Guideline sources The following summarized guidelines for the evaluation and management of neurocysticercosis are prepared by our editorial team based on guidelines from the Infectious Diseases Society of America (IDSA 2018). 1 2 2 3 4 5 Definition Neurocysticercosis is a helminthic neurological infection that characteristically manifests as acquired epilepsy. 2 Epidemiology Neurocysticercosis is caused by the tapeworm Taenia solium, and is acquired via the fecal-oral route, by ingestion of Taenia solium eggs found in the feces of an infected human carrier. Taenia solium is endemic in most low-income countries where pigs are raised. After eggs are ingested, they hatch in the intestine and release oncospheres, which penetrate the intestinal wall. Among other organs, oncospheres travel to the brain, where they develop into cysticerci and lead to neurocystercosis. 2 Pathophysiology The incidence of neurocysticercosis in the United States is estimated at 0.2-0.6 cases per 100,000 person-years. In the Hispanic population, the incidence is estimated at 1.5-5.8 cases per 100,000 person-years. 3 https://web.pathway.md/diseases/recxAzJUG68jxHZiT 1/4 6/30/23, 1:16 AM Neurocysticercosis Pathway Disease course Although no pathognomonic clinical picture exists, adult-onset seizures in patients living in endemic regions are highly suggestive of neurocysticercosis. Symptomatic clinical manifestations range from recurrent seizures, focal neurological deficits, increased ICP, cognitive decline, and ocular symptoms. 4 Prognosis and risk of recurrence In the United States, the case fatality rate from neurocystercosis is estimated at 2.2%. 5 Guidelines 1. Diagnostic investigations Screening for latent tuberculosis: Consider screening for latent tuberculosis infection in patients likely to require prolonged corticosteroids. C Consider screening or empiric therapy for Strongyloides stercoralis in patients likely to require prolonged corticosteroids. C Initial evaluation: Obtain a full history, physical exam, and neuroimaging as part of the initial evaluation of patients with suspected neurocystercosis. Recognize that the two common clinical presentations of neurocystercosis are seizures and increased ICP. Fundoscopy: obtain a fundoscopic examination prior to initiation of anthelminthic therapy in all patients with neurocystercosis. B Neuroimaging: obtain both brain MRI and non-contrast CT scan to classify newly diagnosed patients. B Serologic testing: Obtain serologic testing with enzyme-linked immunotransfer blot as a confirmatory test in patients with suspected neurocystercosis. B Avoid ELISAs using crude antigen due to poor sensitivity and specificity. D 2. Medical management Monitoring for treatment-related adverse effects: monitor for hepatotoxicity and leukopenia in patients receiving treatment with albendazole for > 14 days. B Antiepileptic drugs: administer anti-epileptic drugs to all patients with seizures. B https://web.pathway.md/diseases/recxAzJUG68jxHZiT 2/4 6/30/23, 1:16 AM Neurocysticercosis Pathway 3. Specific circumstances Pregnant patients: consider deferring antihelminthic therapy until after pregnancy. C Patients with intraventricular neurocysticercosis in the lateral and third ventricles: remove the cysticerci by minimally invasive neuroendoscopy. B Show 2 more Pediatric patients: no evidence is available that management of neurocysticercosis in children should be different than in adults with the same form of disease. Offer weight-based dosing. B Patients with ocular cysticercosis: consider surgical removal of intraocular cysticerci rather than treatment with anti-parasitic drugs. C Patients with spinal neurocysticercosis: administer corticosteroid treatment for patients with evidence of spinal cord dysfunction or as adjunctive therapy along with anti-parasitic therapy. B Patients with hydrocephalus or diffuse cerebral edema: implement management of elevated ICP alone rather than anti-parasitic treatment in patients with neurocystercosis and untreated hydrocephalus or diffuse cerebral edema. B Patients with single enhancing lesions: Consider initiating corticosteroids prior to antiparasitic therapy in patients with single enhancing lesions. B Consider albendazole rather than no antiparasitic therapy in all patients with single enhancing lesions. C Patients with viable intraparenchymal neurocysticercosis: initiate anti-parasitic drugs in all patients with viable parenchymal neurocystercosis. B Show 3 more Patients with subarachnoid neurocysticercosis: Administer anti-inflammatory therapy prior to initiating anti-parasitic drugs. B Administer anti-parasitic drugs in patients with subarachnoid cysts. B Patients with calcified parenchymal neurocysticercosis: Consider obtaining brain MRI in patients with seizures or hydrocephalus and only calcified parenchymal neurocysticercosis on CT. C Administer symptomatic therapy alone instead of anti parasitic drugs. B Patient with multiple enhancing lesions: treat patients with multiple enhancing lesions and seizures initially with antiepileptic drugs, antiparasitic therapy, and corticosteroids as outlined in the section on viable parenchymal cysticerci. B 4. Follow-up and surveillance Management of persistent cysts: consider retreatment with antiparasitic therapy for parenchymal cystic lesions persisting for 6 months after the end of the initial course of therapy. C https://web.pathway.md/diseases/recxAzJUG68jxHZiT 3/4 6/30/23, 1:16 AM Neurocysticercosis Pathway Screening of household contacts: consider screening for tapeworm carriage in the household members of patients with neurocysticercosis who probably acquired neurocysticercosis in a nonendemic area. C Serial imaging assessment: repeat MRI at least every 6 months until resolution of cystic components. B References 1. White AC Jr, Coyle CM, Rajshekhar V et al. Diagnosis and Treatment of Neurocysticercosis: 2017 Clinical Practice Guidelines by the Infectious Diseases Society of America IDSA and the American Society of Tropical Medicine and Hygiene ASTMH .1. Clin Infect Dis. 2018 Apr 3;66 8):e49-e75. Open 2. Coyle CM. Neurocysticerosis: An Individualized Approach. Infect Dis Clin North Am. 2019 Mar;33 1 153 168. Open 3. Serpa JA, White AC Jr. Neurocysticercosis in the United States. Pathog Glob Health. 2012 Sep;106 5 256 60. Open 4. Garcia HH, Nash TE, Del Brutto OH. Clinical symptoms, diagnosis, and treatment of neurocysticercosis. Lancet Neurol. 2014 Dec;13 12 1202 15. Open 5. Sorvillo FJ, Portigal L, DeGiorgio C et al. Cysticercosis-related deaths, California. Emerg Infect Dis. 2004 Mar;10 3 465 9. Open 6. Baird RA, Wiebe S, Zunt JR et al. Evidence-based guideline: treatment of parenchymal neurocysticercosis: report of the Guideline Development Subcommittee of the American Academy of Neurology. Neurology. 2013 Apr 9;80 15 1424 9. Open https://web.pathway.md/diseases/recxAzJUG68jxHZiT 4/4