Armando E. Gonzalez

Proposed diagnostic criteria for neurocysticercosis.

Neurocysticercosis is the most common helminthic infection of the CNS but its diagnosis remains difficult. Clinical manifestations are nonspecific, most neuroimaging findings are not pathognomonic, and some serologic tests have low sensitivity and specificity. The authors provide diagnostic criteria for neurocysticercosis based on objective clinical, imaging, immunologic, and epidemiologic data. These include four categories of criteria stratified on the basis of their diagnostic strength, including the following: 1) absolute—histologic demonstration of the parasite from biopsy of a brain or spinal cord lesion, cystic lesions showing the scolex on CT or MRI, and direct visualization of subretinal parasites by funduscopic examination; 2) major—lesions highly suggestive of neurocysticercosis on neuroimaging studies, positive serum enzyme-linked immunoelectrotransfer blot for the detection of anticysticercal antibodies, resolution of intracranial cystic lesions after therapy with albendazole or praziquantel, and spontaneous resolution of small single enhancing lesions; 3) minor—lesions compatible with neurocysticercosis on neuroimaging studies, clinical manifestations suggestive of neurocysticercosis, positive CSF enzyme-linked immunosorbent assay for detection of anticysticercal antibodies or cysticercal antigens, and cysticercosis outside the CNS; and 4) epidemiologic—evidence of a household contact with Taenia solium infection, individuals coming from or living in an area where cysticercosis is endemic, and history of frequent travel to disease-endemic areas. Interpretation of these criteria permits two degrees of diagnostic certainty: 1) definitive diagnosis, in patients who have one absolute criterion or in those who have two major plus one minor and one epidemiologic criterion; and 2) probable diagnosis, in patients who have one major plus two minor criteria, in those who have one major plus one minor and one epidemiologic criterion, and in those who have three minor plus one epidemiologic criterion.

Taenia solium cysticercosis

Cysticercosis, the infection of the larval tissue stage of the pork tapeworm, Taenia solium, is endemic to most developing countries and increasingly is seen in industrialized countries because of immigration. Human infection is a major cause of acquired epilepsy, and porcine infection causes important economic losses to farm workers. Clinical manifestations vary because of the variable number, size, and location of the cysts and the immune response of the host. Diagnosis is based on imaging techniques (CT and MR imaging) and serology (immunoblot). Current research efforts are centered on control and eradication of the taeniasis cysticercosis complex in endemic areas.

Prevention and control of cystic echinococcosis

Human cystic echinococcosis (hydatid disease) continues to be a substantial cause of morbidity and mortality in many parts of the world. Elimination is difficult to obtain and it is estimated that, using current control options, achieving such a goal will take around 20 years of sustained efforts. Since the introduction of current (and past) hydatid control campaigns, there have been clear technological improvements made in the diagnosis and treatment of human and animal cystic echinococcosis, the diagnosis of canine echinococcosis, and the genetic characterisation of strains and vaccination against Echinococcus granulosus in animals. Incorporation of these new measures could increase the efficiency of hydatid control programmes, potentially reducing the time required to achieve effective prevention of disease transmission to as little as 5-10 years.

Current Consensus Guidelines for Treatment of Neurocysticercosis

SUMMARY Taenia solium neurocysticercosis is a common cause of epileptic seizures and other neurological morbidity in most developing countries. It is also an increasingly common diagnosis in industrialized countries because of immigration from areas where it is endemic. Its clinical manifestations are highly variable and depend on the number, stage, and size of the lesions and the hosts immune response. In part due to this variability, major discrepancies exist in the treatment of neurocysticercosis. A panel of experts in taeniasis/cysticercosis discussed the evidence on treatment of neurocysticercosis for each clinical presentation, and we present the panels consensus and areas of disagreement. Overall, four general recommendations were made: (i) individualize therapeutic decisions, including whether to use antiparasitic drugs, based on the number, location, and viability of the parasites within the nervous system; (ii) actively manage growing cysticerci either with antiparasitic drugs or surgical excision; (iii) prioritize the management of intracranial hypertension secondary to neurocysticercosis before considering any other form of therapy; and (iv) manage seizures as done for seizures due to other causes of secondary seizures (remote symptomatic seizures) because they are due to an organic focus that has been present for a long time.

Calcific neurocysticercosis and epileptogenesis

Neurocysticercosis is responsible for increased rates of seizures and epilepsy in endemic regions. The most common form of the disease, chronic calcific neurocysticercosis, is the end result of the host’s inflammatory response to the larval cysticercus of Taenia solium. There is increasing evidence indicating that calcific cysticercosis is not clinically inactive but a cause of seizures or focal symptoms in this population. Perilesional edema is at times also present around implicated calcified foci. A better understanding of the natural history, frequency, epidemiology, and pathophysiology of calcific cysticercosis and associated disease manifestations is needed to define its importance, treatment, and prevention.

