Short Communications

Pediatric Neurocysticercosis: Three Cases
Presented in the Netherlands with Divergent
Clinical Presentations
Laura A. van de Pol1

T. E. M. van Doeveren1

M. van der Kuip2

1 Department of Child Neurology, VU University Medical Center,

Amsterdam, The Netherlands
2 Department of Pediatric Infectious Diseases and Immunology, VU
University Medical Center, Amsterdam, The Netherlands

N. I. Wolf1

R. J. Vermeulen1

Address for correspondence Laura A. van de Pol, MD, PhD,
Department of Child Neurology, VU University Medical Center,
Amsterdam, The Netherlands (e-mail: l.vandepol@vumc.nl).

Neuropediatrics 2015;46:130–133.

Abstract

Keywords

► neurocysticercosis
► pediatric
► MRI

Background Neurocysticercosis is a helminthic disease that affects the central
nervous system by the larvae of the Taenia solium, the pork tapeworm. Because of
the growing number of immigrants from endemic areas, its incidence is increasing in
Western Europe.
Cases We describe three children, aged between 2 and 13 years, two of whom have a
definite and one a probable diagnosis of neurocysticercosis based on the “Del Brutto
criteria.” They presented with different symptoms and signs: symptomatic epilepsy,
asymmetric cerebral palsy, and headache. Serological evaluation was negative in two of
the three cases. All cases showed comparable abnormalities on magnetic resonance
imaging of the brain: solitary or multiple, cystic lesions, with surrounding edema. In one
of them, the “scolex” (part of the larvae) could be visualized. One case was treated with
albendazole, the other two cases did not receive medication.
Conclusion A prompt diagnosis of neurocysticercosis by recognition of its typical
brain lesions is important to prevent unnecessary diagnostic tests and treatment.

Background

Case Reports

Neurocysticercosis is an infection of the central nervous
system by the larvae of the pork tapeworm, Taenia solium.
It is a major cause of neurological disease and even the most
common cause of epilepsy in the developing world.1 Neurocysticercosis is rare in Western Europe, however, its incidence
has increased over the past 30 years as a result of immigration
from endemic areas.2 Clinical presentation is variable and
prognosis is often favorable.1
We describe three cases of children diagnosed with neurocysticercosis in our hospital in the past 5 years. These cases
illustrate the variety in clinical symptoms, which, in combination with the relative rarity of the disease in the developed
world, may lead to diagnostic delay and unnecessary diagnostic procedures and treatment.

Case 1

received
June 6, 2014
accepted after revision
November 15, 2014
published online
February 2, 2015

A 13-year-old girl from Nepal who had been living in the
Netherlands for 11 years presented with two generalized
epileptic seizures. Neurological examination showed mild
gait ataxia and a minor intention tremor of the left arm.
Brain magnetic resonance imaging (MRI) revealed a calcification in the left parietal lobe and a partially enhancing annular
lesion, with significant perilesional edema in the right parietal area (►Fig. 1). Blood investigation showed an erythrocyte
sedimentation rate of 67 mm/h and a C-reactive protein level
of 67.0 mg/L. Serological testing (LDBio immunoassay [LDBIO
Diagnostics, Lyon, France]3 and in the case of nonconclusive
results an additional test: the Qualidcode of Immunetics
[Immunetics Inc., Boston, Massachusetts, United States])

© 2015 Georg Thieme Verlag KG
Stuttgart · New York

DOI http://dx.doi.org/
10.1055/s-0034-1399753.
ISSN 0174-304X.

This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.

130

van de Pol et al.

Fig. 1 Case 1. (a) Computed tomographic (CT) scan of the brain at presentation showing a lesion in the right parietal area. (b) T2-weighted
magnetic resonance imaging (MRI) scan at presentation showing edema in the right parietal lobe. (c) T1-weighted MRI scan with gadolinium at
presentation showing a partially enhancing annular lesion with perilesional edema. (d) CT scan of the brain showing a calcified lesion in the left
parietal lobe. (e, f) T2-weighted MRI scan and T1-weighted MRI scan with gadolinium at follow-up show reduction of the edema and the lesion in
the right parietal lobe.

