CHAPTERI

INTRODUCTION

1.1 BACKGROUND
Neurocysticercosis (NCC) is an infection of central nervous system by the larvae of
pork tapeworm. It is the oldest and most common parasitic infection that leads to
acquired epilepsy worldwide.1 Humans are the definitive hosts for this parasite, and
swine are the intermediate hosts. The adult tapeworm develops in human hosts after
they ingest live cysticercus in undercook pork. NCC develops when humans
accidentally ingest eggs. This occurs when feces of human carriers contaminates
food, although the most important risk factor for the acquisition of cysticercosis is the
proximity of a tapeworm carrier. The disease is mostly localized to the regions where
sanitation facilities such as running water supplies and underground sewage systems
are lacking and hygiene (personal and otherwise) conditions are generally poor. The
movement of people for better lives via immigration, migration and globalization
processes has increased the spread of this disease to non-endemic regions of the
world.2
Neurocysticercosis is highly endemic in Latin America, Mexico, Eastern Europe,
Asia, Africa, and Spain. The estimated serologic prevalence in Mexican adults is
3.6%, with positive confirmation by autopsy in 1.9%. Approximately 50 million
people worldwide are infected. World Health Organization estimates that more than
50,000 individuals die each year from neurocysticercosis.3 Cysticercosis in Indonesia
was first reported in Bali and in Paniai district, Irian Jaya, in the 1970s. The disease
was first introduced into Irian Jaya (Wissel Lakes area) in 1971, and has since spread
to the other parts of the island.4
In the paper is organized with the aim to find out what the etiology, natural history,
how to diagnose and differential diagnosis, prognosis and how to treat the patient.

1.2 PROBLEM IDENTIFICATION

1. What is Neurocysticercosis?
2. What are the sign and symptoms of Neurocysticercosis?
3. How to manage a patient with Neurocysticercosis?
1.3 AIMS
1. To know about Neurocysticercosis
2. To know about the sign and symptoms of Neurocysticercosis
3. To know about the management of Neurocysticercosis
1.4 BENEFITS
1. To understand the sign and symptoms of Neurocysticercosis
2. To understand the diagnosis of a patient with Neurocysticercosis
3. To understand about the management of Neurocysticercosis

CHAPTER II

CONTENT
2.1 DEFINITION
Neurocysticercosis is the infection caused by the larval form of the tapeworm Taenia
solium, is the most common parasitic disease of the central nervous system and the
most common cause of acquired epilepsy worldwide. This has primarily been a
disease that remains endemic in low-socioeconomic countries, but because of
increased migration neurocysticercosis is being diagnosed more frequently in highincome countries. During the past three decades improved diagnostics, imaging, and
treatment have led to more accurate diagnosis and improved prognosis for patients.
This article reviews the current literature on neurocysticercosis, including newer
diagnostics and treatment developments.
Neurocysticercosis (NCC) is a neurologic infection caused by the larval stage of the
tapeworm Taenia solium. In the developing world, neurocysticercosis infection of the
central nervous system (CNS) with the T. solium larvae, is the most common cause of
acquired epilepsy. Because of globalization, many clinicians in industrialized
countries who are unfamiliar with neurocysticercosis are now faced with managing
this disease. Humans are the definitive hosts for this parasite, and swine are the
intermediate hosts. The adult tapeworm develops in human hosts after they ingest live
cysticercus in undercook pork. Neurocysticercosis develops when humans
accidentally ingest eggs. This occurs when feces of human carriers contaminates
food, although the most important risk factor for the acquisition of cysticercosis is the
proximity of a tapeworm carrier. Adult tapeworms shed proglottids, and each
proglottid contains approximately 1000 to 2000 eggs. Once the hexacanth embryo
reaches the parenchyma it forms cysticerci which undergo four stages of involution.
The first is the vesicular stage characterized by a cyst with a translucent vesicular
wall, transparent fluid, and a viable invaginated scolex. During this stage there is little
host inflammatory reaction. The cyst then develops a thick vesicular wall, the fluid
becomes turbid, and the scolex degenerates during the next stage, which is termed the

