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Neurocysticercosis: updated concepts about an old disease

Hector H Garcia, Oscar H Del Brutto, for The Cysticercosis Working Group in Peru

Neurocysticercosis, the infection of the human brain by the larvae of Taenia solium, is a major cause of acquired epilepsy in most low-income countries. Cases of neurocysticercosis are becoming more common in high-income countries because of increased migration and travel. Diagnosis by neuroimaging and serological assessment has greatly improved over the past decade, and the natural progression of the disease and response to antiparasitic drugs is now much better understood. Neurocysticercosis is potentially eradicable, and control interventions are underway to eliminate this infection. Meanwhile, updated information on diagnosis and management of neurocysticercosis is required, especially for clinicians who are unfamiliar with its wide array of clinical presentations.

Lancet Neurol 2005; 4: 65361 Cysticercosis Unit, Institute of Neurological Sciences, Lima, and Department of Microbiology, Universidad Peruana Cayetano Heredia, Lima, Peru (H H Garcia MD); Department of Clinical Neurosciences, Hospital-Clinica Kennedy, Guayaquil, Ecuador (O H Del Brutto MD) Correspondence to: Dr Hector H Garcia, Cysticercosis Unit, Instituto de Ciencias Neurolgicas, Jr Ancash 1271, Barrios Altos, Lima 1, Peru hgarcia@jhsph.edu

Neurocysticercosis is an old disease. Known in ancient Greece as a disease of swine and since the 17th century as a human ailment, neurocysticercosis was not considered a public health problem until the second half of the 20th century, when British investigators recognised the disease among soldiers returning from India. Since then, hundreds of studies have described the epidemiological characteristics and the clinical manifestations of neurocysticercosis.1,2 During the past three decades, the introduction of modern diagnostic tools and potent cysticidal drugs has allowed accurate diagnosis and improved the prognosis for many patients.1,3 Despite these advances in diagnosis and therapy, neurocysticercosis remains endemic in most lowincome countries, where it represents one of the most common causes of acquired epilepsy.1 WHO has calculated that over 50 000 deaths are due to neurocysticercosis each year, and many times this number of people have active epilepsy, with all the social and economic consequences that this implies.4 Neurocysticercosis is being diagnosed with increasing frequency in high-income countries because of increased migration of people with the disease5 or tapeworm carriers,5,6 and because of tourism and travel to endemic areas. It is also one of a few conditions included in a list of potentially eradicable infectious diseases of public-health importance,7 and control or eradication programmes are urgently needed to reduce its effect.

contaminated with human faeces and thus T solium eggs. However, recent epidemiological evidence suggests that the most common source of infective eggs is a symptom-free tapeworm carrier in the household.8,9 Therefore, cysticercosis should be seen as a disease mostly transmitted from person to person, whereas the role of infected pigs is to perpetuate the infection. In the usual cycle of transmission of T solium, pigs have access to contaminated human stools by their coexistence with human beings in the domestic setting and the lack of household sewage or sanitary facilities. Pig-to-pig transmission has been recently described,10 but its effect on transmission pressure is not yet known. Figure 1 shows the larval cysts in the meat of an infected pig. The cyst consists of a scolex, or head, of the future tapeworm surrounded by a vesicle formed by the extension of the parasites tegument (vesicular wall).11 T solium is a 24 m atworm that lives in the human upper small intestine, most often without noticeable symptoms. It excretes eggs and proglottids irregularly, which if ingested cause cysticercosis. After ingestion, eggs hatch and liberate the embryos or oncospheres into the intestine. Oncospheres actively cross the intestinal

Life cycle of Taenia solium

Although the pig is the usual intermediate host of the tapeworm Taenia solium, human cysticercosis occurs when the eggs, which are excreted in the faeces of an individual carrying the parasite, are ingested. A common misconception is that one can acquire neurocysticercosis by eating pork. However, ingestion of infected pork only causes adult tapeworm infestation (taeniasis), because infected pork contains the larval cysts that develop into adult worms in human intestine, and does not contain the eggs that cause cysticercosis. Transmission was previously thought to be by indirect means, such as by the ingestion of vegetables irrigated with water
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Figure 1: Infected pork showing multiple viable cysticerci



wall, enter the bloodstream, and are carried into the tissues of the host where they develop into larval cysts. These cysts are rapidly destroyed by the hosts immune system in most circumstances, except for those located in immunologically privileged sites such as the eye and the nervous system.12

