Original Article

Bilateral Demyelinating Tumefactive Lesions
in Three Children With Hemiparesis
Zuhal Yapici, MD; Mefkure Eraksoy, MD

ABSTRACT
We present the results from the evaluations of three children ages of 2, 7, and 11 years with hemiparesis and multiple whitematter lesions on magnetic resonance images (MRIs). The initial symptoms were mainly acute/subacute hemiparesis in
all and headache/vomiting in one of them. Before admission, one of them had a history of upper respiratory tract infection, whereas another had undergone urinary tract surgery, and the other reported no history of any infection or stress-related
factor. In all of the children, MRI showed multiple superficial and deep white-matter hyperintensity in T
-weighted and
2
proton density images with perifocal edema in the acute phase. During the symptomatic period, all of the patients underwent corticosteroid treatment. Whereas two of the patients demonstrated signs of recovery during the first week of
treatment, the other patient demonstrated almost a full recovery with minimal neurologic sequela. Follow-up MRI demonstrated not only a remarkable decrease in the size and number of the lesions, with complete resolution for many of them,
it also demonstrated a loss of contrast enhancement. None of these three patients, who had been followed up clinically
and through MRI for 5 years, have shown either a clinical relapse or new lesions. The clinical pictures and MRI of the children were different in some aspects from acute multiple sclerosis and acute disseminated encephalomyelitis. Regarding
both the clinical follow-up and treatment strategy, it is essential and interesting to state the fact that tumefactive lesions
involving both hemispheres are likely to appear during the monitoring of the monophasic courses among inflammatory
demyelinating diseases of childhood such as acute disseminated encephalomyelitis. (J Child Neurol 2002;17:655-660).

Inflammatory demyelinating diseases include a large spectrum and various clinical presentations in childhood. Acute

disseminated encephalomyelitis, childhood cerebellitis,
postimmunization inflammatory demyelinating diseases

(optic neuritis, transverse myelitis, etc), multiple sclerosis,
and some metabolic and toxic diseases constitute the majority of inflammatory demyelinating diseases in childhood1
A relapsing and remitting course can be associated with
many of these diseases (multiple sclerosis, homocystinuria, Leigh’s disease, etc), but clinical features and laboratory findings usually indicate the correct diagnosis.’ We

report three children who presented with hemiparesis as the
main clinical feature, monophasic encephalopathy, and multifocal/tumefactive white-matter lesions detected in magnetic
resonance imaging (MRI). The clinical pictures and MRI findings of these patients are discussed in an attempt to characterize the lesions and to assign the cases to one of the
inflammatory demyelinating diseases, especially acute dis-

seminated encephalomyelitis and multiple sclerosis.
CASE REPORTS
Patient 1
A 2-year-old girl was admitted with a history of left-sided weakness

Received Jan 8, 2002. Received revised May 16, 2002. Accepted for publication May 17, 2002.

and headache that had developed within the previous 3 days. One

From the Department of Neurology (Drs Yapici and Eraksoy), Division of
Child Neurology, University of Istanbul, Istanbul School of Medicine, Istanbul,

demyelinating lesions of Tl-weighted hypointensity and T 2 -weighted
hyperintensity, exerting minimal mass effect in the centrum semiovale (Figures 1 and 2). No contrast enhancement was observed
(see Figure 2). Corticosteroid therapy was initiated by a neurosurgeon, who referred her to our clinic on the tenth day of the symptoms. Dexamethasone 0.5 mg/kg was initiated, gradually reduced,

Turkey.
Presented at the VIIIth International Child Neurology Congress, Ljubljana,
Slovenia, 1998.