colloidal stage. An intense inflammatory host response is seen and is reflected in the
pathology which reveals varying degrees of acute and chronic inflammation.
Radiographic examination reveals cystic lesions with edema and enhancement and
seizures are common. The cyst continues to degenerate as it moves into the granular
stage which is characterized by a thick vesicular wall, degenerated scolex, gliosis, and
little inflammatory host response. Ultimately the parasite transforms into coarse
calcified nodules; the calcific stage.5
2.2 ETIOLOGY
Neurocysticercosis is the infection of the CNS and its meninge
coverings by the larval stage of the pork tapeworm Taenia solium.6
Taenia solium, the pork tapeworm, belongs to the Class Cestoda,
which is composed of flat, segmented, ribbon-like worms. The larval
stage of T. solium is a fluid-filled vesicle or cysticercus. 7 The
tapeworm, characterized by a head (scolex) with 4 suckers and a
double crown of hooks and a large body composed of hundreds of
proglottids (segmental units containing thousands of eggs), lives
within the small intestine of the human and can measure several
meters in length.8 These larvae normally infect pigs causing
condemnation of infected meat. However, when humans get
infected, cysts in the nervous system (neurocysticercosis, NCC) may
cause epilepsy, intracranial hypertension and other neurological
symptoms. NCC is a major contributor to the burden of seizures and
epilepsy in developing countries.7
2.3 PATHOPHYSIOLOGY
Neurocysticercosis is cysticercosis that infects the brain. Cysticercosis is caused by
the infection of the larval tapeworm. Taenia solium is the main cause of human
cysticercosis. A human can be infected from ingestion of food contaminated with
feces containing eggs of the parasites, or by autoinfection.9

Taenia solium infects human in the form of egg or in the form of larval (cysticerci).
Cysticerci are fluid-filled vesicles that contain a single inverted protoscolex.
Cysticerci are usually oblong and approximately 1 cm or less in diameter, but Taenia
solium cysticerci can grow up to 10-15 cm in areas such as the subarachnoid space of
the brain. Cysticerci do not usually stimulate an inflammatory response while they
are alive, or after they have died and become calcified. However, while they are
degenerating they can become inflamed.10

Figure 1 Life Cycle of Taenia

Solium1

When eggs of Taenia solium is ingested and has reached the intestine, the egg will
lose its protective capsule due to the gastric acid in the stomach and turn into larval
cyst called oncosphere, which is able to penetrate intestinal wall and circulate to
musculature and also the blood stream. Through the blood stream, it can reach many
types of tissue, including the brain, thus causing neurocysticercosis.9 It is not widely
understood how the oncosphere can bypass the blood-brain barrier, but it is suspected
that some enzymes may weaken the barrier, while some suspect special permeability
allows the passage without even damaging the barrier.11
In the brain, oncosphere will lodge in cerebral vessels and form cyst.
Neurocysticercosys can affect the main part of the brain (parenchymal tissue) causing
parenchymal neurocysticercosis or it can affect the meningen, ventricles, or

subarachnoidal space and causing the extraparenchymal neurocysticercosys which

will manifest as hydrocephalus or meningitis The cyst enables the parasite to be
dormant in a relatively long time because other than being in the brain as a
immunological-previleged area, the cyst will develop several sophisticated
mechanism to avoid host immunological response and therefore it is protected from
the host immune response.11 Once in the parenchyma of the brain, cyst of taenia
solium will undergo four stages.12
1. Vesicular cyst
The larval cyst (cysticerci) is viable and has minimal enhancement due to little
host immune response. Scolex is identified as an eccentric nodule within the cyst.
The cyst will later degenerates and leaks fluid into the parenchyma and generates
strong immune response
2. Colloidal cyst
The cyst is enhancing, without well-defined scolex
3. Nodular/granular
The cyst will further deteriorates and form nodules
4. Calcified granulomas
The degenerating cyst forms a calcified granuloma
The four stages of the cyst is associated with the onset of seizure as the most
commonly found clinical sign in neurocysticercosis patients. The products from the
degeneration of the cysts are proven to be epileptogenic. In the first and new seizure
onset, patients are commonly having an active cyst (either a vesicular or colloidal
cyst). While chronic epilepsy is associated with calcified granuloma. The most
epileptogenic stage of the cyst is the one that is active and undergoing degeneration
(vesicular and colloidal cyst). The conversion from vesicular to colloidal cysts is
associated with the recurrence rates of seizure.12
2.4 DIAGNOSIS
The diagnosis of nerocysticercosis (NCC) can be confirmed by the constellation of
finding including epidemiology, signs, symptoms, serology, and brain imaging. 13,14,15
In epidemiology most patients have lived in endemic regions for long periods, usually
in resource poor areas with on-going transmission. Lack of significant exposure is
particularly helpful in nonendemic regions and should suggest an alternative
diagnosis or search for non-traditional exposures. Patients may have had trivial
6