revealed anticysticercal antibodies in serum. Serological tests
and an in-house developed polymerase chain reaction (PCR)
for the detection of Taenia solium DNA (a CO1 and NADH1
PCR) were negative in cerebrospinal fluid (CSF). On the basis
of the “del Brutto criteria” for neurocysticercosis, our patient
fulfilled two major criteria (evidence of lesions highly suggestive of neurocysticercosis on neuroimaging and positive
serum immunoblot for the detection of anticysticercal antibodies) plus one epidemiologic criterion (individuals coming
from or living in an area where cysticercosis is endemic)
consistent with a definitive diagnosis of neurocysticercosis.4
The patient was treated with albendazole (15 mg/kg/d:
300 mg twice daily for 7 days) in combination with dexamethasone (1 mg three times a day) and oxcarbazepine. After
admission, she remained seizure free. Oxcarbazepine was
discontinued after 16 months. A follow-up MRI, 3 months
later, showed clear reduction of the abnormalities.

frontal lobe and a smaller fluid cavity in the right frontal lobe.
Both fluid cavities contained multiple well-definable, annular-enhancing lesions, suggestive of neurocysticercosis. Routine blood investigations were unremarkable. No information
from family members was available, as she had been adopted.
According to the “del Brutto criteria” for neurocysticercosis
she had one major criterion (evidence of lesions highly
suggestive of neurocysticercosis on neuroimaging), one minor criterion (presence of clinical manifestations suggestive of
neurocysticercosis [that is, focal neurological signs]) and one
epidemiological criterion (individuals coming from or living
in an area where cysticercosis is endemic) compatible with a
probable diagnosis of neurocysticercosis.4 Her stable neurological development since she had been adopted was the
reason to refrain from treatment. A follow-up MRI after
2 years showed no changes.

Case 3
Case 2
A 2-year-old girl, born in Cambodia and adopted at 6 months
of age, presented with a motor developmental delay and a
preference for the left side. No medical history was available.
Physical examination showed plagiocephaly on the left side.
She did not walk independently until the age of 24 months. A
mild asymmetric cerebral palsy (Gross Motor Function Classification Scale class I5 was found on neurological examination, consisting of hypertonia and hyperreflexia of the right
arm and leg. Brain MRI revealed a large fluid cavity in the left

A 7-year-old vegetarian boy of Indian ancestry, born in the
Netherlands, with a travel history in India, reported severe
headaches regularly accompanied by nausea and vomiting since
2 weeks. Physical examination was unremarkable. Brain MRI
showed two confluent small cysts in the right occipital lobe of
which at least one showed a possible scolex (►Fig. 2), which is
one of the absolute criteria for a definitive diagnosis of neurocysticercosis according to the “del Brutto criteria.”4 Blood investigations (including cysticercosis serology) were unremarkable.
Lumbar puncture showed an increased opening pressure of
Neuropediatrics

Vol. 46

No. 2/2015

131

This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.

Pediatric Neurocysticercosis

Pediatric Neurocysticercosis

van de Pol et al.

Fig. 2 Case 3. (a) T2-weighted magnetic resonance imaging (MRI) at presentation showing two confluent small cysts in the right occipital lobe,
of which at least one showed a possible scolex. (b) T1-weighted MRI with gadolinium at presentation showing enhancement of the lesion.
(c) T2-weighted MRI 3 months later showing clear reduction of the lesion. (d) Susceptibility weighted MRI at follow-up showing calcification of the
lesion. (e) T1-weighted MRI with gadolinium at follow-up showing clear reduction of the lesion.

38 cm H2O. CSF analysis revealed a leukocyte count of 9/uL, with
an increased proportion of eosinophilic granulocytes. No serological testing or PCR was performed in CSF. A follow-up MRI,
1 month later, showed a clear reduction of the lesion. On
susceptibility weighted MRI calcification of the lesion was
evident, indicating progression to the granular-calcified stage
of the disease. Because of quick spontaneous regression of
symptoms and MRI lesions no treatment was started.