Address correspondence to Dr Zuhal Yapici, 14501 Montfort Dr, Apt 1613;
Dallas, TX 75254. Tel: 972-490-3590; e-mail: quitpast@hotmail.com.

week after the onset of symptoms, MRI showed two large bilateral

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656

tures and viral serology were negative, immunoglobulin G index
was

0.55, and no oligoclonal band was detected in cerebrospinal

fluid. Electroencephalography (EEG) showed generalized slowing

(3.5-4.5 Hz). Mental status was also normal. There was no abnormality on ophthalmologic examination. The findings of chest radiography, electrocardiography, and echocardiography were normal.
Magnetic resonance angiography revealed normal neck and cerebral vessels. The patient partially recovered 3 weeks after the
onset of symptoms. She could partially control her left hand. The
corticosteroids were tapered and discontinued at the end of 6
weeks. Except for left hyperreflexia, follow-up examinations (at
the end of 2 months and 57 months, respectively) were normal.
Patient 2
A 7-year-old boy presented with left-sided weakness that had devel-

Figure 1. Patient 1: T2-weighted axial, fourth day of symptoms. Two
large lesions in the white matter.

and eventually ceased over a period of 11/2 months. Partial resolution
of hemiparesis was noted 3 days after the administration of corti-

costeroids. Medical history did not suggest any prenatal problems,
neurodevelopmental milestones, or systemic diseases. There was
no history of vaccination or infection antedating the illness. The
fmdings of systemic examination were normal. Neurologic examination showed a mild left upper motoneuron paresis (4/5) with
hyperreflexia and extensor plantar reflexes. She had a hemiparetic
gait. Complete blood count, sedimentation rate, blood urea nitrogen, serum electrolytes, and liver function tests were all normal.
Screening for sickle cell disease was negative. Urinalysis excluded
homocystinuria. Cerebrospinal fluid fmdings were normal, cul-

oped over the previous 2 days, followed by headache a day after
the onset of weakness. On the third day of symptoms, he had nausea and vomiting and could not walk. Magnetic resonance imaging performed 5 days after the onset of symptoms showed
white-matter lesions with perifocal edema and mass effect in the
right parietal, left parietal, and left frontal areas (Figures 3 and 4).
There were nodular and diffuse contrast enhancements (see Figure 4). The lesions were thought to be of neoplastic nature by a neurosurgeon at another institution, and corticosteroid therapy was
started 6 days after the onset. Pre-, peri-, and postnatal history was
unremarkable. No history of migraine or migraine equivalents was
present. Physical examination was within normal limits. On neurologic examination 13 days after the onset, he displayed mild left
upper motor paresis (4/5) with hyperreflexia and Babinski reflex
on the left side. Complete blood count, sedimentation rate, blood
urea nitrogen, serum electrolytes, and liver function tests were
within the normal range. Screening for sickle cell disease and vasculitis was negative. Urinalysis for homocystinuria was nonrevealing. No atypical cells were found in cerebrospinal fluid. The
other fmdings are shown in Table 1. Pattern visual evoked potentials, chest radiography, electrocardiography, and echocardiogram
were within the normal range. The IQ score was 110 (Alexander
Test). Electromyographic and EEG activities were also within normal limits. There was a dramatic clinical improvement 12 days after
the administration of corticosteroids. On the 20th day of the illness,
a considerable decrease in the lesions was observed on MRI. Dex-

amethasone was gradually reduced and eventually ceased in a
month. Seven months after the onset, MRI showed minimal focal

gliosis in the precentral gyrus (Figure 5). No abnormalities were
found during a follow-up period of 5 years.
Patient 3
An 11-year-old boy presented with focal motor status epilepticus
in the right extremities that developed 2 days after a urinary tract

operation. The seizures were kept under control by administration
of intravenous clonazepam. Hemiparesis was detected on the right
side. Inquiry into prenatal, natal, delivery, and mental-motor neu-

Figure 2. Patient 1: Tl-weighted axial, with contrast. No contrast
enhancement.

rodevelopmental features did not reveal any abnormal findings. A
history of recurrent urinary infections complicated by renal failure was obtained. There was a first-degree consanguineous marriage. No family member suffered from any neurologic disorder. On
physical examination, his weight and height were below the 3rd per-

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657

.

