Review
a r t i c l e
i n f o
Article history:
Received 14 September 2008
Accepted 31 October 2008
Keywords:
Neurocysticercosis
Cysticidal therapy
Surgical management
Endoscopy
a b s t r a c t
Neurocysticercosis (NCC) is an infection of the brain and its coverings by the larval stage of the tapeworm
Taenia solium. It is the most common helminthic infestation of the central nervous system and a leading
cause of acquired epilepsy worldwide. NCC induces neurological syndromes that vary from an asymptomatic infection to sudden death. Neuroimaging is the mainstay of diagnosis. The diagnosis is suggested
in patients living in endemic areas with typical CT scan ndings and a compatible clinical picture. Since
the late 1980s, successful medical treatment has been established with relatively short courses of either
albendazole or praziquantel. The selection of cases for medical or surgical treatments has improved and
these two forms of therapy are complementary. In general, indications of surgery are: cysts that compress
the brain and cranial nerves locally, intracranial hypertension or edema refractory to medical treatment,
intraventricular NCC, spinal NCC with cord or root compression and ocular cysts. Recently, endoscopic
approaches for ventricular NCC have been developed, which are now the treatment of choice for ventricular NCC with hydrocephalus.
2009 Elsevier Ltd. All rights reserved.
1. Introduction
3. Clinical presentation
Neurocysticercosis (NCC) is an infection of the brain and its coverings by the larval stage of the tapeworm Taenia solium. It is the
most common helminthic infestation of the central nervous system
(CNS) and a leading cause of acquired epilepsy worldwide.16 NCC
results from ingestion of the eggs of T. solium. The oncospheres
hatch in the intestine, penetrate the intestinal wall and disseminate to several body tissues, showing strong tropism to the CNS.
The clinical manifestations of NCC are non-specic and varied
and depend on the number of lesions and the developmental stage
of the cysticercus. Seizures are the commonest manifestation,
occurring in 50% to 80% of patients.3,4 NCC has been classied
depending on the location of cysts, its clinical presentation, prognosis and cyst viability.710
NCC induces neurological syndromes that vary from an asymptomatic infection to sudden death. Differences in the clinical picture depend on the number, size, stage and localization of cysts
and the patients immune response. Patients commonly present
with seizures or headaches.5,6 The clinical manifestations of NCC
are listed in Table 1.
868
Ingestion raw
pork
HUMAN
Cysticerci in
small
intestine
HUMAN
Cysticerci in
brain, muscle,
eyes
Cross GIT
PIG
Cysticerci in
brain, muscle,
eyes, other
organs
Cross GIT
HUMAN
Oncospheres
in stomach
HUMAN
Cestode in
small
intestine
Feco-oral contamination
PIG
Oncospheres
in stomach
Table 1
Clinical manifestations of neurocysticercosis (after91)
Clinical manifestation
Pathology
Epilepsy
Raised intracranial pressure
Focal decits
Meningitis
Myelo radiculopathy
Others
Mental changes (dementia)
Encephalitis
SAH
Trigeminal neuralgia
Subdural hematoma
Stroke/TIA
Dizziness
Endocrinological or
ophthalmic symptoms
CSF = cerebrospinal
ischaemic attack.
uid,
SAH = subarachnoid
haemorrhage,
TIA = transient
immune response, characterized by strong enhancement on contrast CT scans or MRI. This is the colloidal cyst, which has contrast
enhancement but lacks a well-dened scolex. As the cyst further
degenerates, it develops into a nodular cyst, which still shows some
contrast enhancement. Finally, the degenerated cyst calcies and is
recognized as a punctuate calcication on CT scan (Fig. 3).
