Acute disseminated encephalomyelitis mimicking brain lymphoma

ACUTE DISSEMINATED ENCEPHALOMYELITIS
MIMICKING BRAIN LYMPHOMA
Saleh Al Ajlouni MD*, Mahmoud Alawneh MD*, Wael Khresat MD*, Rawan Tarawneh MD*
ABSTRACT
We report here on a 10-years-old female patient, previously healthy, presented with a short history of
neurological symptoms, among which ataxic gait, headache, recurrent falling down and inability to move left
leg were found. Initial diagnosis was brain lymphoma depending on the brain MRI findings. CT scan of the
chest, abdomen, and pelvis were normal. Bone marrow biopsy showed no bone marrow involvement by
lymphoma or leukemia. Brain biopsy was performed and showed evidence of demyelination but no evidence
of malignancy. Acute disseminated encephalomyelitis became a very likely diagnosis on the basis of the
clinical, brain MRI and biopsy findings. The patient was started on methylprednisolone pulse therapy for five
days after which she gradually improved regarding her gait and neurological status and general well being. A
follow up brain MRI revealed significant improvement. She was followed up and she was back to normal over
four weeks duration. We conclude that acute disseminated encephalomyelitis may present as lymphoma and
should be considered in the differential diagnosis.
Key word: ADEM, Children, Jordan.
JRMS February 2010; 17(Supp 1): 35-38
Case Report
RS, a 10-year-old female patient, previously
healthy, presented with a 5-day history of ataxic
gait, headache and recurrent falling down many
times per day. She was unable to move her left leg
and dragged it behind her when she attempted
walking.
She had recurrent vomiting on the day of
admission. She also reported a history of an upper
respiratory tract infection two weeks prior to her
admission. There were no other neurological or
systemic complaints. Her family’s history was
unremarkable.
Her general physical examination revealed a
mildly congested throat. She looked ill and did not
respond fully to questions, but was conscious and
oriented.
The power was 4/5 in her left leg, normal on the
right side. Reflexes were +3 bilaterally. She was
also found to have positive Romberg’s sign. Sensory
examination was normal without the presence of
sensory level but impaired proprioception; planters
were up going in both lower limbs, neurological
examination of the upper limbs was normal.
The patient was admitted to hospital and
investigated. Her complete blood picture, renal and
liver profile, serum electrolytes, sugar and
coagulation profiles were normal. CSF studies were
normal and oligoclonal band was negative. Viral
CSF study (mosaic biochip CNS profile) was
normal. CT scan of the chest, abdomen, and pelvis
were normal, bone marrow biopsy showed no bone
marrow involvement by lymphoma or leukemia.
Alpha-feto protein level was 0.9 mg/ml and HCG
*From the Department of Pediatrics, King Hussein Medical Center, (KHMC), Amman-Jordan
Correspondence should be addressed to Dr. S. Al-Ajlouni, (KHMC), E-mail salehajlouni@hotmail.com
Manuscript received November 28, 2005. Accepted March 16, 2006
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Fig. 1. Brain MRI showed large irregular hypo-intense lesions on T1W and hyper-intense lesions in T2W at the
gray-white matter junctions in both parietal and both frontal lobes extending to the periventricular white matter. The
lesions show irregular and inhomogeneous enhancement pattern, suggestive of brain lymphoma.
Fig. 2a, b. Brain MRI after treatment revealed hyper-intense lesions on T2 WI that were significantly smaller in size
and there was no evidence of any new lesions and no evidence of enhancement in these lesions
was less than 5.0 MI/ML. Immunoglobulin
electrophoresis, and serology for HIV and TORCH
were all normal. Visual evoked potentials showed
normal bilateral P100 pattern.
Brain CT revealed white matter hypodensities
symmetrically in both parietal regions. Brain MRI
was done for better evaluation and showed large
irregular hypo-intense lesions at T1W, and hyper
intense in T2W at the gray-white matter junctions in
both parietal and both frontal lobes extending to the
periventricular white matter mainly seen to the left
side. Lesions show irregular and inhomogeneous
enhancement pattern, suggestive of brain lymphoma
as shown in Fig. 1. Fundoscopy examination was
normal. Brain biopsy was performed which showed
evidence of demyelination with no evidence of
malignancy.
In view of this, acute disseminated
encephalomyelitis became a very likely diagnosis
on the basis of the clinical, Brain MRI and biopsy
finding. The patient was started on methyl
prednisolone pulse therapy for five days after which
she gradually improved regarding her gait and
neurological status and general well being. On her
next visit to the clinic two weeks later, her clinical
examination showed that her gait and neurological
exam were back to normal. A repeat brain MRI
revealed hyper-intense lesions on T2 WI that were
significantly smaller in size and there was no
evidence of any new lesions and there was no
evidence of enhancement in these lesions.
