journal

1. CASE REPORT
Good maternal and fetal outcomes of predominantly sensory
Guillain–Barre´ syndrome in pregnancy after intravenous
immunoglobulin
Yasuo Matsuzawa • Ryuji Sakakibara •
Toshiko Shoda • Masahiko Kishi • Emina Ogawa
Received: 14 August 2008 / Accepted: 28 October 2009 / Published online: 25 November 2009
Ó Springer-Verlag 2009
Abstract Guillain–Barre´ syndrome (GBS)-complicating
pregnancy is rare; and it is generally believed that it carries
a high maternal risk. We reported a case of good maternal
and fetal outcomes of predominantly sensory GBS (gen-
eralized pain and numbness, mild deep sensory ataxia,
positive serum IgM anti-GalNAcGD1a antibody)-compli-
cating pregnancy after intravenous immunoglobulin
treatment.
Keywords Guillain–Barre´ syndrome Á Pregnancy Á
Intravenous immunoglobulin
Introduction
Guillain–Barre´ syndrome (GBS)-complicating pregnancy
is a relatively rare event [1]. It is generally believed that it
has a high maternal risk. Based on this belief, termination
of pregnancy had been carried out whereas its effect on the
maternal GBS remained obscure [1, 2]. In contrast, inten-
sive immune therapy including plasmapheresis and intra-
venous immunoglobulin (IVIG) might improve the course
of GBS in pregnancy [1, 3]. Here, we add a case of good
maternal and fetal outcomes of predominantly sensory
GBS-complicating pregnancy after IVIG treatment.
Case report
A 38-year-old woman had a 5-year history of Hashimoto’s
thyroiditis that required 75 lg/day of levothyroxin sodium.
She was pregnant at the end of the second trimester. She
developed acute flu-like symptoms. One day after onset,
she suddenly experienced extreme pain and numbness in
the face, neck, back and extremities. On day 7, she noticed
right facial weakness, whereas she had no weakness in the
arms, fingers, legs and feet at all. On day 8, the patient was
referred from a gynecological clinic. On admission to our
hospital, she was chair-bound because of severe sponta-
neous pain. However, she was alert and well oriented. She
had right side-dominant facial diplegia, but other cranial
nerves were normal. She had mildly brisk deep tendon
reflexes in all four limbs, whereas the plantar responses
were flexor. Pinprick and touch sensation was preserved in
all parts of her body. However, she had paresthesia that
was evoked by pinprick in the distal parts of all four
extremities. Whereas she had normal joint position sensa-
tion in all four extremities, she had mildly staggering gait.
She had mild neck stiffness but without positive Kernig
sign. She had no postural hypotension or urinary dys-
function. Routine laboratory tests showed no increase in
virus titers or autoantibodies against collagen diseases,
whereas increased serum IgM anti-GalNAc-GD1a gangli-
oside antibody was found. She had no coagulopathies. The
cerebrospinal fluid examination showed an increased total
protein of 100 mg/dl (normal 40) but normal cell count
of 3/mm3
. Brain MRI was normal. A nerve conduction
study on day 10 revealed increased distal motor latency,
decreased nerve conduction velocity, and decreased com-
pound motor action potential amplitudes in the right
median [5.1 ms (normal limit 4.5 ms), 47.6 m/s (normal
limit [48 m/s), 1.53 mV (normal limit [4.4 mV)], ulnar
Y. Matsuzawa Á T. Shoda
Respiratory Diseases Division, Department of Internal Medicine,
Sakura Medical Center, Toho University, Sakura, Japan
R. Sakakibara (&) Á M. Kishi Á E. Ogawa
Neurology Division, Department of Internal Medicine,
Sakura Medical Center, Toho University, 564-1 Shimoshizu,
Sakura 285-8741, Japan
e-mail: sakakibara@sakura.med.toho-u.ac.jp
123
Neurol Sci (2010) 31:201–203
DOI 10.1007/s10072-009-0188-6

2. [4.4 mV (normal limit [4.6 ms)], and peroneal nerves
[10.2 ms (normal limit 6.2 ms)]; and decreased sensory
nerve action potential amplitudes in the right median
[7.5 lV (normal limit [11.1 lV)], and ulnar nerves
[6.5 lV (normal limit[7.8 lV)], the findings suggestive of
a demyelinating nature of her sensory dominant neuropa-
thy. The clinical, CSF, and electrophysiological features
favored the diagnosis of predominantly sensory GBS [4–6].
Our patient wished to continue her pregnancy. With her
informed consent, a 5-day course of treatment with IVIg
(400 mg/kg body weight/day) was initiated, which ame-
liorated all her neurological abnormalities. Careful gyne-
cological assessment of the patient and the fetus was
completely normal after administration of IVIg. No coag-
ulopathy or renal dysfunction appeared after IVIg. On day
24, she was discharged from our hospital. At a follow-up
examination 3 months after her first visit, she could walk
without difficulty, had no pain or facial weakness, and her
deep tendon reflexes became mildly decreased. The follow-
up nerve conduction study revealed almost normal findings
(Table 1). Four months later, she could have a healthy baby
at a full term.
