Obstet Med 2008;1:99-101
doi:10.1258/om.2008.080011
© 2008 Royal Society of Medicine Press
Weakness in pregnancy – expect the unexpected
S Furara MRCOG *,
M Maw MBChB MRCP 
,
F Khan MRCOG * and
K Powell MRCOG 
* SPR Obstetrics and Gynaecology;
SHO Medicine;
Consultant in Obstetrics and Gynaecology, Mid Staffordshire General Hospitals, Western Road, Stafford ST16 3SA, UK
Correspondence to: Dr M Maw, 7 Charlotte Road, Edgbaston, Birmingham B15 2NQ, UK Email: montague_maw{at}hotmail.com
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Summary
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Guillain-Barré syndrome (GBS) is rare in pregnancy with
an incidence estimated to be between 1.2 and 1.9 cases per 100,000
people annually, and it is generally accepted that it carries
a high maternal risk. Delayed diagnosis is common because the
initial non-specific symptoms may mimic changes in pregnancy.
GBS should be considered in any pregnant patient complaining
of muscle weakness, general malaise, tingling of the fingers
and respiratory discomfort. This case aims to highlight the
importance of early diagnosis, allowing prompt initiation of
the immunomodulatory treatments which have been shown to improve
outcome alongside multidisciplinary care.
Key Words: Guillain-Barré syndrome • acute inflammatory demyelinating polyradiculopathy • pregnancy
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CASE REPORT
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An 18-year-old gravida 5, para 1 presented at 26 weeks of pregnancy
describing pain across her shoulders and upper back, ‘pins
and needles’ in her fingers and shortness of breath. She
had normal reflexes and, initially, there was no demonstrable
weakness. She was admitted with a provisional diagnosis of ‘hyperventilation’.
It was considered that her symptoms could be psychological given
the normal examination findings and blood tests. However, over
the next eight days she developed an ascending weakness with
speech and swallowing difficulties. She had bilateral lower
motor neurone facial weakness, bulbar speech, flaccidity in
the limbs, areflexia and flexor plantar responses. There was
reduction in all sensory modalities in her arms and legs. A
diagnosis was made of Guillain-Barré syndrome (GBS) and
this was supported by the results of a lumbar puncture: a raised
cerebrospinal fluid (CSF) protein of 1.66 g/L and a normal cell
count. She had no history of preceding illnesses and serological
tests for Campylobacter, Cytomegalovirus, Epstein-Barr virus
(EBV) and
Mycoplasma pneumoniae were negative. Antibodies to
the ganglioside GQ1b were positive. On day nine she developed
progressive respiratory failure and was moved to the critical
care unit for regular airway assessment and subsequent ventilation.
A five-day course of intravenous immunoglobulins (0.4 g/kg/day)
was commenced the same day. Episodic hypertension and tachycardia,
representing autonomic dysfunction, were treated with labetalol.
From day 16 onwards she was gradually weaned from mechanical
ventilation as her muscle power improved. Throughout her admission
regular fetal monitoring and ultrasonography demonstrated a
normally growing fetus. She was discharged from critical care
at 29 + 5 weeks gestation to a neuro-rehabilitation centre where
she made an excellent recovery. There was spontaneous rupture
of the membranes at 39 weeks, progressing to a normal vaginal
delivery of a girl weighing 3 kg with an Apgar score of eight
at one minute and nine at five minutes.
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DISCUSSION
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GBS is an acute inflammatory demyelinating polyradiculopathy
(AIDP).
2 It is thought to be immune-mediated, but its pathogenesis
remains uncertain.
5 About two-thirds of patients have had an
infection within the previous six weeks, most commonly a flu-like
illness or gastroenteritis. Implicated infectious agents include
M. pneumoniae, Campylobacter jejuni, Cytomegalovirus and EBV.
2 The preceding infection may cause an autoimmune response with
the patient's antibodies being triggered to attack various components
of the peripheral nerve myelin and sometimes the axon.
5 GBS
typically presents with pain, numbness, paraesthesia or weakness
in the limbs and this can be mistaken for a psychological complaint,
2 leading to delay in diagnosis and treatment. The interval from
onset of symptoms to diagnosis in pregnancy was reported to
be more than one week in 50% of cases in one review of 22 pregnant
patients with GBS, attributed to initial non-specific symptoms
of GBS mimicking common pregnancy complaints.
1 The diagnosis
of GBS depends on clinical criteria supported by CSF findings
and neurophysiological testing. Essential clinical criteria
are progressive motor weakness and areflexia.
6 Other features
include respiratory failure, facial nerve involvement, bulbar
and ocular nerves (in the Miller-Fisher variant), mild sensory
symptoms and autonomic dysfunction. The disease reaches its
peak at one to four weeks and then, after a variable plateau
phase, recovery occurs over weeks or months.
2 The CSF typically
shows raised protein content and a normal cell count, but it
may be normal in the first week.
1,6 Nerve conduction studies
are abnormal in approximately 90% of cases, showing multi-focal
demyelination associated with secondary axonal degeneration.
6 Mechanical ventilation may be required within 24 hours of symptom
onset. Up to 20% of patients are disabled after one year as
a result of GBS
2 and a maternal mortality of 7% has been quoted
(Table
1).
The management of GBS in pregnancy is similar to that in the
non-pregnant population
7 and includes intravenous (i.v.) immunoglobulins
and plasmapheresis. It is important that physicians and obstetricians
manage the patient jointly.
1 Ventilatory support is required
in 25–30% of non-pregnant patients,
2 but respiratory problems
may be worse in pregnancy because of splinting of the diaphragm.
8 In cases requiring ventilatory support in pregnancy, the risk
of premature birth has been noted to be greatly increased.
7 Thromboprophylaxis is indicated given hypercoagulability of
pregnancy and immobility. Routine screening for respiratory
and urinary infections is recommended. Labetalol is the agent
of choice for management of autonomic dysfunction in the gravida,
manifested by fluctuating pulse and blood pressure.
2 This drug
allows good blood pressure control without interfering with
placental or fetal blood flow.
9
A Cochrane review has shown that there are no outcome differences between i.v. immunoglobulins treatment and plasmapheresis.4 In pregnancy, the safety of i.v. immunoglobulins has been established based on its use in treating conditions, such as maternal idiopathic thrombocytopenia purpura and fetal alloimmune thrombocytopenia.1 We found 12 cases of GBS in pregnancy in which i.v. immunoglobulins have been used, with no report of treatment-induced maternal or fetal complications (Table 1). In patients who have shown no signs of recovery within two weeks, a second course of i.v. immunoglobulins has shown to be beneficial.10 The benefit of plasmapheresis is great when started within seven days of disease onset, although it still provides some advantage within 30 days.5 On grounds of equivalent efficacy, similar cost, greater convenience and ease of administration, i.v. immunoglobulins have replaced plasmapheresis to be the preferred treatment in many hospitals.11
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ACKNOWLEDGEMENT
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We would also like to thank Dr P R Daggett (Consultant Physician,
Mid Staffordshire General Hospitals) for his support in writing
this case report.
(Accepted June 10, 2008)
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Summary
CASE REPORT
