Address correspondence to
Dr Oscar H. Del Brutto, Air
Center 3542, PO Box 522970,
Miami, FL 33152-2970,
oscardelbrutto@hotmail.com.
Relationship Disclosure:
Dr Del Brutto has received
travel expenses for serving
on the Safe Implementation
of Treatment of Stroke
steering committee.
Unlabeled Use of
Products/Investigational
Use Disclosure: Dr Del Brutto
reports no disclosure.
* 2012, American Academy
of Neurology.
Neurocysticercosis
Oscar H. Del Brutto, MD, FAAN
ABSTRACT
Purpose of Review: Neurocysticercosis occurs when humans become intermediate
hosts in the life cycle of Taenia solium by ingesting its eggs directly from a taenia
carrier or, less often, by contaminated food. Within the nervous system, cysticerci may
lodge in the brain parenchyma, subarachnoid space, ventricular system, or spinal cord,
causing a number of pathologic changes that are responsible for the pleomorphism of
neurocysticercosis. This article discusses the clinical manifestations, diagnosis, and treatment of neurocysticercosis.
Recent Findings: Formerly endemic in the developing world, mass immigration of
people from disease-endemic to nonendemic areas has caused a recent increase in the
prevalence of neurocysticercosis in developed countries, where this condition should no
longer be considered exotic. Recent advances in neuroimaging and immune diagnostic
methods, and the introduction of a set of diagnostic criteria, have enhanced the
diagnostic accuracy for neurocysticercosis. Likewise, introduction of potent cysticidal
drugs has radically changed its prognosis.
Summary: Neurocysticercosis is the most common helminthic infection of the CNS and
a major cause of acquired epilepsy worldwide. Diagnosis of neurocysticercosis is possible
after interpretation of clinical data together with findings of neuroimaging studies and
results of immunologic tests in a proper epidemiologic context. The use of cysticidal drugs
reduces the burden of infection in the brain and improves the clinical course of most
patients. Further efforts must be directed to eradicate the disease through the implementation of control programs against all interrelated steps in the life cycle of T. solium,
including human carriers of the adult tapeworm, infected pigs, and eggs in the environment.
Continuum Lifelong Learning Neurol 2012;18(6):13921416.
INTRODUCTION
Neurocysticercosis, defined as infection
of the CNS by the larval stage of the pork
tapeworm Taenia solium, is currently
considered the most common helminthic disease of the CNS in humans and
a major public health challenge for
most of the developing world. In rural
areas of endemic countries, almost all
conditions favoring the transmission
of the disease, including warm climate,
poverty, and illiteracy, are combined.
The complex and unpredictable
nature of the immunologic reaction
of the host against cysticerci, as well as
the myriad pathologic lesions that
parasites may induce in the CNS, make
neurocysticercosis a fascinating disease. Basic and clinical aspects of neuro-
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FIGURE 8-1
KEY POINTS
h In broad terms,
neurocysticercosis is
endemic in most Latin
American countries,
sub-Saharan Africa,
and some regions
of Asia, including the
Indian subcontinent,
Indonesia, Vietnam,
Korea, and China.
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Neurocysticercosis
KEY POINT
FIGURE 8-2
ETIOPATHOGENESIS
Life Cycle of Taenia Solium
The life cycle of T. solium involves two
hosts: humans and pigs. Humans are
FIGURE 8-3
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the only definitive hosts for the adult cestode, whereas both pigs and humans may
act as intermediate hosts for the larval
form called cysticercus (Figure 8-3).
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FIGURE 8-4
FIGURE 8-5
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Neurocysticercosis
Anatomopathologic findings in
neurocysticercosis. A, Vesicular cysts in brain
parenchyma. B, Subarachnoid parasitic
membranes surrounded by dense mononuclear inflammatory
reaction. C, Dense exudate surrounding the brainstem and
the engulfing basilar artery.
FIGURE 8-6
KEY POINTS
h Human cysticercosis
should now be
considered as a disease
mostly transmitted from
person to person; the
role of infected pigs is
to perpetuate the
infection.
h It is common to find
cysticerci in different
involutive stages in the
same person. It is
unknown whether this
represents cysts of
different ages from
recurrent infections
or a single infection
in which only some
parasites have been
attacked by the hosts
immune system.
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and parasites grow as the result of a degenerative process called hydropic degeneration, caused by the continuous
entrance of CSF into the vesicles.
Tissue Reaction
Around Cysticerci
Parenchymal brain cysticerci in the
vesicular stage elicit a scarce perilesional inflammatory reaction that is
mainly composed of plasma cells, lymphocytes, and eosinophils. Colloidal cysticerci are surrounded by a thick collagen
capsule and a mononuclear inflammatory reaction that usually includes the
parasite itself. The surrounding brain
parenchyma shows an astrocytic gliosis
associated with microglial proliferation,
edema, neuronal degenerative changes,
and perivascular cuffing of lymphocytes.