Magnitude of the disease burden from neurocysticercosis in a developing country

Cysticercosis contributes to higher epilepsy rates in developing countries than in industrialized ones, yet no estimate exists for the associated burden of disease. We used epidemiological data on neurocysticercosis in Peru to calculate the burden of disease and applied our model to the other countries of Latin America where neurocysticercosis is endemic to determine a regional estimate. Analysis of 12 population-based community studies demonstrated that neurocysticercosis was endemic in highland areas and high jungles, with seroprevalences from 6% to 24%. In one community, the adult seizure disorder rate was 9.1% among seropositive persons versus 4. 6% among seronegative persons; we used this difference for estimates. On the basis of average prevalence rates in areas of endemicity of 6%-10%, we estimated that there are 23,512-39,186 symptomatic neurocysticercosis cases in Peru. In Latin America, an estimated 75 million persons live in areas where cysticercosis is endemic, and approximately 400,000 have symptomatic disease. Cysticercosis contributes substantially to neurological disease in Peru and in all of Latin America.

Neurocysticercosis: Association between seizures, serology, and brain CT in rural Peru

Background:Neurocysticercosis (NCC) is the commonest helminthic CNS infection and the main cause of adult-onset seizures in developing countries, also frequent in industrialized countries because of immigration from endemic zones. Although NCC is commonly seen in individuals with seizures in endemic areas, its role as a cause of epilepsy has been questioned on the basis of the poor methodology of published studies. Objective:To determine, in a cysticercosis-endemic area of the northern Peruvian coast, the frequency of 1) epileptic seizures, 2) serum antibodies to Taenia solium, 3) NCC-compatible findings on brain CT, and 4) the associations between these variables. Methods:A community-wide screening survey for possible seizure cases was performed using a validated questionnaire. Positive respondents were later examined in the field by neurologists. Seizure cases were categorized as single seizure, active epilepsy, or inactive epilepsy. Serology was performed for all consenting individuals using immunoblot. Noncontrast brain CT scans were performed in all individuals with seizures and two groups of control subjects without seizures (seropositive and seronegative). Results:The screening survey was applied to 903 permanent residents. Most positive respondents (114/137 [83.2%]) were examined by neurologists. The overall prevalence of epilepsy was 32.1 per 1,000 and that of active epilepsy was 16.6 per 1,000. Seroprevalence was 24.2% (200/825). Seroprevalence was associated with seizures (odds ratio 2.14; p = 0.026). Brain CT abnormalities compatible with NCC were more frequent in individuals with seizures and in those seropositive. Conclusion:In this hyperendemic area, an important proportion of seizure cases are associated with neurocysticercosis as demonstrated by serology or brain CT.

Treatment of neurocysticercosis: Current status and future research needs

Here we put forward a roadmap that summarizes important questions that need to be answered to determine more effective and safer treatments. A key concept in management of neurocysticercosis is the understanding that infection and disease due to neurocysticercosis are variable and thus different clinical approaches and treatments are required. Despite recent advances, treatments remain either suboptimal or based on poorly controlled or anecdotal experience. A better understanding of basic pathophysiologic mechanisms including parasite survival and evolution, nature of the inflammatory response, and the genesis of seizures, epilepsy, and mechanisms of anthelmintic action should lead to improved therapies.

Epilepsy and Neurocysticercosis in Atahualpa: A Door-to-Door Survey in Rural Coastal Ecuador

Summary:  Purpose: To determine the prevalence of epilepsy and the role of neurocysticercosis in the occurrence of epilepsy in Atahualpa.

Perilesional brain oedema and seizure activity in patients with calcified neurocysticercosis: a prospective cohort and nested case–control study

BACKGROUND Cysticercosis due to Taenia solium is a cause of adult-acquired seizures and epilepsy even in patients with only calcified larval cysts. Transient perilesional brain oedema is seen around the calcified foci but its importance, association with seizures, incidence, and pathophysiology are unknown. METHODS 110 patients with only calcified lesions and a history of seizures or severe headaches were followed prospectively in a cohort design to assess the incidence of seizure relapse. In a nested case-control substudy, perilesional oedema was assessed by MRI at the time of seizure in symptomatic patients and in matched asymptomatic controls taken from the study population. FINDINGS Between November, 1999, and December, 2006, 29 patients had an incident seizure during a median follow up of 32.33 (SD 19.99) months, with an estimated 5-year seizure incidence of 36% (95% CI 25% to 49%). 24 of 29 (83%) patients with seizure relapse had an MRI evaluation within 5 days of the event; perilesional oedema was seen in 12 patients (50%) compared with two (9%) of 23 asymptomatic matched controls. INTERPRETATION Perilesional oedema is common and associated with episodic seizure activity in patients with calcified neurocysticercosis. Our findings are probably representative of symptomatic patients in regions where T solium neurocysticercosis is endemic and suggest a unique and possibly preventable cause of seizures in this population.