Natural history
There is little information on the natural history of human cysticercosis or neurocysticercosis. Data from pigs show that cysts reach their maximum size in 23 months,13 and that while alive they trigger little perilesional inammation.14 However, most pigs are slaughtered at about 9 months of age and thus this model only reects the early stages of the infection.15 In human beings, the initial perspective of neurocysticercosis (based on the few cases diagnosed by radiography, and a series of cases attending surgery or with the disease detected only at necropsy) was that of a lethal, aggressive disease, mostly causing intractable epilepsy and progressive intracranial hypertension.16 Two natural epidemiological scenarios helped to understand the dynamics of infection and disease, at least in part. Early in the 20th century, British troops were sent on duty to India for dened periods of time. Many (up to 450) of them or their direct relatives had seizures and were studied by a special unit of the British Army, which was able to nd evidence of neurocysticercosis in up to 75% of those with seizures.16,17 More importantly, the onset of neurological symptoms was recorded according to the date of return to the UK (a

cysticercosis-free country), and most individuals had their initial seizure 25 years after their return, suggesting a long latent stage. In a second, unrelated episode in 1968, the King of Bali sent pigs as a preelectoral gift to the peasants of Papua New Guinea, an island that was free of cysticercosis. 2 years later, a sudden epidemic of burns was caused by people falling into their bonres because of seizures that occurred while sleeping close to the re. Necropsy studies of some of these people conrmed neurocysticercosis as the cause of the seizure disorder.18 These bouts of neurocysticercosis, in cases for which the date of infection could be traced, were evidence against symptoms appearing at the time of initial exposure. The introduction of CT and MRI unveiled a whole spectrum of mild infections in symptom-free individuals or in patients with sporadic seizures, which changed the denition of neurocysticercosis from a fatal or severe disease, to a less aggressive one. For example, in India, most individuals with neurocysticercosis present with a single degenerating cysticercus, whereas in Latin America and China a substantial proportion of infected individuals present with a few viable brain cysts.19 Recently, several studies with CT in endemic villages of Latin America have found that 1020% of symptom-free villagers have one or more intraparenchymal brain calcications.2024 We have hypothesised that in most mild exposures the parasite dies in its early stages by action of the hosts immune system, whereas a small subgroup of infections (probably those with heavier egg challenges) become established and survive as viable cysts.9

Figure 2: Diverse presentations of neurocysticercosis Multiple viable cysts (vesicular stage; A); single enhancing lesion (degenerating cysts; B); multiple intraparenchymal calcications (C); intraventricular cyst (D); basal subarachnoid cysticercosis (extraparenchymal neurocysticercosis; E); cysticercotic encephalitis (extraparenchymal neurocysticercosis; F); ocular cysticercosis (G); and muscle cysticercosis (H).


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General features of neurocysticercosis

Clinical description
Although neurocysticercosis can cause almost any neurological symptom, late-onset epilepsy and intracranial hypertension are its most common clinical manifestations.25,26 Symptomatic neurocysticercosis results from a combination of factors, including the number, stage, and localisation of the parasites within the nervous system, as well as the severity of the hosts immune response against the parasites (gure 2). Seizures occur in up to 70% of patients.26 Patients may also present with intracranial hypertension that can be associated with seizures, dementia, or focal signs. Hydrocephalus, related to arachnoiditis, granular ependymitis, or ventricular cysts, is the most common cause of this syndrome.27 Intracranial hypertension also occurs in patients with giant cysts and in those with the rare form of cysticercotic encephalitis (resulting from infection with many cysticerci inducing a severe immune response from the host).28 Various focal neurological ndings may also occur in patients with neurocysticercosis. Whereas focal signs usually follow a subacute or chronic course, some patients present with acute focal signs due to the occurrence of a cerebrovascular event.29 Patients are likely to have transient and eeting focal neurological decits that may either be postictal or are elicited by acute inammatory responses to parasites.

