Guillain-Barre syndrome in Oxfordshire: clinical features in relation to age

Age and Ageing,  May, 1993  by S.J. Winner,  J. Grimley Evans

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Introduction

Guillain-Barre syndrome (GBS) has received little attention as a cause of peripheral neuropathy in elderly people. We have recently shown that age-specific incidence rates of GBS in Oxfordshire, UK, increase throughout adult life [1], and similar findings have been reported from other parts of the world [2-6]. Clinical features in five elderly patients with GBS [7] have been described, but there are no detailed reports comparing old and young adults.

With the development of effective therapies such as plasma exchange and intravenous immunoglobulin infusion, the clinical features of GBS are under scrutiny [8, 9]. The range of clinical features has been described in geographically-defined populations using standardized diagnostic criteria in Israel [10], USA [4], and Australia [6], but not in the United Kingdom. We have examined the clinical features of GBS and their relation to age in an epidemiologically-based retrospective study of a defined population using standard diagnostic criteria.

Methods

Methodological and epidemiological details of this study have been reported previously [1] Residents of Oxfordshire admitted to hospital with GBS between 1 January 1974 and 31 December 1986 were sought using data from the Oxford record linkage study (ORLS) [11] as a diagnostic index. Hospital medical records were retrieved. Cases were defined using diagnostic criteria for GBS formulated by the United States National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) [12], augmented firstly by the inclusion of patients with reduced rather than absent reflexes when accompanied by electrophysiological evidence suggesting demyelinating neuropathy, as in a recent clinical study [13], and secondly by including patients with the Miller-Fisher syndrome (ophthalmoplegia, ataxia, and areflexia) [14] without limb weakness, as suggested by Asbury [15]. Patients whose illness took a chronic or relapsing course suggestive of chronic inflammatory polyradiculo-neuropathy [16] were excluded.

Limb weakness was recorded using the Medical Research Council (MRC) grading system [17]. Significant disability was defined as requiring assistance from another person in one or more simple activities of daily living (as listed in the Barthel Index of activities of daily living [18] but excluding bathing). To test whether patients over 60 years of age had different clinical characteristics from younger adults (20-59 years), comparisons were made using Fisher's exact test and the [X.sup.2] test with Yates' correction; durations were compared by the Kolmogorov-Smirnov two-sample test [19].

Results

There were 72 patients, with a mean age at onset of 44.1 years (SD 21.9 range 2-85). Forty (56%) were women, and 32 (44%) were men. Ten (14%) were aged less than 20 years, 41 (57%) were 20-59 years, and 21 (29%) were 60 years and over.

Preceding events: A preceding event or illness was recorded in 47 patients (65%) within 4 weeks of the first symptom of GBS, and 53 patients (74%) within 8 weeks (Table I). More than one event or illness was recorded in three patients. An infective agent was implicated by immunological tests in ten patients: cytomegalovirus (CMV, three patients with upper respiratory symptoms, and one with headache and fever), Epstein-Barr virus (EBV, two patients), influenza (two), varicella (one) and mumps (one).

Table I. Preceding events in 53 patients with GBS
                                     Frequency of
                                      occurrence
                               0-4 weeks
                                before        5-8 weeks
                                onset         before
Upper respiratory infection      31              5
Other respiratory                 3
Gastro-intestinal                 7
Other infections                  4
Trauma or surgery                 2(*)           1(*)
Pregnant                          2([dagger])
Immunization                      1
Total (events)                   50              6
(*) One patient fell from a horse. Two patients had
surgery with general anaesthesia but no blood transfusions
(one 8 weeks before GBS onset).
([dagger]) Gestation 28 weeks and 37 weeks at onset of GBS.

Presenting symptoms: Paraesthesiae were reported as presenting symptoms by 30 patients (42%), seven of whom also had weakness, pain, or both at onset (Table II). Twenty-nine patients (40%) had pain, and in 24 (33%) this was the sole presenting symptom. Muscular weakness at presentation occurred in 28 patients (39%), and was the sole symptom in 18 (25%).

Table II. First symptoms in 72 patients with GBS
                    Paraesthesiae    Pain    Weakness
Arms                      7            2         3
Legs                      8           10        14
Both                     14            3         6
Trunk/back                            14
Face                      1                      1
Oculomotor                                       3
Bulbar                                           1(*)
Neck                                             1
Total (Patients)         30           29        28
(*) Also had weak legs.