Guilian Sun

Zhiliang Yang

BMC Ophthalmology

Springer - Publisher Connector

CASE REPORT Open Access
Bilateral abducens nerve palsy in an infant
case of fulminant acute disseminated
encephalomyelitis: a case report
Zhiliang Yang* and Guilian Sun
Abstract
Background: Sixth (abducens) nerve palsy (ANP) is far less frequent in children and has not been reported as a
sign of acute disseminated encephalomyelitis (ADEM). We present an infant case of ADEM with bilateral abducens
nerve palsy (BANP).
Case presentation: We report one case of BANP in a 15-month-old boy of fulminant ADEM. The patient
underwent physical examinations and brain MRI scan three times during about six months follow-up. The patient
had BANP and developmental regression when he regained consciousness from a coma, and the signs had
persisted for 6-months.
Conclusions: BANP can be a symptom of ADEM.
Keywords: Fulminant acute disseminated encephalomyelitis, Abducens nerve palsy, Case report, Infant
Background
Acute disseminated encephalomyelitis (ADEM) is an
autoimmune inflammatory disorder of the central nervous
system. The etiopathogenesis is thought to be immune-
mediated. It mostly follows an antecedent infection and,
rarely, an immunization [1]. The average age of onset is
between 6 and 8 years and the disease is much less com-
mon in children younger than 2 years [2]. Clinical features
are manifold and not pathognomonic.
Sixth (abducens) nerve palsy (ANP) is far less frequent
in children. Common causes include trauma, raised
intracranial pressure, and neoplasms [3]. Here, we report
a rare case of ADEM in a 15-month-old boy who fell
into a coma after a seizure. He had bilateral abducens
nerve palsy (BANP) and developmental regression when
he regained consciousness. To our knowledge, no cases
of ADEM with BANP have been reported.
Case presentation
A 15-month-old normal developmental Chinese boy was
brought to our hospital with the chief complaints of
having a fever for 3 days in the morning, and then one
episode of seizure occurred in the afternoon. After the
seizure stopped, he fell into a coma. He was evaluated
and the Glasgow coma score (GCS) was 3/15 (E1V1M1).
On examination, his pupils were small, the pharyngeal
reflex, the pupillary, corneal, and orbital reflexes were
absent. No meningeal sign was present. He had been
given with intravenous antibiotic (ceftriaxone) at the
clinic before hospitalization. He was first diagnosed with
encephalitis.
A brain MRI scan was also performed urgently. The
MRI images showed inhomogeneous areas of increased
signal in T2-weighted images in the dorsal part of pons,
midbrain, medulla, bilateral thalamus, and right frontal
cortex and left cerebellar hemispheres (Fig. 1-1). An
electroencephalogram revealed generalized cerebral
dysfunction with no definitive irritable foci. Initial in-
vestigations revealed a white blood count of 3.4 × 109/L
(4-10 × 109/L), hemoglobin of 125 g/L (120–140 g/L),
platelet count of 180 × 109/L (10-30 × 109/L), C-reactive
protein of 16.7 mg/dL (0–8 mg/dL), ceruloplasmin of
260 mg/L (220–330 mg/L), and lactic acid level of
7.0 mg/L (3–10 mg/dL). Fundoscopy was normal and the
opening pressure on lumbar puncture was 55mmH2O.
Lumbar puncture revealed that the cerebral spinal fluid
* Correspondence: sizhewujiu@163.com
Department of Pediatrics, The First Affiliated Hospital of China Medical
University, No. 155 Nanjing North Street, Heping District, Shenyang 110001,
Liaoning, China
© The Author(s). 2016 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0
International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and
reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to
the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver
(http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
Yang and Sun BMC Ophthalmology  (2016) 16:185 
DOI 10.1186/s12886-016-0365-3
(CSF) cell count was 3 × 106/L cells (67 % lymphocytes,
33 % neutrophils), protein was 604 mg/L, and glucose was
3.4 mmol/L (capillary blood glucose was 4.1 mmol/L), and
chloride was 114 mmol/L. Tests of immunoglobin M for
coxsackie virus, herpes virus, cytomegalovirus, toxoplas-
mosis gondii, rubella virus, adenovirus, respiratory syncyt-
ial virus, and Mycoplasma pneumoniae were negative. The
diagnosis of ADEM was considered.
He was started on intravenous methylprednisolone
(250 mg, about 25 mg/kg per day) pulse therapy for 3 days
and intravenous immunoglobin (IVIG; 5 g per day, about
400 mg/kg per day) for 5 days. Supportive therapies such
as nasal feeding and neurotrophic drugs were applied.
Hypodermic injection of interferon-α1b was also applied.
The methylprednisolone pulse therapy was followed with
intravenous methylprednisolone 20 mg/day (about 2 mg/
kg per day) because he was unable to take prednisone or-
ally. He showed a good response. On day 3 after starting
methylprednisolone pulse therapy and IVIG, he cried and
opened his eyes when a subcutaneous injection was given.
The GCS was 7/15 (E4V2M1). On day 5, the GCS was 10/
15 (E4V2M4). His eyeballs could move up and down, but
not left and right following a light, without the presence of
nystagmus, BANP was suspected. He could follow what
his parents said to him and looked in the direction of their
voices. however, he was too young to answer some ques-
tions. A neurological examination revealed that he could
not control his head well.
On day 20, the power in his upper and lower limbs
were about IV/V, and he could control his head better,
turn over, and crawl a little, but he could not sit or stand
unsupported. The MRI imaging of brain and spinal cord