Panel: Proposed diagnostic criteria for neurocysticercosis Absolute 1 Histological demonstration of the parasite from biopsy of a brain or spinal-cord lesion 2 Cystic lesions showing the scolex on CT or MRI 3 Direct visualisation of subretinal parasites by funduscopic examination Major 1 Lesions highly suggestive of neurocysticercosis on neuroimaging studies (ie, CT or MRI showing cystic lesions without scolex, enhancing lesions, or typical parenchymal brain calcications) 2 Positive serum EITB (with puried extracts of T solium antigens) for the detection of anticysticercal antibodies (assay developed by the Centers for Disease Control and Prevention, Atlanta, GA, USA) 3 Resolution of intracranial cystic lesions after therapy with albendazole or praziquantel 4 Spontaneous resolution of small single enhancing lesions (ie, solitary ring-enhancing lesions measuring less than 20 mm in diameter in patients presenting with seizures, a normal neurological examination, and no evidence of an active systemic disease) Minor 1 Lesions compatible with neurocysticercosis on neuroimaging studies (ie, CT or MRI showing hydrocephalus or abnormal enhancement of the leptomeninges, and myelograms showing multiple lling defects in the column of contrast medium) 2 Clinical manifestations suggestive of neurocysticercosis (ie, seizures, focal neurological signs, intracranial hypertension, and dementia) 3 Positive CSF ELISA for detection of anticysticercal antibodies or cysticercal antigens 4 Cysticercosis outside the CNS (ie, histologically conrmed subcutaneous or muscular cysticercosis, plain radiographic lms showing cigar-shaped soft-tissue calcications, or direct visualisation of cysticerci in the anterior chamber of the eye) Epidemiological 1 Evidence of a household contact with T solium infection 2 Individuals coming from or living in an area where cysticercosis is endemic 3 History of frequent travel to disease-endemic areas
Reproduced with permission from Lippincott Williams and Wilkins.37

Diagnosis of neurocysticercosis is typically made on the basis of neuroimaging studies and conrmatory serological analysis. The most common neuroimaging examination done in endemic areas is CT. The latest generation of CT machines have fairly good diagnostic sensitivity, although some small lesions, especially those in the posterior fossa, close to the bone, or those inside the ventricles or basal cisterns, may be missed.30 MRI has better accuracy, although it may miss some small calcications, and has the important pitfall of being much more expensive and less available in areas where the disease is endemic. Several serological assays to detect specic antibodies have been used for decades with different and somewhat conicting results.31,32 Currently, most centres use an enzyme-linked immunoelectrotransfer blot (EITB) with puried glycoprotein antigens (western blot),33 which can be done in serum samples or in CSF or use ELISAs in CSF samples. An advantage of EITB is that its sensitivity in serum samples is equal to or better than that in CSF samples.34 Although EITB has 100% specicity and an overall sensitivity of 98%, a major problem is that approximately 30% of patients with a single brain parasite may test negative.35 An interesting new development is the introduction of antigen-detection ELISA, although no concrete data on sensitivity and specicity are yet available.36
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A set of diagnostic criteria has recently been proposed to help clinicians and health workers with the diagnosis of neurocysticercosis.37 Proper interpretation of these criteria permit two degrees of diagnostic certainty, denitive or probable (panel).

Treatment should be tailored according to the type of neurocysticercosis (table).38 Physicians in charge of patients with neurocysticercosis should always remember that therapy includes a combination of symptomatic and antiparasitic measures, including analgesics, antiepileptic drugs (AEDs), cysticidal drugs, surgical resection of lesions, and placement of ventricular shunts. The main point of controversy has been over the use of cysticidal drugs, used since 1979.3941 Praziquantel is most often used at doses of 50 mg/kg/day for 15 days, but the drug has been given in regimens of 10100 mg/kg for 321 days,42 or even as a single-day regimen (based on exposing cysticerci to very high concentrations of


Treatment Parenchymal neurocysticercosis Vesicular cysts Single Moderate infections Heavy infections (100 or more cysts) Degenerating (colloidal) cysts Single lesions Moderate infections Heavy infections (encephalitis) Calcications Single or multiple Extraparenchymal neurocysticercosis Subarachnoid neurocysticercosis Giant cyst (usually in Sylvian ssure) Basal subarachnoid (racemose) Ventricular cysts Hydrocephalus Arachnoiditis, angiitis Ependymitis Other forms of neurocysticercosis Spinal cysts Ocular cysts

Albendazole 15 mg/kg/day for 1 week, steroids used only if side-effects occur; or praziquantel 100 mg/kg in three equal doses Albendazole 15 mg/kg/day for 1 week, with simultaneous use of steroids. Albendazole 15 mg/kg/day for 1 week with high doses of steroids Albendazole 15 mg/kg/day for 1 week, steroids used only if side-effects occur; or no antiparasitic treatment Albendazole 15 mg/kg/day for 1 week with steroids No antiparasitic treatment, high doses of steroids, osmotic diuretics (mannitol) No antiparasitic treatment

