Co-morbid Guillain-Barré syndrome and acute
Reema R. Mohammed, MBBS, Mohammed M. Jan, MBChB, FRCPC.
لبلحاو غامدلاب ددعتلما دالحا باهتللااو هيراب ينلوج ةمزلاتم
غامدلا يف ءاضيبلا ةدالما نابيصي ينفلتخم ينضرم امه ىكوشلا
باهتلا دعب ثودلحاو يتاذلا يعانلما ببسلا ىف ناكرتشي نكلو
ملو ًاردان ًايوس ناضرلما ناذه ثودح ربتعيو .ميعطت وأ ىسوريف
ةلاح ريرقتلا اذه يف مدقنو .ًادج ةليلق تلااح يف لاإ هفصو متي
عم داح يعابر للشب تبيصأ ماوعأ ةرشع رمعلا نم غلبت ةلفط
دقو .لوبلاب سابتحاو تلاضعلل ةيبصعلا ةباجتسلإا يف مادعنا
ةبسنو ايلالخا ددع ىف ددعتم عافترا يغامدلا لئاسلا ةنيع تنيب
ىعانصلا سفنتلازاهج ىلع اهعضو ىلإ تجاتحا دقو .ينتوربلا
قيرط نع ةعانلما تاداضم جلاع اهئاطعا مغر اهتلاح نسحتت ملو
ةجودزم ةيرصب ةيئورب باصتل ًاقحلا اهتلاح تروطت لب ديرولا
تاريغت غامدلل ىسيطانغلما يننرلا ةعشأ تنيب دقو .ىعولاب ةلقو
ددعتم داح باهتلا دوجو ىلع تلد ءاضيبلا ةدالماب ةددعتم
دكأتلاو ةلالحا فصوو روطت ىلع ًءانبو .ىكوشلا لبلحاو غامدلاب
باهتلا عم هيراب ينلوج ةمزلاتبم اهصيخشت تم دقف صيخشتلا نم
دعب ةلالحا تنستح دقو .ىكوشلا لبلحاو غامدلاب ددعتم داح
جتنتسن اذل .ديرولا قيرط نع نولوسينديرب ليثيلما جلاع ءاطعا
غامدلاب ددعتلما دالحا باهتللاا عم هيراب ينلوج ةمزلاتم ثودح نأ
اهصيخشت مهلما نم ةديدشو ةردان ةلاح يه ىكوشلا لبلحاو
.اهئافشو ةلالحا نستح مث نمو ةعرسب بسانلما جلاعلا يمدقتل
clinically distinct demyelinating disorders that share
an autoimmune pathogenesis and prior history of
viral infection or vaccination. Concurrent GBS and
ADEM are uncommon with few reported cases. Our
patient is a 10-year-old girl who presented with acute
quadriparesis, areflexia, and urinary retention. Lumber
puncture revealed mild pleocytosis and elevated
protein. She required mechanical ventilation and
failed to improve after intravenous immunoglobulins.
She subsequently developed double vision and
disturbed level of consciousness. Brain MRI revealed
multiple white matter lesions suggestive of ADEM.
Based on the temporal association and exclusion
of alternative etiologies, we made a diagnosis of
(GBS) and acute
Disclosure. The authors declare no conflicting interests,
support or funding from any drug company.
acute post-infectious, inflammatory, demyelinating
neurological disorders.1,2 Guillain-Barré syndrome
affects the peripheral nervous system, while ADEM
affects the CNS. Molecular mimicry and cross-reactive
immune response is considered to play a crucial part in
the pathogenesis of GBS.1,2 The ADEM pathogenesis is
thought to be a result of a T cell mediated autoimmune
response to myelin basic protein, which is triggered by an
infection or vaccination. Although GBS and ADEM are
clinically distinct, they share several features including
an autoimmune pathogenesis, myelin injury, and prior
history of viral infections or vaccination. However,
concurrent GBS and ADEM affecting both the
peripheral and CNS simultaneously is an uncommon
situation, and only few cases have been reported.3-5 Our
disseminated encephalomyelitis (ADEM) are
syndrome (GBS) and acute
GBS and ADEM. She improved remarkably after
intravenous methylprednisolone. We conclude that
co-morbid GBS and ADEM is an uncommon entity
presenting with severe neurological morbidity. Prompt
recognition and treatment can hasten the recovery
and therefore improve the neurological outcome.
Neurosciences 2013; Vol. 18 (2): 166-168
From the Department of Pediatrics, Faculty of Medicine, King
Abdulaziz University, Jeddah, Kingdom of Saudi Arabia.
Received 13th September 2012. Accepted 3rd January 2013.
Address correspondence and reprint request to: Dr. Mohammed M.
S. Jan, Professor and Consultant of Pediatric Neurology, Department
of Pediatrics, Faculty of Medicine, King Abdulaziz University,
PO Box 80215, Jeddah 21589, Kingdom of Saudi Arabia.
Tel. +996 (2) 6401000 Ext. 20208. Fax. +996 (2) 6403975.
Neurosciences 2013; Vol. 18 (2) www.neurosciencesjournal.org
Neurosciences 2013; Vol. 18 (2)
Co-morbid GBS and ADEM … Mohammed & Jan
objective is to present a 10-year-old girl who presented
with features of GBS associated with later development
of ADEM. The few reported cases and related pediatric
literature will be reviewed.