Figure 3. Patient 2: T2-weighted axial, fifth day of symptoms. Bilateral, multiple, irregular, tumorlike, large white-matter lesions.

centile. Neurologic examination showed right upper motor hemiparesis (0/5 in the upper limb, 3/5 in the lower limb) and hyperreflexia with Babinski reflex on the right side. Magnetic resonance
imaging showed bilateral, multiple, irregular lesions (Figure 6)
and heterogeneous contrast enhancement (Figure 7). Complete
blood count and biochemical investigations were normal, except
levels indicating mild to moderate renal failure. His IQ was 72
(Alexander Test). All cerebrospinal fluid fmdings were normal.
EEG showed generalized, bilateral slow waves. Bilateral multiple,
irregular, white-matter hyperintensities were detected on MRI (see
Figure 6). Corticosteroid treatment (2 mg/kg of methylprednisolone

Figure 4.

orally) was initiated, after which the patient completely recovered
within 5 days. Total period of intake of methylprednisolone was 13

weeks. At the end of 5-year follow-up, he exhibited normal find-

ings on neurologic examination. Follow-up MRI (at the end of 3
weeks and 1 year, respectively) revealed normal findings (Figure

8) (see Table 1).
DISCUSSION

Acute disseminated encephalomyelitis often presents as
widespread central nervous system disturbances with coma

Patient 2: Tl-weighted axial, with contrast. Nodular and diffuse contrast enhancement in both A and B.

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658

Table 1.

MRI

=

magnetic resonance imaging; OB

=

Clinical Features and Laboratory Findings of Patients

oligoclonal band; CE

=

contrast enhancement; NL

or drowsiness, headache, seizures, fever, and multifocal
neurologic sign. It can occur with measles, varicella, and
nonspecific upper respiratory tract infections or even without a recognizable preceding event. Whereas there was no
history of antecedent infection or vaccination in our first
patient, the third and second patients had urinary tract
surgery and upper respiratory tract infection, respectively.

=

new

lesion; NE

=

new

episode.

There were bilateral, multiple, scattered, contrast-enhancing nodular lesions in a female patient and two large lesions
in the left subcortical white matter in a male patient. Dagher
et al described a male patient who developed hemiparesis
following flulike symptoms.’ Magnetic resonance imaging

Sometimes it can be difficult to differentiate acute disseminated encephalomyelitis from the acute forms of multiple
sclerosis. In contrast, multiple sclerosis usually presents as
a monosymptomatic syndrome, such as optic neuritis or subacute myelopathy.3 It can also become manifest by
antecedent events like acute disseminated encephalomyelitis. Acute disseminated encephalomyelitis is usually
a monophasic illness, although a few recurrent cases have
been reported.3-7 Nevertheless, no clinical feature is exclusive to one or the other disorder. More diffuse encephalopathic disturbances can rarely be seen with multiple

sclerosis.3

Hemiparesis involving the face is an uncommon presentation of primary demyelinating disorders of the central
system and can cause diagnostic confusion, espeif
disease is monophasic. It is rare in definite multhe
cially
Khan et al described two adult patients with
sclerosis,
tiple
disseminated
multiphase
encephalomyelitis presenting as
The
alternating hemiplegiae
diagnosis was made on the
on
MRI
and
typical appearance
fmdings of the brain biopsy.
nervous

Patient 2: flair coronal, seventh month of the disease. Minimal focal gliosis in the left centrum semiovale.

Figure 5.

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659

Figure 7.

Patient 3: T~-weighted axial with contrast. Heterogeneous

contrast enhancement.

was detected in patient 2 (15 white blood cells/¡J..L), cultures
remained negative, and no atypical cells were found. The presence of oligoclonal bands is a characteristic feature of multiple sclerosis; however, they can also be found in acute
disseminated encephalomyelitis.3 They were observed neither in the blood nor in the cerebrospinal fluid of our patients.
Lesions of acute disseminated encephalomyelitis are
seen in the supra- and infratentorial white matter as asymmetric foci of high signal intensity on long TR images as in

Figure 6. Patient 3: TZ-weighted axial, first day of the disease. Bilateral multiple, irregular white-matter lesions in both A and B.

showed a peritrigonal lesion with moderate mass effect
and homogeneous contrast enhancement. The diagnosis of
these authors for this patient was also acute disseminated
encephalomyelitis, and corticosteroid therapy resulted in a
favorable outcome. Our diagnosis was acute disseminated
encephalomyelitis in patient 3, who developed hemiparesis
after focal status epilepticus and showed characteristic
findings on MRI.
Cerebrospinal fluid in acute disseminated encephalomyelitis can be normal or can show mild leukocytosis with
elevated protein levels or oligoclonal bands, as in multiple
sclerosis.3 Although mild cerebrospinal fluid pleocytosis

Figure 8. Patient 3: T2-weighted axial, third week of the disease.
Complete recovery and no new lesions.