When parasites enter into the granular
and calcified stages, the edema subsides
but the astrocytic changes in the vicinity
of the lesions may become more intense, and epithelioid cells appear and
TABLE 8-1
Stage of
Involution
Vesicular
stage
Pathologic Changes in
the Brain Parenchyma
Granular
stage
Degenerated scolex
Microglial proliferation
Calcified
stage
Intense gliosis
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KEY POINT
h Defining a typical
syndrome of
neurocysticercosis is
unrealistic. In endemic
areas this parasitic
disease has traditionally
been considered the
great imitator, as it
may mimic almost any
neurologic disorder.
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CLINICAL MANIFESTATIONS
Neurocysticercosis may produce no
clinical manifestations at all or may be
severe enough to cause the death of the
patient. This pleomorphism is related
to individual differences in the number and location of the lesions as well
as in the severity of the hosts immune
response to the parasite. Therefore, defining a typical syndrome of neurocysticercosis is unrealistic. In endemic areas this
parasitic disease has traditionally been considered the great imitator, as it may
mimic almost any neurologic disorder.12
A recent systematic review showed that
recurrent seizures occur in approximately
80% of symptomatic neurocysticercosis
cases, confirming previous findings that
epilepsy is the most common clinical manifestation of the disease.13 Other manifestations of neurocysticercosis include
focal neurologic deficits (16%), increased
intracranial pressure (12%), and cognitive
decline (5%). Cysticercosis outside the
CNS is not associated with clinical manifestations, with the exception of ocular
cysticercosis and some cases with massive muscular involvement.12
Cysticercosis affects males and females
equally from infancy to old age, with a
peak incidence among middle-aged
adults. The course of the disease is somewhat different in infants and children
compared to adults, and neurocysticercosis tends to be more severe in women.
The reasons for these findings are incompletely understood; however, it is possible that the interaction of several factors,
including increased reactivity of the
immune system in children and women,
could be responsible for the age- and
gender-related observed differences in
the pattern of disease expression.
Geographic differences in the clinical
spectrum of the disease have also been
noted. For unclear reasons, subcutaneous
and muscular cysticercosis is observed
far more frequently in Asia and Africa
than in the Americas. Moreover, almost
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KEY POINTS
h Neurocysticercosis is
a leading cause of
acquired epilepsy in
endemic areas and is
partly responsible for
the increased
prevalence of epilepsy
in the developing world.
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KEY POINT
h The frequency of
positive stool
examinations for
T. solium eggs has
varied from one series
to another and seems
to be related to the
severity of infection.
Patients with heavy
infections have a
greater chance of also
having taeniasis.
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Case 8-1
A 60-year-old woman was evaluated because of progressive headache and
vomiting. She had been admitted 2 months before at another hospital
because of hydrocephalus with asymmetric dilatation of the lateral ventricles,
as well as dilatation of the third and fourth ventricles. A right ventricular
shunt was placed with isolated reduction in the size of the right lateral
ventricle, and 15 days later, she underwent the placement of another
ventricular shunt, this time on the left side, with reduction in the size of left
lateral and third ventricles. However, because the fourth ventricle remained
dilated she was transferred to the present institution for further evaluation.
On admission, neurologic examination revealed abnormal downward gaze
and generalized increased muscle stretch reflexes with bilateral Babinski
signs. CT of the head showed collapse of both lateral ventricles resulting from
shunt placement and an abnormally dilated fourth ventricle (Figure 8-7). MRI
showed no evidence of a cystic lesion in the fourth ventricle. A serum
immunoblot test for the detection of anticysticercal antibodies was positive.
She underwent the placement of another shunt device for drainage of
the fourth ventricle, with progressive clinical improvement and further
reduction in the size of that ventricle.
Comment. This
woman had double
compartment
hydrocephalus
related to
simultaneous
occlusion of the
cerebral aqueduct
and the Luschka and
Magendie foramina.
She also had
ependymitis at the
scans of a patient with double
FIGURE 8-7 CT
level of the right
compartment hydrocephalus due to
Monro foramen,
simultaneous occurrence of aqueductal stenosis and occlusion
of Luschka and Magendie foramina.
which explains the
fact that the first
ventricular shunt
only reduced the size of the right lateral ventricle, and it was only after
the second derivative procedure that the size of the left lateral and third
ventricle returned to normal. Double compartment hydrocephalus in
neurocysticercosis may also be related to a fourth ventricle cyst occluding
the CSF transit at both the cerebral aqueduct and the Luschka and Magendie
foramina levels. In this patient, the absence of a fourth ventricle cyst on
MRI and the finding of an incomplete Parinaud syndrome favored the
diagnosis of aqueductal stenosis, since fourth ventricle cysts have rarely, if
ever, been associated with Parinaud syndrome. Differential diagnosis between
these two pathogenetic mechanisms causing an isolated fourth ventricle in
patients with neurocysticercosis is important, as the therapeutic approaches
are completely different. For patients with a fourth ventricle cyst, endoscopic
resection of the lesion is advised. In contrast, multiple shunts are needed for
patients with segmental occlusion of the CSF transit at different levels because
of the simultaneous occurrence of ependymitis and basal arachnoiditis.
