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 treatment. 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. frailty. 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. shown to be effective in thar it reduces the severity of an 2 Aloor M. The epidemiology of Guillain-Barre episode of GBS and speeds recovery. Evidence is Syndrome. Ann Neural 1990;27(sup): 57.80. 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. elderly patients. 6. National Institute of Neurological and Communication Disorders and Stroke; ad hoc committee. Criteria for Prognosis diagnosis of Gulllain-Barre Syndrome. Ann Neurol 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 management.