Discussion
The diagnosis of predominantly sensory GBS in this case
was based on (1) clinical features of acute onset of severe
generalized pain and numbness, mild deep sensory ataxia,
which progressed rapidly, while motor function was almost
completely spared except for facial diplegia, (2) the CSF
finding of cyto-albumino dissociation, and (3) electro-
physiological features of a demyelinating sensory domi-
nant neuropathy. Electrophysiological data of our case
seemed to conform to those of previously reported ‘sensory
GBS’ cases; which range from motor and sensory nerve
involvement [4–6] to pure sensory nerve involvement
[7, 8]. Although she had mildly brisk deep tendon reflexes
initially, she lacked any evidences of myelitis such as a
sensory level or urinary dysfunction. Brisk deep tendon
reflexes have been reported in some patients with GBS
[9, 10].
Predominantly sensory GBS is rare [5, 6]; therefore
specific aetiologies remain unknown. However, a sensory
ataxic form of GBS overlapping with Miller Fisher syn-
drome was previously described in a patient with IgG type
anti-GD1b antibody after Campylobacter jejuni infection
[11]. In our case, positive IgM anti-GalNAcGD1a antibody
in the serum was noted. Previously, antibodies to GM1,
GM1b, GD1a, and GalNac-GD1a are in particular impli-
cated in acute motor axonal neuropathy (AMAN) subtype
GBS and, with the exception of GalNacGD1a, in acute
motor and sensory axonal neuropathy (AMSAN) subtype
GBS [12]. Since a demyelinating nature of sensory domi-
nant neuropathy in our case does not conform to that of
AMAN or AMSAN, the role of positive serum IgM anti-
GalNAcGD1a antibody remains uncertain.
GBS-complicating pregnancy is rare [1]. Previously,
termination of pregnancy has been carried out whereas its
effect on the maternal GBS remains obscure [1, 2]; some
reports showed a good outcome [1, 13] while others did not
[1, 10]. Since our patient wished to continue her pregnancy,
with her informed consent, we started her on IVIg. IVIg is
known to have several adverse effects [14, 15]; one of the
most common is aseptic meningitis. In addition, arterial or
venous thrombosis due to a rise in plasma viscosity,
including pulmonary embolism and stroke, are potential
adverse events of IVIg, the reported rates being as high as
Table 1 Nerve conduction study result on days 10, 24 and after 3
months
Day 10 Day 24 3 months Normal
limit
Motor nerves
Right median (APB)
CMAP (mV) 1.53 3.27 5.53 4.4
DML (ms) 5.04 6.8 3.96 4.5
NCV (m/s) 47.6 57.3 55.1 48
Right ulnar (ADM)
CMAP (m/V) 4.4 6.07 6 4.6
DML (ms) 2.31 2.52 2.08 3.5
NCV (m/s) 51.8 59 60.7 48
Right peroneal (EDB)
CMAP (m/V) 1.93 1.33 1.6 1.2
DML (ms) 10.2 5.96 4.92 6.2
NCV (m/s) 48.7 55 50.4 39
Right tibial (AH)
CMAP (mV) 8.67 9 not performed 5.8
DML (ms) 6.12 4.98 5.6
NCV (m/s) 48.7 47.3 38
Sensory nerves
Right median
SNAP (lV) 7.53 13.3 10.6 11.1
NCV (m/s) 50 54.6 47.1 44
Right ulnar
SNAP (lV) 6.5 14 15.3 7.8
NCV (m/s) 48.9 43.2 58.5 40
Right Sural
SNAP (lV) 4.33 4.66 not performed 3
NCV (m/s) 39.2 44.1 40
The values in bold indicate abnormal values
CMAP compound muscle action potential, DML distal motor latency,
SNAP sensory nerve action potential, APB abductor pollicis brevis,
ADM abductor digiti minimi, EDB extensor digitorum brevis, AH
abductor hallucis
202 Neurol Sci (2010) 31:201–203
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3. 1.1–4.5% [14, 15]. Acute renal tubular necrosis is also
reported to occur with IVIg therapy [14, 15]. Since preg-
nancy leads to mild coagulopathy and renal dysfunction,
these disorders should be checked extensively. However,
our patient developed no coagulopathy or renal dysfunction
after the initiation of IVIg. In contrast, our patient achieved
good maternal outcome of predominantly sensory GBS
(complete resolution within 1 month) and fetal outcome
(normal delivery at full term). This is in line with previous
observations that none of GBS cases in pregnancy had any
IVIg-induced fetal complication [1]. From above obser-
vations, including ours, active treatment with the use of
IVIg can be a safe and recommended method in the man-
agement of GBS in pregnancy.
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