exposure such as short-term travel to endemic areas or even with no discernible

exposure.13,15 Signs and symptoms caused by NCC are usually not specific. The most
common symptoms are seizures, focal findings, headache and intracranial
hypertension. Seizures, usually focal with generalization or generalized are by far the
most common manifestation. In a large majority, seizures are due to degenerating
cysts or calcified granulomas, more rarely by infarcts in patients with subarachnoid
involvement. Focal neurological findings are less common, non-specific and due a
variety of types of lesions and mechanisms. Headaches are frequent but are nonspecific, and are commonly associated with seizures. In a community study, NCC was
more common in patients with migranous headaches compared to those without
headaches. Hydrocephalus and intracranial hypertension are common in complicated
NCC and may manifest as nausea, vomiting, headache, wide based gait, loss of
consciousness, and other symptoms and findings. When appropriately evaluated,
cognitive deficits and dementia are also frequently found.13,14

Brain Imaging
CNS imaging is essential to establish the diagnosis and to determine the type of
disease, emergency measures required, choice of treatments including amount and
degree of immunosuppression, efficacy and duration of therapy. MRI is better for
detecting intraventricular types and extraparenchymal disease and visualizing the
scolex within the cysticercus (as high intensity inside a cyst) while CT scanning is
better for detecting intracerebral calcifications. The process of cyst degeneration is
best depicted by MRI examinations. Viable cysts appear clear to gross examination
(same density as CSF), hypointense on T1 and fluid-attenuated inversion recovery
(FLAIR) examinations. The first indication of degeneration is the presence of
enhancement, which indicates dysfunction of the blood brain barrier, most likely due
to pericyst inflammation around a still viable cyst. When the cyst becomes grossly
opaque, the MRI appearance changes from no signal to a bright signal on T1 and
FLAIR sequences accompanied by dense enhancement. Single enhancing lesions are
a common diagnostic problem because the morphology is not conclusive and the

serology is commonly negative. In endemic regions they are conditionally considered

NCC if they are 20 mm or less in diameter, not caused by another entity such as
tuberculosis, and do not increase in size over time. Calcified cysts are typically
buckshot-like-small, punctate, and round, although they may be much larger, chunky
and irregular in shape at times. A single calcification is common in endemic
populations, rare in non-endemic populations and therefore can be assumed to be due
to NCC in the proper context. Multiple calcifications are more suggestive. 13,15
Serology Test

An enzyme-linked immunoblot transfer blot (EITB) assay can demonstrate

serum or CSF anticysticercal antibodies. The findings in the serum are more
sensitive than those in the CSF. The assay is highly specific for exposure to T
solium. The sensitivity is high (94%) in patients with multiple lesions or
extraparenchymal infection but may be as low as 28% in patients with a single
parenchymal lesion. EITB assay findings may revert to negative after the
cysticercus dies and are often negative in patients with only calcified lesions.