Discussion
Neurocysticercosis is endemic in the third world countries,
but it is still a rare disease in the developed world.1 In the
past 5 years, we have seen three cases, one of whom had a
probable diagnosis, in our center in Amsterdam, The
Netherlands. Fabiani and Bruschi recently reviewed all
case studies of neurocysticercosis in Europe published
since 1970. 6 Only three Dutch case reports, describing 16
patients, with neurocysticercosis have been published in
the past 44 years.6 Of the 176 cases in total published from
Europe, the majority were imported cases. Rosenfeld et al
described 47 children with neurocysticercosis in Chicago,
of whom 96% were Hispanic.7 These studies show that the
majority of cases of neurocysticercosis in the developed
world are imported from endemic areas. This holds true for
our patients as well; patients 1 and 2 were born in Nepal
and Cambodia, respectively, and patient 3 frequently
travelled to India. Epidemiologic information is therefore
an important clue to the diagnosis.
Neuropediatrics

Vol. 46

No. 2/2015

In children of all ages, seizures, headache, and vomiting are
the most common presenting symptoms.8 This is consistent
with our patients, presenting with symptomatic epilepsy
(patient 1) and headache with vomiting (patient 3). Our
second patient, however, presented differently with cerebral
palsy. In the literature, neurocysticercosis is renowned for its
pleomorphic clinical presentation, depending on the stage,
number, location, and size of the parasites, as well on the age
and immune response of the host.1 Neurocysticercosis should
be considered as a diagnosis in a wide variety of neurological
symptoms, independent of age.
By eating undercooked pork meat, Taenia solium cysts
can develop into adult parasites in the human digestive tract,
which is called cysticercosis. Neurocysticercosis can develop
when a person is infected with eggs from the Taenia solium
by fecal–oral transmission from a person with cysticercosis,
that become encysted in the central nervous system.1 So,
under poor hygienic circumstances, also vegetarian patients,
such as our third patient, can develop neurocysticercosis.
The cysts will evolve through four stages. First, in the
vesicular stage, the cysts contain a living larva, which may
be visible on MRI as the “scolex.” Degeneration of the cyst is
initiated by an immunological reaction of the host, and the
colloidal stage is entered, in which the vesicular fluid
becomes turbid and the larvae show signs of hyaline degeneration. The third phase is the “granulonodular” phase as the
membrane of the cyst thickens, and the cysticercus is no
longer viable. Finally, calcification of the lesion will take
place.1,9

This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.

132

Pediatric Neurocysticercosis

133

antiparasitic drug alone.13 Despite these recent efforts, treatment of neurocysticercosis remains controversial.12,14 We
feel that the indication for and type of treatment should be
considered in every patient individually.
Neurocysticercosis is a rare infectious disease in developed
countries. Because of increased migration from endemic
areas, it should be included in the differential diagnosis of a
variety of neurological symptoms in children. Clinical manifestations combined with an epidemiological history and
characteristic MRI abnormalities lead to the diagnosis in most
cases. A just and timely diagnosis can prevent children from
unnecessary diagnostic testing and treatment.

Acknowledgments
The authors acknowledge the assistance of Dr. M. Manohar
Shroff with coassessment and advice on the neuroimaging
of case 3.

References
1 Del Brutto OH. Neurocysticercosis. Continuum (Minneap Minn).

Infect Dis 2012;18:1392–1416
2 Garcia HH, Del Brutto OH; Cysticercosis Working Group in Peru.

Neurocysticercosis: updated concepts about an old disease. Lancet
Neurol 2005;4(10):653–661
3 Michelet L, Fleury A, Sciutto E, et al. Human neurocysticercosis:
comparison of different diagnostic tests using cerebrospinal fluid.
J Clin Microbiol 2011;49(1):195–200
4 Del Brutto OH, Rajshekhar V, White AC Jr, et al. Proposed diagnostic criteria for neurocysticercosis. Neurology 2001;57(2):177–183
5 Palisano R, Rosenbaum P, Walter S, Russell D, Wood E, Galuppi B.
Development and reliability of a system to classify gross motor
function in children with cerebral palsy. Dev Med Child Neurol
1997;39(4):214–223
6 Fabiani S, Bruschi F. Neurocysticercosis in Europe: Still a public health
concern not only for imported cases. Acta Trop 2013;128(1):18–26
7 Rosenfeld EA, Byrd SE, Shulman ST. Neurocysticercosis among
children in Chicago. Clin Infect Dis 1996;23(2):262–268
8 Singhi P, Singhi S. Neurocysticercosis in children. Indian J Pediatr
2009;76(5):537–545
9 Baird RA, Wiebe S, Zunt JR, Halperin JJ, Gronseth G, Roos KL. Evidencebased guideline: treatment of parenchymal neurocysticercosis: report of the Guideline Development Subcommittee of the American
Academy of Neurology. Neurology 2013;80(15):1424–1429
10 Lucato LT, Guedes MS, Sato JR, Bacheschi LA, Machado LR, Leite CC.
The role of conventional MR imaging sequences in the evaluation
of neurocysticercosis: impact on characterization of the scolex and
lesion burden. AJNR Am J Neuroradiol 2007;28(8):1501–1504
11 Sáenz B, Ruíz-Garcia M, Jiménez E, et al. Neurocysticercosis:
clinical, radiologic, and inflammatory differences between children and adults. Pediatr Infect Dis J 2006;25(9):801–803
12 Burneo JG, Escalaya AL, Gonzalez-Duarte A. Evidence-based guideline: Treatment of parenchymal neurocysticercosis: report of the
guideline development subcommittee of the American Academy
of Neurology. Neurology 2013;81(16):1474–1476
13 Garcia HH, Gonzales I, Lescano AG, et al; Cysticercosis Working Group
in Peru. Efficacy of combined antiparasitic therapy with praziquantel
and albendazole for neurocysticercosis: a double-blind, randomised
controlled trial. Lancet Infect Dis 2014;14(8):687–695
14 Singh G, White AC Jr. Determining better treatments for neurocysticercosis. Lancet Infect Dis 2014;14(8):658–659