869
The spinal form occurs in 1.6% to 13% of patients with NCC. The
clinical features depend on the lodgment site of the parasite. Two
forms of spinal NCC are recognized: (i) leptomeningeal (extramedullary) and (ii) intramedullary. The leptomeningeal (extramedullary) form is 6 to 8 times more common than the intramedullary
form. This form occurs as a consequence of downward migration
of larvae from the cerebral to the spinal subarachnoid space. The
intramedullary form is uncommon. It occurs through hematogenous or ventriculoependymal migration. The parasite commonly
lodges in the thoracic spinal cord according to percentage distribution of blood ow to the spinal cord.25,26
Figs. 46. (left to right) 4: Viable parenchymal cyst. Fluid attenuated inversion recovery (FLAIR) axial MRI showing a viable parenchymal cyst. 5: T2-weighted coronal MRI
showing a cyst with the scolex seen as a central low density lesion. 6: Contrast enhanced axial T1-weighted MRI showing a viable cyst (vesicular cyst) with the scolex seen as
an eccentric nodule within the cyst. Note the minimal contrast enhancement due to a weak host immune response.
870
Fig. 7. Noncontrast axial CT Scan showing a cyst in the aqueduct (arrow) causing
obstructive hydrocephalus.
Fig. 8. Noncontrast axial CT Scan showing a fourth ventricular cyst with obstructive
hydrocephalus. Note the deformation of the shape of the ventricular cavity and the
same density of the cyst and cerebrospinal uid, making them indistinguishable.
4. Diagnosis of NCC
The diagnosis of NCC is suspected in patients living in endemic
areas presenting with a compatible clinical picture and lesions suggestive of NCC on CT scans. Neuroimaging is the mainstay of diagnosis of NCC. Table 2 shows proposed objective diagnostic
criteria.32 These criteria are complex and need validation in population or hospital-based studies. The major drawback of these criteria is that they do not differentiate between NCC and
tuberculoma, which is of utmost importance in endemic countries,
such as India.3335 Moreover, access to enzyme-linked immunoelectrotransfer blot assay (EITB) is limited in India and consequently the usefulness of these criteria has been questioned.36,37
4.1. Radiological investigations
4.1.1. Plain X-ray
Plain X-rays of muscles and the skull may show cigar-shaped
calcications.
Table 2
Diagnostic criteria for denitive or probable diagnosis of neurocysticercosis (after32)
Level of
criteria
Findings
Absolute
Major
Pathologic demonstration of the parasite; CT scans or MRI showing a cystic lesion with scolex; direct funduscopic visualization of parasite
Lesions highly suggestive of neurocysticercosis on neuroimaging; positive serum enzyme-linked immunoblot assay for cysticercal antibodies; resolution of
cysts after antiparasitic therapy; spontaneous resolution of a small solitary enhancing lesion
Lesions compatible with neurocysticercosis on neuroimaging; clinical manifestations suggestive of neurocysticercosis; positive cerebrospinal uid enzymelinked immunosorbent assay for anticysticercal antibodies or cysticercal antigens; cysticercosis outside of the central nervous system
Household contact with Taenia solium infection; immigrants from or living in an endemic area; history of frequent travel to disease-endemic areas
Minor
Epidemiologic
Diagnosis: denitive one absolute criterion or two major + one minor + one epidemiologic criterion; probable one major + two minor criteria, or one major + one
minor + one epidemiologic criterion, or three minor + three epidemiologic criteria.
871
4.2. Serology
Fig. 10. T1-weighted sagittal MRI showing a well-delineated fourth ventricular cyst
with obstructive hydrocephalus.
The available serological tests are of little value in clinical practice. These have a sensitivity of 65% to 98% and a specicity of 67%
to 100%, depending on the specic test used, and the cyst burden,
location, and phase of infection.4951 The most commonly used enzyme-linked immunosorbent assay is neither sensitive nor spe-
872
Dilated ophthalmologic examination is sensitive for the detection of ocular cysts and is necessary for anyone diagnosed with
cysticercosis to rule out ocular involvement.54
5. Treatment
The treatment modalities available to patients with NCC
include:
1. cysticidal agents to kill larvae
2. corticosteroids to decrease or prevent the inammatory
reaction.
