332 Letters to the Editor – Brief Communications / European Journal of Obstetrics & Gynecology and Reproductive Biology 228
Biology 228 (2018) 331–338
Guillain–Barr[25_TD$IF]é syndrome in pregnancy:
Successful multidisciplinary approach
Dear Editor,
A 30 year old primigravida presented to our emergency
department at 20 weeks gestation with rapid onset distal
paraesthesia, generalised weakness and motor difficulty. Two weeks
prior to the onset, she reported having had the influenza vaccination
as recommended in pregnancy. She had no other medical history of
note and reported no recent travel. The patient underwent various
investigations including lumbar puncture, MRI brain and spine,
blood tests including electrolytes, thyroid function, autoimmune
antibody screen, HIV, hepatitis and Epstein Barr virus (EBV); all of
which were normal. A nerve conduction study confirmed demyelin-
ation of the peripheral nerves. A diagnosis of Guillain–Barr[25_TD$IF]é
syndrome was suspected based on the clinical findings.
During admission, the patient deteriorated presenting with
respiratory muscle weakness as evidenced by reduced vital lung
capacity. She was transferred to our intensive care unit (ITU) but
was able to self- ventilate. She was commenced on a course of
intravenous immunoglobulin therapy (IVIG). She completed 5
cycles during a 10 week admission and underwent physio and
occupational therapy daily. She was transferred to a tertiary neuro-
rehabilitation centre for further supportive care at 30 weeks
gestation. Throughout the pregnancy four weekly growth and
doppler scans revealed a normal healthy fetus. The patient had an
induction of labour at 39 + 2 weeks and proceeded to have a normal
vaginal delivery. Postnatally, the patient completed weekly plasma
exchange for up to 6 weeks and continues to regain mobility
without aided support.
Guillain–Barr[25_TD$IF]é syndrome (GBS) is a rare but serious autoim-
mune disorder which affects peripheral nerve tissue. The reported
incidences of GBS in pregnancy are 1.2–1.9 cases per 100,000 [1].
The most common form of the disease is an acute inflammatory
demyelinating polyradiculoneuropathy (AIDP), which presents as
progressive symmetric motor weakness, usually beginning in the
legs and advancing proximally [2]. Two-thirds of patients present
with gastroenteritis or influenza like symptoms 4–6 weeks prior to
onset of symptoms [2].
GBS in pregnancy is associated with significant maternal
morbidity as 3% of patients die secondary to respiratory failure.
Perinatal morbidity is also associated with risk of pre-term labour.
In non- pregnant patients 25–30% require ventilatory support. This
figure may increase in pregnancy because of increased demand for
maternal metabolic processes and reduced lung volume and
capacity [3].
Pregnancy often results in remission of immune mediated
diseases. This has been explained by the predominance of Th2
helper cells which secrete anti-inflammatory non -cytotoxic
cytokines over Th1 cells, which secrete pro-inflammatory
cytotoxic cytokines during pregnancy [4]. There is also a
significant increase in GBS relapse two weeks post partum due
to return of cellular immunity and an increase in delayed type
hypersensitivity [4].
Treatment in pregnancy is supportive with IVIG or plasmapheresis,
which is associated with an outcome of full recovery in 70–80% of
patients [5]. Close fetal monitoring and progression of disease will
dictate timing of delivery. If the patient presents with worsening
hemodynamic stability, the need to expedite delivery may be
necessary. The clinician must outweigh the risk of maternal mortality
with risk of pre-term delivery of the fetus. GBS itself is not an indication
for caesarean section. Literature review reports cases of successful
vaginal, instrumental and [26_TD$IF]4caesarean deliveries with GBS [5].
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The approach to management in pregnancy is multi-disciplin-
ary. One has to consider mechanical ventilation, treatment of
underlying infective sources, pain control in addition to monitor-
ing of the fetus and decision for timing and mode of delivery. A high
index of suspicion for early diagnosis and prompt intensive
multidisciplinary care can improve the prognosis for both the
mother and the fetus.
Conflicts of interest
None.
References
[1] Zafar MS, Naqash MM, Bhat TA, Malik GM. Guillain–Barre syndrome in
pregnancy: an unusual case. J Fam Med Prim Care 2013;2(January (1)):90–1.
[2] Vasudev R, Raina TR. A rare case of Guillain–Barre syndrome in pregnancy
treated with plasma exchange. Asian J Transfus Sci 2014;8(January (1)):59–60.
[3] Gupta R, Chhabra B, Senthilnathan T, Bharadwaj M, Ghei A, Thakur A. Critical
care of a pregnant patient with Guillain–Barré syndrome. Indian J Anesth
2003;47:50–2.
[4] Meenakshi-Sundaram S, Swaminathan K, Karthik SN, Bharathi S. Relapsing
Guillain–Barre syndrome in pregnancy and postpartum. Ann Indian Acad
Neurol 2014;17(July (3)):352–4.
[5] Zilberlicht A, Yonai N, Cohen K, Bardicef M. Gullian–Barre syndrome in
pregnancy—a case report and review of the literature. Gynecol Obstet
(Sunnyvale) 20166(348) 2161–0932.10003.
L.S. Kasaven*
C. Basu
Kent University Hospital, William Harvey, Kennington Rd.,
Willesborough, Ashford TN24 0LZ, United Kingdom
* Corresponding author.
E-mail address: lk226@doctors.org.uk (L. Kasaven).
Received 10 April 2018
http://dx.doi.org/10.1016/j.ejogrb.2018.06.017
Autosomal recessive polycystic kidney disease
prenatally diagnosed in a fetus with unreported
paternal inherited PKHD1 mutation
Autosomal recessive polycystic kidney disease is caused by
mutations in PKHD1, a large 500 Kb gene [1] with a complex
splicing pattern located on chromosome 6p21.1-p12. The product
of PKHD1, fibrocystin/polyductin (FPC), is a single-membrane
spanning protein with multiple isoforms [2]. It is expressed
predominantly in the kidney, liver and pancreas. The exact
function of FPC remains unclear. To date, >300 pathogenic
mutations have been cataloged in the ARPKD mutation database
(http://humgen.rwth-aachen.de) [3].
The patient is a 24 year old gravid 1 who presented at 28 weeks
and 2 days for obstetrical control. She and her husband were
healthy and nonconsanguineous. No significant history of renal
disease was identified. Current pregnancy began uneventfully until
28 weeks when ultrasound revealed enlarged ecogenic fetal
Kidneys and oligo-anhydramnios (Fetal kidneys of 61  28 and
59 x 26 mm). Pulsatility index of renal arteries was [15_TD$IF]1,658 and 1,873.
Lose of cortex-medullar differentiation was almost ascertains.
Small pericardial effusion was present. The ultrasound findings
were suspicious for autosomal recessive polycystic kidney disease
(ARPKD). The patient and her husband have ultrasound normal
kidneys. The couple chose end pregnancy in base of the worse
prognosis of gestation. Diagnostic cordocentesis was done and