Significant improvement was noted (see Fig. 2a and
2b).
The patient was followed up later for many times
in the outpatient clinic with normal general and
neurological examination.
Discussion
Many reports, coming from all parts of the world
have been recently published on the characteristics
of acute disseminated encephalomyelitis (ADEM) in
children. (1,2,3)
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Table I. Different definitions of ADEM used in recently published series of pediatric and adult patients
Author
Definition
Dale et al (1) Inflammatory disease at multiple sites within the CNS, either monophasic or multiphasic if
relapses are thought to represent part of acute monophasic immune process
Hynson et al (2) Acute onset of neurological disturbance and MRI changes involving the white matter in
distribution consistent with ADEM
Murphy et al (4) Acute onset of neurological signs and symptoms together with MRI evidence of multifocal,
hyperintense lesions on the flair and T2 weighted images
Tenenbaum et al (9) Occurrence of presumed inflammatory demyelinating event of acute or subacute onset,
affecting multifocal areas of the CNS with polsymptomatic presentation in an individual who
has no history of neurological symptoms suggestive of earlier demyelinating episodes. the
patient has to show white matter changes without radiological evidence of previous
destructive white matter process.
Mikaeloff et al (10) A first demyelinating event with polysymptomatic onset with mental state change and a
suggestive brain MRI (poorly limited lesions associated, at a time, with thalamus and/or basal
ganglia lesions) with exclusion of infections and systemic disorders.
Schwartz et al (3) Acute neurological symptoms without a history of previous unexplained neurological
symptoms. One or multiple supra or infratentorial demyelinating lesions. Absence of black
holes on T1-weighted MRI (as sign of a previous destructive inflammatory demyelinating
process), exclusion of cerebrospinal fluid infection, vasculitis or other autoimmune disease.
No evidence of a second clinical episode of CNS demyelination.
Anlar et al (14) Diffuse or multifocal neurological findings of acute or subacute onset associated with diffuse
or multiple areas of increased signal intensity on T2 weighted MRI involving the white matter
or central grey matter.
Table II. Preceding infectious illnesses
A. Infections B. Vaccines
* Viral
* Rabies
* Measles
* Diphtheria, tetanus, pertussis
* Mumps
* Smallpox
* Influenza A or B
* Measles
* Hepatitis A or B
* Japanese B encephalitis.
* Herpes simplex
* Polio
* Human herpes virus E
* Hepatitis B
* Varicella, rubella
* Influenza.
* Epstein-Barr virus
* Cytomegalovirus
* HIV
*Others
* Mycoplasma pneumoniae
* Chlamydia
* Legionella
* Campylobacter
* Streptococcus
ADEM has no internationally recognized definition
or diagnostic criteria. Attempts to unify these
criteria were made by seven out of the eleven
published case reports (six children and one adult)
which used nearly similar but slightly different
definitions (see Table I). It is usually preceded by
viral infection or vaccinations and has a wide
clinical spectrum ranging from subclinical episode
to a rapidly progressive disease culminating in
seizure and coma. (1,3) In our case there was a history
of upper respiratory tract infection two weeks prior
to presentation. Viral and bacterial infections and
vaccines that have been implicated in ADEM are
listed in Table II. Patients may present with
different neurological manifestation like optic
neuritis, hemiparesis, cranial nerves palsies and
ataxia. The diagnosis is usually made based on
clinical symptoms and is confirmed by MRI
findings.
In the absence of a biological marker, the
distinction between ADEM and MS cannot be made
with certainty at the time of the first presentation. A
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viral prodrome, early onset of ataxia, highly lesions
load on the MRI, involvement of the deep gray
matter and absence of oligo-colonal bands are more
indicative of ADEM. (4,5) Although ADEM is
typically a monophasic illness, it is difficult to be
distinguished from an acute attack of multiple
sclerosis (MS). (6) Lesions on the thalamus are
described more often in ADEM than MS. (7) In our
case report brain lymphoma was greatly suspected
and tumour-like lesions have been reported in a few
patients. (8) Treatment of ADEM is based on
intravenous administration of steroids, which
usually leads to a favorable outcome. (9,10,11)
In some cases where corticosteroids have failed to
work the use of immunoglobulin and Plasmapheresis
has been shown to produce dramatic
(12, 13)
improvement.
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