were checked. Increased signal in T2-weighted images
(Fig. 1-2) was still observed in the bilateral thalamus, the
dorsal part of pons, midbrain still, and no abnormal sig-
nals were seen in the spinal cord.
The boy was discharged on day 22. At the time of dis-
charge, he was clear consciousness, with a GCS of 11/15
(E4 V2 M5). Oral prednisone 5 mg was given twice daily
for about one month, then tapered to 5 mg once daily
for about one month and finally to 5 mg every two days.
Three months after the onset, he could sit without
support but still could not walk without assistance. He
also showed ataxia when he tried to grab something
with his hands. Both eyes showed esotropia and could
not abduce horizontally, which was compatible with the
lesion in the the dorsal part of pons in images, and ac-
quired central BANP was confirmed. At the 6-month
follow-up, the MRI images (Fig. 1-3) showed hypoin-
tensity involving the the dorsal part of pons and thal-
amus, and the child’s movement disablement and fixed
esotropia still existed.
Discussion
The annual incidence of ADEM is presumed to be 0.8/
100,000 persons. ADEM may begin abruptly, acutely, or
Fig. 1 The brain T2-weighted MRI images. The leisions in the dorsal part of pons (arrows) were considered to cause the bilateral abducens palsy.
1: T2-weighted MRI image showing areas of hyperintensity involving the left cerebellar hemisphere, the dorsal part of pons, thalamus, and right
frontal lobe. 2: T2-weighted MRI image after 15 days showing areas of hyperintensity that still involved the dorsal part of pons and thalamus, but
not the left cerebellar hemisphere and right frontal lobe. 3: T2-weighted MRI image after 6 months showing areas of hypointensity involving the
dorsal part of pons and thalamus
Yang and Sun BMC Ophthalmology  (2016) 16:185 Page 2 of 3
over a period of a few days, typically within 1 to 2 weeks
following an antigenic challenge. Neurological symp-
toms typically evolve over several days in ADEM, and
alterations in consciousness may range from mild irrit-
ability or somnolence to prominent behavioral change
or coma [4]. There are no specific biomarkers currently
available to diagnose ADEM, hence, diagnosis is made
after excluding clinical and laboratory findings, and re-
lies on suggestive neuroradiological features of ADEM.
To exclude hereditary metabolic diseases, blood ceru-
loplasmin and lactic acid levels were tested and were
normal. CSF is usually normal, and a mild elevation of
protein with lymphocytic pleocytosis may sometimes
be found. Our case had mild elevation of CSF protein
without abnormal cells. Some studies have suggested
that bilateral thalamic lesions on MRI images may be
diagnostic for ADEM [5–7]. In our case, the brain MRI
showed lesions in the pons, midbrain, medulla, bilateral
thalamus, right frontal cortex, and left cerebellar hemi-
sphere. The lesions in the bilateral thalamus were lar-
ger than 2 cm and tumefactive, which were the most
important features for the diagnosis in our case. The
diagnosis of our patient was based on his clinical pres-
entation with fever, altered level of consciousness,
motor deficits, and brain imaging findings, according
to the revised ADEM definitions in 2013 [8].
Signs of cranial nerve involvement are present in 20–
37 % of patient with ADEM [9, 10], but ANP has not been
reported. In our case, the child’s eyeballs were able to
move up and down, but not left and right following a light
after he regained consciousness. After about 2 months,
esotropia was observed and his eyeballs could not abduce
horizontally. The lesions in the bilateral thalamus and
pons still existed at the 6-month follow-up. The leisions
in the dorsal part of pons are compatible with the loca-
tions of abducens nucleus. The lesions in the abducens
nucleus were considered, acquired central BANP could
be confirmed.
Conclusions
We report the case of an infant with fulminant ADEM
that showed fixed esotropia and marked developmental
regression during 6 months. The BANP and developmen-
tal regression can be symptoms of ADEM in infants.
Abbreviations
ADEM: Acute disseminated encephalomyelitis; ANP: Abducens nerve palsy;
BANP: Bilateral abducens nerve palsy; CSF: Cerebral spinal fluid; GCS: Glasgow
coma score; IVIG: Intravenous immunoglobin
Acknowledgements
Not Applicable.
Funding
No funding was obtained for this study.
Availability of data and materials
All data have been presented within the manuscript and in the form of
images.
Authors’ contributions
ZY is responsible for acquisition of the clinical information and writing up of
the manuscript. GS is responsible for acquisition of the clinical information
and reviewing the manuscript. Both authors read and approved the final
manuscript.
Competing interests
The authors declare that they have no competing interests.
Consent for publication
Written informed consent was obtained from the patient’s parents for
publication of this case report and any accompanying images. A copy of
the written consent is available for review by the Editor of this journal.
Ethics approval and consent to participate
Not Applicable.
Received: 8 June 2016 Accepted: 14 October 2016
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Bilateral abducens nerve palsy in an infant case of fulminant acute disseminated encephalomyelitis: a case report

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Bilateral abducens nerve palsy in an infant case of fulminant acute disseminated encephalomyelitis: a case report

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