Guillain–Barrè syndrome

Guillain–Barrè syndrome has an incidence of about 0.01 per 1000 persons per year (Bak, 1985). About 35% will have
respiratory insufficiency and 17% will require ventilatory support. One recent study following up patients at a median 7 years
after onset found that 73% were symptom free. About one-fifth have some disability (Vedeler, Wik, & Nyland, 1997).

Plasmapheresis and total lymphoid irradiation. Plasmapheresis is an effective means of removing antibodies and the relative
success of such treatment in Guillain–Barré syndrome and myasthenia gravis gives plasmapheresis a reasonable theoretical
case in the treatment of multiple sclerosis. However, the results are conflicting. Some authors have found that long-term
plasmapheresis, in combination with other immunosuppressive drug regimens (particularly cyclophosphamide and
prednisolone), has produced considerable benefit in chronic progressive multiple sclerosis (Khatri, 1988). Other authors are
less enthusiastic (Tindall, 1988). Once more, no clear conclusion can be reached at the present time. A meta-analysis of
clinical studies of the efficacy of plasma exchange did find a significant reduction in the proportion of people who
experienced neurological decline at 12 months follow-up (Vamakas et al., 1995). However, the authors still concluded that
further clinical research was needed to refine the place of plasma exchange in the overall management of multiple sclerosis.
The same conclusion can be drawn from studies on total lymphoid irradiation. After initial enthusiasm more recent
work has shown modest benefit (Cook et al., 1995) or no benefit (Wiles et al., 1994). The former study indicated that adding
lowdose prednisolone could enhance beneficial effect following total lymphoid irradiation.

Guillain–Barré syndrome (GBS)

GBS or acute inflammatory polyradiculoneuropathy (AIP) produces a variable degree of acute or subacute quadriparesis; this
is frequently severe. GBS affects between 0.75 and 2.0 persons per 100,000 per year (Ropper, 1992). These incidence figures
have more recently been confirmed in theUK(Rees et al., 1998). Patients often become unable to walk and there can be
associated respiratory, bulbar, and autonomic failure. Admission to an intensive therapy unit is sometimes needed and about
20% of patients require artificial ventilation (Ropper, 1992). In a UK series prior to the use of immunomodulatory treatment
(Winer et al., 1988), 13% of patients died and, at 12 months, 20% were left significantly disabled, whereas 67% had
recovered completely; 88% lost the ability to walk at the height of the illness. Plasma exchange (Ropper, 1992) or
intravenous IgG (Van Der Meche et al., 1992) effectively attenuate the severity of GBS if commenced within 2 weeks of the
onset. Such therapy is indicated in patients who are unable to walk or have significant bulbar or respiratory weakness.
Intravenous IgG infusion is currently the preferred treatment because of the ease of application (Hahn, 1998). In a recent UK
study, 68% of patients with GBS had immunomodulatory treatment. At 1 year, 8% of all patients had died, 62% had made a
complete or almost complete recovery, 4% remained bedbound or ventilator dependent, 9% were unable to walk unaided, and
17% were unable to run (Rees, 1998).
This highlights the fact that a significant minority of patients remain disabled despite immunomodulatory treatment
and require continuing rehabilitation. However, many aspects of the management in both the acute and more chronic stages
require a multidisciplinary approach, which is applicable to all patients with GBS.
At an early stage, various prognostic factors can be used to predict the likely degree and duration of disability
(Winer et al., 1988). Older age, small compound muscle action potentials on nerve conduction tests, inability to walk within 4
days, onset after campylobacter infection (Rees et al., 1995), or the need for artificial ventilation all predict a poor outcome.
Reliable and reproducible functional measures of severity are useful; a lack of demonstrable improvement in the first few
weeks suggest a poor outlook with prolonged or permanent disability.
From the outset, high priority must be given to the prevention of complications if long-term disability is to be
minimised. In severely affected patients, close monitoring of respiratory, cardiovascular, and bulbar function (on an intensive
therapy unit) is vital to prevent potentially lethal complications. Attention to positioning, posture, bowel and bladder
function, and chest physiotherapy can prevent pressure ulcers, peripheral nerve damage, constipation, urinary infection, and
chest infections. Passive limb movements and moulded limb splints can avoid contractures. In this regard, the use of a tilt
table to prevent contractures should be supervised closely because of the dangers of severe postural hypotension in patients
with autonomic involvement. Compression stockings (and probably subcutaneous heparin) are aimed at the prevention of
venous thromboembolism. Pain, perhaps mediated by nervi nervorum in inflamed nerve roots (Thomas, 1979), can be severe
and should be borne in mind, especially in the ventilated patient. It can be resistant to all treatments apart from intrathecal
opioids. Attention to positioning and limb support, passive movements, cold or heat, vibration or transcutaneous neural
stimulation (TNS) can be helpful.
The psychological impact of GBS can be devastating (Rice, 1977) and depression is common (Ropper, 1992).
Reassurance, explanation, and attention to a frequently deranged sleep pattern are important. Support from ex-patients can be
enlisted from the local branch of the Guillain–Barré Support Group (see the section useful addresses at the end of the
chapter). During recovery a full range of movement should be maintained in all joints.
Muscle strengthening will initially involve movements that are assisted and can exclude the effect of gravity. Hydrotherapy
can have a place here. Later, active movement against gravity will target trunk and head control, sitting, unsupported,
kneeling, standing from sitting and walking. In most patients, the use of special seating (including head support), walking
aids, and functional splinting will be more or less temporary. Activities of daily living will be gradually reintroduced.
Graduated strengthening exercises, and exercises to improve balance and coordination will also be started at around this time.
The relative value of the various techniques in use is entirely unexplored.
Patients often fatigue early: both exercise regimes and a patient’s daily routine must take this into account. Fatigue
can continue even when power has returned to normal on clinical testing. This can be shown to be due to residual motor,
rather than psychological, factors by myometry (Nicklin et al., 1987).

Reference:

Greenwood, R., Barnes, M.P., McMillan, T.M., & Ward, C.D. (2003). Peripheral Nerve Disorders. In Handbook of
Neurological Rehabilitation (2nd ed.) (pp.10, 539, 697-698). New York, NY: Psychology Press