Key Points
- Neurocysticercosis (NCC) is a spectrum of diseases that differ in pathogenesis and optimal management.
- Symptomatic therapyᵃ should be the focus of initial and emergency management.
- Anti-parasitic treatment is important, but never an emergency.
- Parenchymal cystic NCC has better outcomes if treated with anti-parasitic drugs along with corticosteroids.
- Subarachnoid NCC does not respond well to single anti-parasitic drugs at doses and durations used for parenchymal NCC. Optimal management may require chronic anti-inflammatory therapy, intensive anti-parasitic therapyᵇ, and surgical therapyᶜ.
- Ventricular NCC of the 3rd and lateral ventricles should be treated with minimally invasive surgery when possibleᵈ, but minimally invasive and open craniotomy are options for the 4th ventricular disease. Open craniotomy or cerebrospinal fluid (CSF) diversion along with anti-parasitic drugs are optimal in select cases. Anti-parasitic therapy should be deferred until after surgical therapy.
- Calcified lesions do not contain viable parasites and should not be treated with anti-parasitic drugs.
a Symptomatic therapy includes anti-epileptic drugs for seizures, anti-inflammatory drug such as corticosteroids and methotrexate, and surgery for hydrocephalus
b Anti-parasitic therapy for subarachnoid NCC may include prolonged courses of albendazole, high dose albendazole, or combinations of praziquantel and albendazole
c Surgical therapy for subarachnoid NCC may include CSF diversion for hydrocephalus or minimally invasive surgical debulking
d Adherent cysticerci should be managed with CSF diversion along with anti-parasitic drugs. Open craniotomy is effective for 4th ventricular lesions and the choice of approaches should depend on local surgical expertise.
Definition of Terms
| Terms | Definition |
|---|---|
| Taeniasis (also termed taeniosis) | Infestation of the human intestines with the tapeworm form |
| Cysticercosis | Infection of the tissues with the larval cyst (or metacestode) |
| Neurocysticercosis | Cysticercosis involving the central nervous system, including the brain parenchyma, ventricles, basilar cisterns, sulci, gyri, spine, and retina |
Diagnosis
Manifestations of Neurocysticercosis
| Locationᵃ | Symptoms |
|---|---|
| Parenchymal | Seizures or headache |
| Ventricular | Obstructive hydrocephalus |
| Subarachnoid | Communicating hydrocephalus, meningitis, stroke, or focal neurologic findings |
a Mixed forms are common.
- While there is a wide range of clinical manifestations of neurocysticercosis, the 2 most common clinical presentations are with seizures and increased intracranial pressure (fact no grade).
- Initial evaluation should include careful history and physical examination, and neuroimaging studies (fact no grade).
- The Panel recommends serologic testing with enzyme-linked immunotransfer blot as a confirmatory test in patients with suspected neurocysticercosis (S-M). ELISA tests using crude antigen should be avoided due to poor sensitivity and specificity (S-M).
- The Panel recommends both a brain MRI and a non-contrast CT scan for classifying patients with newly diagnosed neurocysticercosis (S-M).
- The Panel suggests screening for latent tuberculosis infection in patients likely to require prolonged corticosteroids (W-L).
- The Panel suggests screening or empiric therapy for Stronglyoides stercoralis in patients likely to require prolonged corticosteroids (W-L).
- The Panel recommends that all patients with NCC undergo a fundoscopic examination prior to initiation of anthelminthic therapy (S-M).
- The Panel suggests that the patient with NCC who has probably acquired NCC in a non-endemic area have their household members screened for tapeworm carriage (W-L).
Remark: This is a public health issue and can often be addressed by the local health department.
S, strong; W, weak; H, high; M, moderate; L, low; VL, very low quality of evidence; GP, good practice; NR, no recommendation