Case Report. A previously healthy 10-year-old girl
presented to the emergency room of King Abdulaziz
University Hospital in Jeddah, Saudi Arabia, with a
2-day history of generalized weakness, low-grade fever,
and urinary retention. Ten days earlier, she had a mild
upper respiratory tract infection (URTI). Her medical,
family, and social histories were unremarkable. On
examination, her vital signs were stable and she was
conscious with no meningeal signs. Neurologically,
she had severe quadriparesis that was worse in the
lower limbs with inability to sit or stand. Her reflexes
were absent with negative Babinski. No sensory
abnormalities were noted. Her pupils were equal and
reactive to light. Her urinary bladder was palpable,
and the remaining systems were unremarkable. Initial
investigations included a normal complete blood count,
liver, and renal profiles. Her erythrocyte sedimentation
rate was 50 mm/hr and C-reactive protein was 5.3
mg/L. She was started on broad-spectrum antibiotics
and Acyclovir (Teva Pharmaceutical, USA). Initial
non-contrast brain CT scan was normal. Lumber
puncture (LP) revealed mild pleocytosis (14 cells/
cubic mm), mainly lymphocytes, with elevated protein
and negative gram stain. The next day her weakness
worsened quickly with respiratory distress and double
vision. On examination, she was confused with
nystagmus and generalized paralysis. She was placed
on mechanical ventilation and started on intravenous
immunoglobulins IVIG (Biotest Pharmaceuticals, New
York, NY, USA) of a total dose of 2 gram/kg. On follow
up examination, she was noted to have facial diplegia,
bulbar palsy, and features of right-sided sixth nerve
palsy with normal fundal examination. Over the next
few days, she remained confused and her weakness
showed no significant improvement. Brain and spinal
MRI showed multiple white matter lesions with high
signal intensity involving the brain and brainstem,
suggestive of ADEM (Figures 1 & 2). Viral studies came
back negative including human immunodeficiency
viral testing. Repeated LP was attempted and failed.
Intravenous methylprednisolone (Sandoz, Annapolis,
MD, USA) was started on day 6 of admission at a dose
of 15 mg/kg/day for 3 consecutive days. She improved
rather quickly and was extubated within the next week.
She was shifted to the pediatric ward 3 weeks after initial
presentation with slowly improving muscle power. She
received extensive physiotherapy and was discharged
home ambulating after spending a total of 5 weeks in
Discussion. We present an unusual case with GBS
and subsequent features of ADEM. The presenting
history was classic for GBS with preceding URTI and
severe lower motor neuron weakness that progressed
to involve the respiratory and bulbar muscles.
Guillain-Barré syndrome is an acute demyelinating
polyradiculoneuropathy that is characterized by
progressive ascending muscle weakness and areflexia.
Immune-mediated mechanisms due to molecular
mimicry are considered to be responsible for the
pathogenesis of GBS. Our patient failed to improve
Figure 1 - Brain MRI showing a white matter lesion with high signal
intensity on T2 Flair.
Figure 2 - Another MRI cut showing multiple white matter lesions
with high signal intensity suggestive of acute disseminated
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Co-morbid GBS and ADEM … Mohammed & Jan
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on IVIG and subsequently developed disturbed
level of consciousness and double vision suggesting
an encephalitic picture. The findings on LP and
MRI were suggestive of ADEM. Acute disseminated
encephalomyelitis (ADEM) is a monophasic syndrome
occurring in the context of an infection, immunization,
or vaccination. It typically follows a prodromal phase
of 1-4 weeks followed by the development of multiple
white matter lesions in the brain, brainstem, and
or spinal cord. Acute urinary retention is common
in ADEM as seen in our patient. Successful use of
high-dose steroids has been widely reported to provide
marked improvement of the symptoms, which was
also the case in our patient.6 Acute disseminated
encephalomyelitis usually has a favorable long term
neurological outcome; however, recent literature
suggests that a significant proportion of patients with
ADEM will later develop multiple sclerosis. Follow up
experience from developing countries does not support
this view, and therefore further long term research is
necessary to better describe this entity and its prognosis.
Simultaneous GBS and ADEM have been rarely
reported in the pediatric literature. Patients may start by
manifesting GBS, with brain MRI showing features of
ADEM, while others may start with features of ADEM
and then develop GBS during their hospitalization.
Occasionally, patients may demonstrate transverse
myelitis that evolves into GBS and associated with
demyelination on brain MRI.7 Combined ADEM
and acute motor axonal neuropathy has been rarely
reported.8 The response of these cases to high dose IVIG
and steroid treatment has been variable. In our patient,
no clinical response was noted after IVIG therapy and
rapid improvement of the neurological symptoms was
only noted after pulse steroids. It is possible that the
response is the result of the cumulative effects of both
treatments as they were given in sequence.
The pathogenesis of both GBS and ADEM is
thought to be due to autoimmunity to myelin protein
antigens; however, several authors proposed that they
may represent a continuous clinical spectrum resulting
from an antibody-mediated post-infectious syndrome.9
This concept suggests a single autoimmune disease
involving both the peripheral and CNS. Based on this
hypothesis, the simultaneous occurrence of GBS and
ADEM suggests that the responsible pathogen share
an antigen of both peripheral and central myelin.
Others authors suggested that the immune response
against a component of the myelin of the CNS may
carry cross-antigenicity with the peripheral system. A
significant increase in activated and helper inducer T-
cells was observed in both GBS and ADEM suggesting
a common pathogenic mechanism. This combined
clinical entity represents a generic acute immunological
nervous disease with various clinical presentations.
We conclude that co-morbid GBS and ADEM is
an uncommon entity generally presenting with severe
neurological morbidity; however, prompt recognition
and treatment can significantly hasten the recovery
and therefore may improve the neurological outcome.
Further research of this unusual association and its
pathogenesis is necessary to help understand its clinical
course and prognosis.
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