Albendazole 15 mg/kg/day for 1 month, with high doses of steroids; or surgical excision Albendazole 15 mg/kg/day for 1 month, with high doses of steroids. Endoscopic aspiration or surgical resection, use of antiparasitic drugs is controversial No antiparasitic treatment, ventricular shunt No antiparasitic treatment, high doses of steroids for 1 month No antiparasitic treatment, ventricular shunt if indicated, high doses of steroids Surgical resection, albendazole may be used Surgical resection

Table: Treatment guidelines for the diverse forms of neurocysticercosis

praziquantel, by giving three doses of 2530 mg/kg at 2 h intervals).43 As a drawback, serum concentrations of praziquantel decrease when steroids are also used.44 Albendazole was initially given at doses of 15 mg/kg/day for 1 month.40 Further studies showed that at similar doses, the length of therapy could be shortened to 1 week without lessening the effectiveness of the drug.45,46 In general, albendazole has higher parasiticidal effect than does praziquantel.40 Cysticidal drug therapy has been harshly criticised by some clinicians because treatmentassociated parasite death leads to an acute, severe inammatory reaction in the surrounding brain tissue, increasing intracranial hypertension and potentially leading to the death of the patient.47 Another major argument against the use of cysticidal drugs has been that there was no evidence that killing the parasites would lead to fewer seizures during follow-up.48 However, as noted below, current evidence favours their use in patients with viable intraparenchymal or extraparenchymal parasites.49,50 The administration of a single rst-line AED results in seizure control in most patients with neurocysticercosis-related epilepsy.26 However, the optimum duration of antiepileptic drug therapy in these patients has not been settled. Up to 50% of those patients who remain seizure free for 2 years while on AEDs will have relapses after AED withdrawal, suggesting that intracranial cysticerci are permanent substrates for seizures and may be reactivated when the inhibitory inuences of AEDs are absent.51 Prognostic

factors associated with seizure recurrence include the development of parenchymal brain calcications, and the presence of recurrent seizures and multiple brain cysts before the institution of therapy.52 Although some clinicians have proposed that AEDs could be discontinued after 3 months of resolution of a single brain-enhancing lesion,53 controlled data supporting this afrmation are still required.5355 Corticosteroids are the main form of therapy for cysticercotic encephalitis, angiitis, and chronic meningitis that causes progressive entrapment of cranial nerves56 (particularly for extraparenchymal neurocysticercosis). Simultaneous administration of corticosteroids ameliorates the secondary effects of headache and vomiting that may occur during cysticidal drug therapy. Such manifestations are not associated with the toxic effects of the drugs but to the destruction of parasites within the brain, and are reliable indicators of drug efcacy. In patients with giant subarachnoid cysticerci, ventricular cysts, spinal cysts, and multiple parenchymal brain cysts, corticosteroids must be given before, during, and even some days after the course of cysticidal drugs to avoid the risk of cerebral infarcts, acute hydrocephalus, spinal-cord swelling, or massive brain oedema.56 The most common surgical indication in neurocysticercosis is ventricular shunting to resolve hydrocephalus.57 Hydrocephalus secondary to neurocysticercosis is associated with high rates of shunt dysfunction; indeed, it is common for these patients to have protracted courses of disease and high mortality, correlated with the number of surgical interventions to revise the shunt.58 Twice weekly prednisone treatment reduces the risk of shunt dysfunction.59 Other surgical indications include the excision of giant cysts or intraventricular cysts.

Location of neurocysticercosis
One of the main problems in analysing the abundant literature on neurocysticercosis is the generalisation of concepts while ignoring the differences between the disease types. Mixing different types of neurocysticercosis leads to confusing assessments of accuracy of diagnostic tests, therapeutic approaches, and prognosis. To avoid the risks of unwarranted generalisation, we have categorised the main clinical presentations of neurocysticercosis, and describe their associated clinical manifestations and specics of diagnosis, therapy, and prognosis. A major factor is whether parasites are in the brain parenchyma or in extraparenchymal structures.