Antigen detection tests using monoclonal antibodies (developed against

the closely related T. saginata) in a capture ELISA format have been developed.
They are specific for current viable infection, which is their particular utility, but
are not as sensitive as antibody assays. Antigen is commonly present in patients
with subarachnoid disease but is less frequent in parenchymal disease. The
concentration of antigen is higher in the CSF than the serum. Levels grossly
correlate with the extent of involvement. A major unsettled clinical issue is when
to stop anthelminthic treatment in complicated subarachnoid disease. Serial
measurements may be able to establish efficacy of treatment and parasite death.
14,15

2.5 PROGNOSIS
Neurocysticercosis with single lesion ussualy have a good prognosis. The lesions can
disappear within 6 months in 60% cases and the seizure can well controlled. While
the neurocysticercosis with multiple lesions and with calcification often have frequent
seizure recurrences. The extraparenchymal neurocysticercosis have a guarded
prognosis.16

2.6 TREATMENT
Treatment of neurocysticercosis depends upon the viability of the cyst and its
complications. Management includes symptomatic treatment as well as treatment
directed against the parasite.
The goals of pharmacotherapy for neurocysticercosis are to reduce morbidity, prevent
complications, and eradicate the infestation. Albendazole treatment was evaluated to
reduce active brain cysts of neurocysticercosis and seizure recurrence. Patient were
given albendazole 400 mg twice a day for eight day and for children weight less than
< 45 kg given albendazole 15mg/kg for 8 day. Albendazole treated patient found that,
active brain cysts were cured and a higher reduction of number of brain cysts was
achieved. However, although a significant reduction in brain cysts was obtained,
patients with albendazole treated neurocysticercosis were not free of seizures for
significantly longer.17 Albendazole and praziquantel are two most frequently used
anticisticercal drugs. A meta-analysis suggest that albendazole is more effective than
praziquantel. Corticosteroid are used by most experts but their use has not been
systematically studied.18 According to American Academy of Neurology treatment
combination between Albendazole and corticosteroid also help patient to reduce brain
lesion.
Management of symptomatic treatment like Seizure can be treated by using anticonsulvant drug although meta-analysis studied on American Academy of Neurology
show, neurocysticercosis patient that treated by Albendazole having a constant
decrease on number of patient of seizure. Meningitis can be developed on this case,
inflammatory reaction can be treated by anti-inflammatory drugs such as
corticosteroid. In some case surgical treatment needed in patient with developmental
of hydrocephalus.

Unsafe production of pork meat permits the parasite to enter the human food chain.
Furthermore, there is evidence that pig to pig transmission is possible and also other
Taenia species may lead to human cysticercosis. In this case carefulness of pork
production needed as a prevention to this diseases. The prevention include the safe
way of cooking pork and also maintain the hygiene.18

CHAPTER III
CONCLUSION

10

Neurocysticercosis (NCC) is an infection of central nervous system by the larvae of pork

tapeworm, Taenia solium. Taenia solium enters human body through uncook meat that
contain its egg, so human body become definitive hosts and swine are the intermediate
hosts. NCC develops in region where lack of clean sanitation and poor hygiene. Taenia
solium belongs to the class Cestoda, characterized by a head with four suckers and a
double crown of hooks and a large body composed of hundreds of proglottids. It lives in
small intestine of human body and can measure several meters in length. Cysticerci
(Taenia soliums larvae) are usually oblong and approximately 1 cm or less in diameter,
but Taenia solium cysticerci can grow up to 10-15 cm in areas such as the subarachnoid
space of the brain. The stages of cyst development in brain is associated with the onset of
seizure as the most commonly found clinical sign in neurocysticercosis patients. In the
first seizure onset patients are commonly having an active cyst (vesicular or colloidal
cyst). The most epileptogenic stage of the cyst is the one that is active and undergoing
degeneration (vesicular and colloidal cyst). The conversion from vesicular to colloidal
cysts is associated with the recurrence rates of seizure.
The diagnosis of nerocysticercosis can be confirmed by the constellation of finding
including

epidemiology,

signs,

symptoms,

serology,

and

brain

imaging.

Neurocysticercosis with single lesion ussualy have a good prognosis. While the
neurocysticercosis with multiple lesions and with calcification often have frequent
seizure recurrences. Neurocysticercosis treatment includes symptomatic treatment as well
as treatment directed against the parasite. The goals of pharmacotherapy for
neurocysticercosis are to reduce morbidity, prevent complications, and eradicate the
infestation. Albendazole treatment was evaluated to reduce active brain cysts of
neurocysticercosis and seizure recurrence.

REFERENCE

11

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