;33:633-637.
requiring ventilatory     support tends to predict a poor       4  Arnason BGW. Acute inflammatory demyelinating
outcome 13. Plasmaphoresis has heen shown to reduce the            polyradiculopathies.   In: Dyck PJ,Thomas PK, Lambert
time spent undergoing assisted ventilation 14,15.                  EH. BunceR(eds). Peripheralneuropathy. Philadelphia:
    Once the acute illness has been treated and the patient        WB Snundrrs. 1984:2050-2099.
starts to recover, a period of format rehabilitation will       5. Passmore AP. Taylor IC, McConnell JG. Acute Guillain-
almost always be required. In elderly patients this can be         Barre Syndrome presenting as acute spinal cord
expected to be longer than in the young and it IS probably         compression      in an elderly woman. J Sot Med
best undertaken on specialised rehabilitation units for              1990;83:333-334.
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                                                                    Disorders and Stroke; ad hoc committee. Criteria for
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    In most patients recover) will occur over a period of           1735;42:1053-1057.
time though up to 85% of patients will have some residual       7 Srldharan JV. Tallis RC, Gautam PC. Guillain-Barre
deficit. Some patients die during the acute illness. and           Syndrome in the Elderly: A retrospective comparitive
some of those deaths can be avoided by careful monitoring          study. Gerontology 1993;37(3):170-175.
and aggressive treatment. Most of the persistent problems       8. Rapper AH. The Guillain-Barre Syndrome. New Engl J
are of a minor nature, though up to 10% of patients may            Med 1992;17:1130-1136.
have permanent disabling weakness.           One study has      9. Guiilain-Barre      Study Group. Plasmaphoresis and
shown that the proportion         of patients with, severe         Acute      Guillain-Barre     Syndrome.     Neurology
persistent problems is higher when definitive treatment            1984;35:1096-1104.
such as plasmaphoresis is not given. The overall median         10 Van Der Meche, Schmitz PM. Dutch Guillain-Barre
time to complete recovery is nine months 13,and despite            Study Group.         A randomized     trial comparing
their presentation with concomitant ilIness and tendency           intravenous immunogloblin and plasma exchange in
for the diagnosis to be delayed, the evidence is that elderly      Guillain-Barre         Syndrome.    New Engl J Med
patients who survive the acute episode do as well as their           1992;326:1123-1129.
younger counterparts      providing   they receive proper       11 Hughes RAC, Newsom-Davis JM, Perkin GD, Pearce
treatment. However, old age has been found to be a poor            JN. Controlled      trial of Prednisolone      in Acute
prognostic Factor during the acute phase, when compared            Polyneuropathy.    Lancet 1978;2:750-743.
with young adults below the age of 40 13.                       12 Hughes RAC. Ineffectiveness of high dose intravenous
                                                                   methylprednisolone        in Guillain-Barre   Syndrome.
Conclusion                                                         Lancet 1991;338:1142.
     The incidence of GBS increases with age and the            13 Winer JB, Hughes RAC, Osmond C. A prospective
diagnosis may not be recognised as early in the elderly as it      study of acute idiopathic neuropathy. Clinical features
is in younger people. The diagnostic criteria in young and         and their prognostic        value. J Neurol Neurosurg
old are the same, though presentation may be different,            Psychiatry 1988;51:605-612.
particularly when the clinical picture is complicated by co-    14 Guillain-Barre          Syndrome        Study     Group.
existing disease in old age. Supportive and definitive             Plasmaphoresis and acute Guillain-Barre Syndrome.
treatment should be given as early as possible to reduce           Neurology 1983; 35:1096-1104.
the severity of the illness, reduce the need for ventilatory    15 Osterman PO. Fagius J, Lundemo G. Beneficial effect
support and to reduce the level of disability, though              of plasma exchange             in acute inflammatory
elderly patients who receive active and timely treatment           polyradiculoneuropathy.      Lancet 1984; 2:1296-1299.
have a good prognosis.        The potential health gain for
elderly people with GBS who are treated actively is very
high and therefore justifies careful and aggressive

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