Intraparenchymal cysts Pathophysiology

Once established, the larval cysts actively evade the hosts immune response through several mechanisms, including inhibition of complement, cytokine release, and masking with host immunoglobulins.60,61 Thus, only
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scarce inammatory changes are seen in the surrounding tissues. In this vesicular stage, parasites look healthy and have a clear vesicular uid (gure 2). Viable cysticerci may remain alive for years, and a substantial proportion of even those lesions with signs of inammation do not die for several months. At some point, the hosts immune system and the inammatory response overcome the immune evasion mechanisms, resulting in the death of the parasite.60,61 The rst stage of involution of cysticerci is the colloidal stage, in which the vesicular uid becomes turbid, and the scolex shows early signs of degeneration. Colloidal cysticerci are surrounded by a thick collagen capsule and the surrounding brain parenchyma shows astrocytic gliosis and diffuse oedema.62 Thereafter, the wall of the cyst thickens and the scolex is transformed into coarse mineralised granules; this is called the granular stage. A single brain cyst in the colloidal or granular stage compose the so-called single enhancing lesions, common in Indian patients.63,64 Finally, in the calcied stage parasite remnants appear as a mineralised nodule. When parasites enter the granular and calcied stages, the oedema subsides, but astrocytic changes in the vicinity of the lesions become more intense than in the preceding stages.11,65

Figure 3: New MRI techniques that may improve diagnostic accuracy for cysticercosis FLAIR (left) and MRI with inversion recovery (right).

Because most cases of neurocysticercosis are already calcied or will eventually resolve and become calcied, this mechanism may be the main reason for morbidity in neurocysticercosis.

A single course of albendazole or praziquantel kills 6085% of viable brain cysts.40,45,46,50 A recent randomised, blinded, controlled trial with albendazole showed the clinical benet of decreased numbers of seizures and enhanced resolution of cysts after treatment, providing evidence for the use of cysticidal drugs in patients with viable intracranial cysts.50 Several randomised trials in Indian patients with single enhancing lesions7074 showed a non-signicant but consistent trend towards fast radiological resolution of lesions and decreased likelihood of seizure relapse (gure 4). Fast radiological resolution after cysticidal treatment may be helpful in the management of patients with single enhancing lesions, thus avoiding diagnostic pitfalls.75 However, in most patients with single enhancing lesions, the lesions disappear spontaneously.76 Patients with cysticercotic encephalitis should not receive cysticidal drugs because they may exacerbate the intracranial hypertension observed in this form of the disease.38 Finally, patients with calcications alone should not receive cysticidal drugs because these lesions represent dead parasites (table).

On CT, viable cysts appear as hypodense, rounded, cystic lesions. Some may enhance after administration of contrast. The scolex can be occasionally seen as a hyperintense dot in the interior of the cyst. On MRI, the cysts are hypointense in T1 and FLAIR sequences, but hyperintense in T2 sequences. MRI is better than CT to show small cysts or those close to the skull or in the posterior fossae. Degenerating cysts appear as contrastenhancing rings or nodules surrounded by areas of brain oedema (gure 2). The scolex is not usually seen using CT or MRI, creating some diagnostic confusion with other infections or even with intracranial neoplasms. FLAIR or diffusion-weighted MRI may allow the visualisation of the scolex in some degenerating cysts, facilitating the correct diagnosis in these cases (gure 3). One of the most important (and difcult) differential diagnoses of a single degenerating cysticercus is a tuberculoma. Rajshekhar and Chandy66 have suggested that lesions measuring 20 mm or less without a shift of the midline structures due to the surrounding oedema are most probably due to cysticercosis. Magnetic resonance spectroscopy seems to detect a peak of lipids in tuberculomas67 not present in degenerating cysticerci. Calcied cysts appear as punctate hyperdense dots on CT, or as areas of subtracted signal on MRI. Because of this, old MRI methods had poor sensitivity to detect calcied neurocysticercosis. At the time of a symptomatic relapse, a third to a half of patients with calcied lesions only may show oedema around at least one calcied lesion.68,69
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Extraparenchymal neurocysticercosis
Extraparenchymal disease varies in its symptoms or prognosis according to whether the parasites are located in the convexity of the cerebral hemispheres, in the basal subarachnoid space, in the sylvian ssure, or in the ventricles. Intracranial hypertension is a common manifestation of extraparenchymal neurocysticercosis and may be due to mass effect, distortion of the normal anatomy of CSF pathways, direct obstruction of the ventricular system by a cyst, or inammatory reaction in