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Neurocysticercosis
Case 8-2
A 20-year-old woman presented with a 1-week history of progressive headache, vomiting, and
somnolence. On admission, neurologic examination showed obtundation, bilateral papilledema
(Figure 8-8), increased muscle stretch reflexes, and bilateral Babinski signs. MRI showed diffuse brain
swelling with collapse of the ventricular system, and multiple small cysticerci disseminated through the
brain parenchyma with predominance of the cerebral cortex.
Lesions showed a ringlike pattern of enhancement after
contrast medium administration (Figure 8-9). Serum
immunoblot for the detection of anticysticercal antibodies was
strongly positive. ELISA and Western blot for the detection
of antibodies against HIV were negative. High doses of
dexamethasone (8 mg IV every 8 hours) and mannitol
(100 mL of a 20% solution every 6 hours) were started.
Standard doses of sodium phenytoin were also added to
the regimen. The patient improved over the next few days.
Mannitol was discontinued after 3 days, and IV
dexamethasone was switched to oral prednisone after 1 week.
She was discharged asymptomatic 2 weeks after admission.
Comment. This young woman had cysticercotic encephalitis,
a severe form of neurocysticercosis related to an intense
FIGURE 8-8 Funduscopic examination
showing papilledema.
inflammatory reaction from the host in response to massive
cysticerci infestation of the brain parenchyma. Diagnosis is
suspected on clinical and imaging grounds and must be confirmed by the practice of a serum immunoblot
test. It is also prudent to evaluate the HIV status of the patient, since Toxoplasma encephalitis or other
HIV-related opportunistic infections of the nervous system may occur with similar clinical and
neuroimaging
findings. Cysticidal
drugs are formally
contraindicated
in patients with
cysticercotic
encephalitis, as
therapy may
exacerbate the
inflammatory
reaction within the
brain parenchyma,
causing further
FIGURE 8-9 MRI of patient with cysticercotic encephalitis. A, T1-weighted imaging showing
diffuse brain edema with collapse of the ventricular system. B, T2-weighted
increase in the
imaging showing multiple colloidal parenchymal brain cysts surrounded by edema. C, After contrast
administration, cysticerci appear as ring-enhancing lesions.
intracranial pressure
and death. In
contrast, prompt administration of corticosteroid and osmotic diuretics usually result in marked clinical
improvement. Decompressive craniotomies have been suggested for patients who do not respond to this
initial therapeutic approach. Patients who survive recover without sequelae, and further neuroimaging
studies from 3 to 6 months after the acute episode usually show complete resolution of lesions.
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Immunologic Diagnosis
Immune diagnostic tests have been used
to assess the prevalence of cysticercosis
in populations and to exclude or confirm
December 2012
KEY POINTS
h A major weakness of
the enzyme-linked
immunoelectrotransfer
blot is the high rate of
false-negative results
(up to 50%) observed in
patients with a single
intracranial cysticercus.
Sensitivity of the
enzyme-linked
immunoelectrotransfer
blot is also poor in
patients with calcified
cysticerci.
Neuroimaging
The advent of modern neuroimaging
techniques has drastically improved
diagnostic accuracy for neurocysticercosis. Both CT and MRI provide objective evidence on the topography of
lesions, the burden of infection, the
stage of involution of cysticerci, and the
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KEY POINTS
FIGURE 8-10
FIGURE 8-11
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(Figure 8-10). Vesicular cysticerci appear as small and rounded cystic lesions
that are well demarcated from the surrounding brain parenchyma. Imaging
shows little or no perilesional edema
and no abnormal enhancement after
contrast-medium administration. Many
vesicular cysts have in their interior
an eccentric hyperdense nodule representing the scolex, giving the lesions
a pathognomonic hole-with-dot
appearance. When the infection is
massive, as in the so-called heavy nonencephalitic form of neurocysticercosis,25 the brain looks like a Swiss
cheese, another imaging finding that
is pathognomonic of neurocysticercosis
(Figure 8-11).
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FIGURE 8-12
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Neurocysticercosis
KEY POINTS
FIGURE 8-13
neurocysticercosis), displacing neighboring structures and behaving as spaceoccupying mass lesions (Figure 8-13).