3. anti-epileptic drugs (AEDs) to prevent or decrease the severity
and number of seizures
4. surgery to remove the cyst or insert a shunt for hydrocephalus. Garcia et al. analyzed the current evidence and reported
consensus guidelines for the management of NCC56 (Table 3).
Fig. 11. A,B. T2-weighted coronal (A) and sagittal (B) MRI showing a rounded welldened cyst in the fourth ventricular cavity with a hyperintense scolex.
Table 3
Guidelines for the treatment of neurocysticercosis (after56)
Type of neurocysticercosis
Fig. 12. Gadolinium-enhanced sagittal MRI of a patient showing an inammatory
cyst with enhancement of the cyst wall and surroundings.
Serology should be used together with clinical and neuroimaging data. Antigen detection assays are also available, which perform well in comparison with other available tests on CSF
samples.52 Antibodies can persist after cysts die; therefore, serology should always be reviewed in light of the presenting clinical
picture and imaging studies.36
4.3. Other tests
Biopsy of brain, skin or muscle can provide a denitive diagnosis in an otherwise ambiguous clinical situation and may be the
diagnostic method of choice for ocular, extraocular muscle, or
painful muscular or subcutaneous cysts.5,5355
Parenchymal
neurocysticercosis
Viable cysts
Calcied
Enhancing lesions
Cysticercotic encephalitis
Extraparenchymal
neurocysticercosis
Intraventricular cyst
Subarachnoid cyst
Hydrocephalus with no
viable cyst
Hydrocephalus + intracranial
cyst
Ocular cysticercosis
Spinal cysticercosis
Treatment
Neuroendoscopic removal
Cysticidal treatment + steroids; VP shunt if
required
VP shunt, no cysticidal treatment
Shunt before cysticidal therapy
Surgery (cysticides avoided)
Surgical treatment
873
encephalitis. Steroids should be given with cysticidal drugs for patients with subarachnoid cysts, chronic meningitis or multiple viable cysts. Oral prednisolone is preferred and should be commenced
2 days to 3 days before cysticidal therapy and continued for 7 days
to 10 days, since maximum exacerbation occurs during this period.
Long-term steroid therapy depends on the parasite load and
inammation. All trials to evaluate cysticidal therapy have used
steroids for a variable period of time.64,82
5.3. Anti-epileptic drugs
A single agent rst-line AED such as phenytoin or carbamazepine usually results in adequate seizure control. The optimal duration of AED therapy has been debated and is estimated by
identifying the parasite stage at the time of the seizure.84 Patients
with degenerating cysticerci develop acute symptomatic seizures
because of the inammatory response of the brain. Therefore, these
patients may be treated for the duration of the acute condition
only, perhaps several months, during the active inammatory response. There are, however, no guidelines for how long AEDs
should be continued after an acute episode. For patients with SCCG,
it is most appropriate to monitor cyst activity with neuroimaging
and to continue AEDs until resolution of the acute lesion. Most
physicians repeat MRI or CT scans after 6 months in patients with
parenchymal cysticercosis (earlier if the patient is symptomatic).
Once the lesion has resolved on neuroimaging, the AED may be tapered off over the next 12 weeks. However, seizures in a patient
with inactive or calcied parasites may be categorized as unprovoked seizures. The treatment should last until the patient has
experienced a seizure-free period of 2 years. Treatment in patients
with multiple lesions or extraparenchymal NCC should be tailored
to the individual.
5.3.1. Future research
More studies are needed to study the prevention of disease
spread and to further elucidate treatment regimes. The Center for
Disease Control and Prevention Working Group on Parasitic Diseases has classied cysticercosis as a potentially eradicable disease.85 Recent studies have demonstrated the potential utility of
various vaccines against Taenia solium for use in pigs, but their
widespread use is not yet a reality.86,87
5.4. Surgery
In general, indications for surgery are:
1.
2.
3.
4.
5.
6.
7.
874
875
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