4 Odds ratio






2 Odds ratio

with a severe local inammatory response with high protein concentrations and cell counts in the CSF.78 Other less common manifestations include basal meningitis, ventriculitis, angiitis, or hydrocephalus with no discernible cysts (commonly associated with inammatory CSF and strong seropositivity). These conditions seem to correspond to residual pathology from old, resolved infections, although the reason for the chronic inammatory CSF response is unclear. MRI is more accurate than CT for the diagnosis of most cases of extraparenchymal neurocysticercosis.30 Coronal and sagittal sections allow better assessment of areas infected by the parasites. Lesions may rst appear as multilobed cysts occupying the full space of the CSF cistern. With further growth, the anatomy of that cistern is disturbed and adjacent parenchymal structures get compressed (gure 2). Cerebral infarctions may also be imaged by either CT or MRI in patients with cysticercotic arachnoiditis when an artery at the base of the brain is occluded as the result of the inammatory reaction surrounding the parasites.79 Subarachnoid or sylvian neurocysticercosis will progress if not treated with cysticidal drugs. However, physicians should be aware that the management of intracranial hypertension, when present, is the priority. In patients with both hydrocephalus and intracranial cysts, cysticidal drugs should be used only after a ventricular shunt has been placed to avoid further increases of the intracranial pressure as a result of drug therapy. Cysticidal drugs must be used with caution in patients with giant subarachnoid cysts because the inammatory reaction developed by the host in response to the destruction of parasites may occlude leptomeningeal vessels surrounding the cyst.80 In such cases, treatment with steroids is mandatory to avoid the hazard of a cerebral infarct.

Figure 4: Treatment of neurocysticercosis with ABZ Odds ratios for lesion disappearance (top) and seizure relapse (bottom) in randomised trials in patients with single enhancing lesions treated with ABZ compared to placebo (three trials) or prednisone (three trials, one had both types of controls). *Included some patients with two enhancing lesions; data for patients with a single enhancing lesion only.

Ventricular cysticercosis
The manifestation of intraventricular cysts depends on the involved ventricle, and is more severe if the fourth ventricle is occupied.81 Contrast-enhanced MRI is the examination of choice to rule out the existence of live cysts in the ventricles or basal cisterns. Hydrocephalus can develop when CSF transit is blocked by parasitic membranes.30 In patients with ventricular cysts, the therapeutic approach with cysticidal drugs should be personalised. Although albendazole successfully destroys many ventricular cysts, the inammatory reaction surrounding those cysts may cause acute hydrocephalus.81 Neuroendoscopic excision is a promising alternative in cysticercosis of the lateral or third ventricles,8284 although it is used less in fourth ventricle cysts, for which microsurgery or antiparasitic treatment are still used in most centres. In the absence of ependymitis, ventricular shunts are not needed after removal of a ventricular cyst in most cases. By contrast, shunt placement should follow or even precede the excision of ventricular cysts associated with ependymitis.
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the meninges leading to arachnoiditis.27,77 Cysts in the convexity of the cerebral hemispheres behave as intraparenchymal cysts. The other locations are discussed separately below.

Basal subarachnoid neurocysticercosis or cysticercosis of the sylvian ssure

When cysts are located outside the brain parenchyma they tend to grow irregularly and trigger a more severe inammatory response. Giant cysts typically develop in areas where more space is available, like the sylvian ssure or the CSF cisterns at the base of the brain, and mostly behave as benign tumours due to their persisting growth.49 By far the worst prognosis is associated with basal subarachnoid neurocysticercosis, in which vesicles in CSF cisterns grow in a very disorganised way, inltrate neighbouring structures, and are associated


Other locations
Patients with intrasellar cysticerci present with ophthalmic and endocrinological disturbances similar to those produced by pituitary tumours.85 Spinal cysticercosis, mostly extramedullary, presents with root pain or motor and sensory decits that vary according to the level of the lesion,86 and is most common in the cervical segments.8789 Ophthalmic cysticercosis is not rare in endemic countries and is found in any of the eye chambers (most commonly the retina or vitreous), causing a decrease of visual acuity or visual eld defects.90,91 Massive cysticercal infection of striated muscles may produce generalised weakness associated with progressive muscle enlargement.1,92
Search strategy and selection criteria References for this review were identied by searches of MEDLINE between 1969 and 2005, Old-MEDLINE since 1949, and references from relevant articles; numerous articles were also identied through searches of the extensive les of the authors. The search terms cysticercosis, neurocysticercosis, Taenia solium, albendazole, praziquantel, and epilepsy were used. Papers published in English, Spanish, or Portuguese were reviewed. The nal reference list was generated on the basis of originality and relevance to the topics covered in the review.