Another common finding in patients
with subarachnoid neurocysticercosis is
hydrocephalus caused by inflammatory
occlusion of the Luschka and Magendie foramina. The fibrous arachnoiditis responsible for the development of
hydrocephalus is seen on CT or MRI as
areas of abnormal leptomeningeal enhancement at the base of the brain.24,30
Cerebrovascular complications of neurocysticercosis are well visualized with
CT or MRI. In patients with cysticercosis-
FIGURE 8-14
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Plain CT showing
ventricular
cysticercus causing asymmetric
dilatation of right lateral ventricle.
FIGURE 8-15
Unification of
Diagnostic Criteria
Despite the introduction of the abovedescribed immune diagnostic tests and
neuroimaging methods, the diagnosis of
neurocysticercosis can still be a challenge
because clinical manifestations are nonspecific, neuroimaging findings are often
not pathognomonic, and immune diagnostic tests are faced with problems
related to poor sensitivity or specificity.
Moreover, histologic demonstration of
the parasite is not possible in most cases.
During the second half of the 20th
century, it was common in field studies
to diagnose neurocysticercosis in patients presenting with seizures and
a positive immunologic test for the
detection of anticysticercal antibodies
T1-weighted gadolinium
contrast-enhanced MRI
of patient with spinal
cysticercosis showing multiple hypointense
cystic lesions in the spinal canal.
FIGURE 8-16
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KEY POINTS
h Accurate characterization
of neurocysticercosis in
terms of viability of cysts,
degree of the hosts
immune response to the
parasites, and location
of the lesions is important
for a rational therapy.
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were frequent but nonspecific manifestations of the disease; and epidemiologic criteria referred to circumstantial
evidence favoring the diagnosis. Interpretation of these criteria permitted
two degrees of diagnostic certainty: (1)
definitive diagnosis in patients who
had one absolute criterion or in those
who had two major plus one minor
and one epidemiologic criteria; and (2)
probable diagnosis in patients who
had one major plus two minor criteria,
in those who had one major plus one
minor and one epidemiologic criteria,
and in those who had three minor plus
one epidemiologic criteria (Table 8-2).34
This set of diagnostic criteria was
promptly adopted by the medical community and is now considered by many
as the gold standard for the diagnosis
of neurocysticercosis.
Advances in neuroimaging from the
time of that publication should be incorporated in the subheading of highly
suggestive lesions to enhance the diagnostic accuracy of MRI. These include
the use of diffusion-weighted imaging
to visualize the scolex in doubtful cases,
the use of susceptibility-weighted images
to enhance the identification of calcifications, and the practice of spectroscopy to
differentiate neurocysticercosis from
neurotuberculosis in selected cases.30Y32
THERAPY
Accurate characterization of neurocysticercosis in terms of viability of cysts,
degree of the hosts immune response
to the parasites, and location of the
lesions is important for a rational therapy.35 Therapeutic approaches may
include a combination of symptomatic
therapy, cysticidal drugs, surgical resection of lesions, and placement of ventricular shunts. General strategies of
therapy described in Table 8-3 may
need some adjustment in the individual
patient, particularly in those who have
mixed forms of the disease.
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TABLE 8-2
b Diagnostic Criteria
Reprinted from Del Brutto OH, et al, Neurology.34 B 2001, with permission from American Academy of
Neurology. www.ncbi.nlm.nih.gov/pmc/articles/PMC2912527/.
Parenchymal Neurocysticercosis
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TABLE 8-3
b Parenchymal Neurocysticercosis
Level 1 evidence favors the use of cysticidal drugs in patients with parenchymal brain vesicular and colloidal cysts. For other forms
of the disease, guidelines are based on Level 2 and Level 3 evidence.
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KEY POINTS
h Calcifications represent
sequelae of previous
infections and should
not be treated with
cysticidal drugs.
FIGURE 8-17
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KEY POINTS
h Higher doses of
albendazole, more
prolonged courses of
therapy, or even repeated
cycles may be needed
for patients with
racemose cysticercus.
h Routine corticosteroid
administration is
mandatory when
treating patients with
large subarachnoid cysts
with albendazole, in
order to avoid the
hazard of a cerebral
infarct.
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KEY POINTS
TABLE 8-4
h Patients with
hydrocephalus due to
cysticercotic arachnoiditis
usually have a protracted
course and a poor
prognosis. The main
problem in these cases
is the high frequency of
shunt dysfunction.
Mortality, which can
be as high as 50% at
2 years, has been directly
related to the number
of surgical interventions
to change the shunt.
b Community Level
Improving living conditions
Health education and awareness of mechanisms of disease acquisition
Mass human chemotherapy (useless without education)
b Infected Pigs
Improved husbandry (pig corralling)
Slaughterhouse control
Control of illegal markets for infected pigs
Freezing of pork meat before human consumption
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KEY POINT
h Neurocysticercosis
eradication programs
must be directed to all
the targets for control,
particularly human
carriers of the adult
tapeworm, infected
pigs, and eggs in the
environment.
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