Neuropediatrics

Vol. 46

No. 2/2015

This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.

In 2001, Del Brutto et al proposed diagnostic criteria for
neurocysticercosis, with different degrees of diagnostic certainty.4 Absolute criteria are based on direct demonstration of
the parasite by biopsy, identification of the scolex on MRI or
funduscopic examination demonstrating subretinal parasites. In clinical practice, the diagnosis is often made by a
combination of clinical and epidemiological information together with MRI and serological testing of serum and CSF.4 In
our patients, we used (at least) the following MRI sequences:
sagittal and/or transversal fluid-attenuated inversion recovery (FLAIR), axonal T1-weighted series with and without
gadolinium, axonal T2-weighted imaging, and diffusionweighted imaging. Lucato et al concluded that FLAIR images
detect a significantly higher number of scolices than other
sequences. For demonstrating the lesions, a T1-weighted
sequence with gadolinium is recommended.10 T2-weighted
sequence is relevant to demonstrate perilesional edema.8 To
detect calcifications, computed tomography has been described to be more sensitive than MRI.8 However, in our third
patient, the susceptibility weighted MRI clearly showed the
calcified lesion, and this sequence together with the fast
imaging employing steady-state acquisition image may be
of value in diagnosing neurocysticercosis.
Pediatric neurocysticercosis differs from that in adults.
Children most often present with a single parenchymal cyst,
whereas extraparenchymal disease is rare.11 Indeed, our
three patients had parenchymal disease. However, two out
of the three patients showed more than one lesion (patients 1
and 2). In addition, CSF abnormalities (elevated cell count and
protein level) are infrequently found in children.8,11 In case 3,
we found a mild pleocytosis with normal protein level and
glucose concentration. However, the increased proportion of
eosinophilic granulocytes was a clue to the diagnosis. Serological testing in serum or CSF, using enzyme-linked immunoelectrotransfer blot, can be performed to confirm
suspected diagnosis and has high sensitivity (98%) and specificity (100%) in subjects with two or more lesions.1,2 However, in subjects with a single parenchymal lesion sensitivity
is low, with a false-negative result in up to 50% of cases.
Therefore, especially in children, negative serology and normal CSF findings do not exclude neurocysticercosis.
Neurocysticercosis is treated using a combination of antiparasitic medication; albendazole and/or praziquantel, steroids, and antiepileptic drugs. However, exact indications for
treatment in the individual patient are not precisely defined.
In 2013, a guideline for the treatment of parenchymal neurocysticercosis was published by the American Academy of
Neurology suggesting to consider albendazole plus steroids
in adults and children both to decrease the number of active
lesions on brain imaging studies and to reduce long-term
seizure frequency. This guideline is based on reviewing the
existing randomized controlled trials.9 However, the studies
reviewed were heterogeneous with regard to number of
lesions and treatment regimen.12 Recently, the results of a
large RCT comparing different treatment regimens in neurocysticercosis were published favoring the use of albendazole
in combination with praziquantel over treatment with one

van de Pol et al.

Copyright of Neuropediatrics is the property of Georg Thieme Verlag Stuttgart and its content
may not be copied or emailed to multiple sites or posted to a listserv without the copyright
holder's express written permission. However, users may print, download, or email articles for
individual use.