Epidemiology and control

Despite occasional scepticism,93 most clinicians agree that neurocysticercosis is the main cause of acquired epilepsy in low-income countries and probably in the world.9496 The prevalence of epilepsy in endemic countries is clearly higher than in North America or Europe, although this could be due to other factors including prenatal and delivery care, or other infections.94 Recently, several articles from different countries in South and Central America consistently showed an association between around 30% of all seizures and cysticercosis.23,24,97 Neurocysticercosis is potentially eradicable, and several attempts to control it in eld conditions have been tried.15,98103 Farmers use the examination of the tongue of the pigs, a time-honoured technique that detects most animals with heavy infections, to take them to clandestine meat commercialisation circuits (bypassing formal slaughterhouse systems).15 Mass human chemotherapy to eliminate the tapeworm stage has been tried in Ecuador, Mexico, Guatemala, Honduras, Peru, and other countries.99103 Most of these programmes achieved only a temporal decrease in the prevalence of cysticercosis (measured in the pig population, which is the most sensitive and practical indicator),15 and returned to preintervention levels soon after the control pressure was interrupted. A wide-based programme to eliminate cysticercosis in a province of Peru is underway, funded by the Bill and Melinda Gates Foundation. Major obstacles include the lack of basic sanitary facilities in endemic areas, the extent of domestic pig raising, the costs of the interventions, and most importantly, their cultural acceptability.

have a single degenerating cysticercus, whereas in Latin America multiple viable cysts are common. To dene and to recognise these main clinical presentations is central to the understanding of results of serological tests and to appropriate medical and surgical treatment. Appropriate management of intracraneal hypertension or epileptic syndromes is the main aim of management of patients with neurocysticercosis. Current evidence favours the use of antiparasitic drugs in most patients with viable or degenerating lesions, although in patients with cysticercotic encephalitis this approach is counterindicated and in those with calcied lesions it is unnecessary. In rural endemic communities, neurocysticercosis seems to be symptomatic in only a few cases, but the disease is an important cause of seizures because the prevalence of infection is so high. Neurocysticercosis is potentially eradicable, and control or eradication programmes are urgently needed to reduce the burden of this disease.
Acknowledgments Research grants P01 AI51976, U01 AI35894, and TW05562 from the US National Institutes of Health, 01107 from the US Food and Drug Administration, 063109 from The Wellcome Trust, UK, and 23981 from The Bill and Melinda Gates Foundation, USA, funded other cysticercosis research by one of the authors (HHG). The sponsors had no role in the design or writing of this manuscript. Figure 1 was kindly provided by Dr A E Gonzalez. Authors contribution Both authors contributed equally. Conicts of interest We have no conicts of interest. References 1 Garcia HH, Gonzalez AE, Evans CAW, Gilman RH, The Cysticercosis Working Group in Peru. Taenia solium cysticercosis. Lancet 2003; 362: 54756. 2 Del Brutto OH, Sotelo J, Roman GC. Neurocysticercosis: a clinical handbook. Lisse: Swets and Zeitliger, 1997. 3 Garcia HH, Del Brutto OH, Nash TE, White AC Jr, Tsang VC, Gilman RH. New concepts in the diagnosis and management of neurocysticercosis (Taenia solium). Am J Trop Med Hyg 2005; 72: 39. 4 Roman G, Sotelo J, Del Brutto O, et al. A proposal to declare neurocysticercosis an international reportable disease. Bull World Health Organ 2000; 78: 399406. 5 Ong S, Talan DA, Moran GJ, et al. Neurocysticercosis in radiographically imaged seizure patients in US emergency departments. Emerg Infect Dis 2002; 8: 60813.

Cysticercosis is a major cause of epileptic seizures in most developing countries. Although there is little information on its natural history, most types and presentations of neurocysticercosis (depending on the stage, number, location and size of the parasites, as well as on the immune response of the host) are well dened. For example, on the Indian subcontinent, most patients
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