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660

’

patient 3 (see Figure 6). They vary greatly in size and number. Gray-matter involvement is less common. In our patients,
the disease course, clinical presentations, fmdings of cerebrospinal fluid, and MRI suggested acute disseminated
encephalomyelitis, especially in patient 3. Plaques are
sharply demarcated and are frequently found adjacent to the
ventricles. The presence of multifocal asymmetric white-matter lesions on MRI is not sufficient evidence to distinguish
between multiple sclerosis and acute disseminated
encephalomyelitis in patients with a single episode of neurologic disturbance.3 Lesions of multiple sclerosis occur at
varying times. However, occurrence of new lesions is not
expected in acute disseminated encephalomyelitis, provided that the follow-up period is sufficiently long. The
interval that indicates that the patient has acute disseminated
encephalomyelitis and not multiple sclerosis is at present
uncertain. In our patients, no newly formed abnormalities

course of clinical

improvement in acute disseminated

encephalomyelitis.3,15 Control MRI showed complete resolution in our patients (see Table 1).
During the 5-year period of the follow-up of our three
patients, it was noteworthy that they had only one clinical
episode. The picture here is very much like that of acute disseminated encephalomyelitis, as the clinical findings and
monophasic pacing suggest. The cranial MRI of patient 3 in
particular confirms this diagnosis. On the other hand, those
very large lesions on both hemispheres, as detected in two
of our patients, are rare in acute disseminated encephalomyelitis or multiple sclerosis. It is striking that in the first
two cases tumor biopsy was considered. Awareness of such
cases is clinically important in that they can be promptly
treated simply by using oral medication instead of resorting to invasive attempts, which are highly likely to cause

complications.

were detected

during a 5-year follow-up.
That bilateral multiple white-matter lesions were seen
in all of our patients raised questions concerning a diagnosis of Schilder’s disease.9-12 In patient 1, two large bilateral
lesions were localized superficially and deeply in the white
matter (see Figure 1). However, the clinical features were
very different from Schilder’s disease in this patient. Demyelination with mass effect has been reported both in acute disseminated encephalomyelitis and multiple sclerosis.5,9.13
Whereas mass effect was more prominent in patient 2 than
in patient 1, there was no mass effect in patient 3. Signs of
tumefactive demyelination in inflammatory demyelinating
conditions are important in avoiding the need for biopsy and
risky treatment. Extensive and relatively symmetric abnormalities were found in the cerebral white matter in patients
1 and 2 (see Figures 1 to 5). Kepes described a possible
immunologically mediated entity presenting with tumefactive demyelinating lesions with atypical features and histories
not consistent with multiple sclerosis has been described1
Such extensive lesions are often mistaken for neoplasms or
abscesses, as in patient 2.
Contrast enhancement noticed in demyelinating processes is caused by focal breakdown of the blood-brain barrier and may be suppressed by corticosteroids. Since acute
disseminated encephalomyelitis is usually a monophasic
disease, it has been postulated that all lesions would show
contrast enhancement as they would all be active.3 Diffuse,
nodular, ring-like, and heterogeneous gadolinium- enhanced
demyelinating lesions have been reported.13-15 Contrast
enhancement was not observed in patient 1 (see Figure 2),
whereas heterogeneous (see Figure 7) and nodular (see
Figure 4) enhancements were observed in patients 3 and 2,
respectively. No contrast enhancement was observed on follow-up MRI of our patients.
Similarly, follow-up MRI demonstrated a marked
decrease in the size and number of lesions, with complete
resolution of some demyelination areas, as occurs in the

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