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)

FIGURE 1. Approach and implications to rating the quality of evidence and strength of recommendations using the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) methodology (unrestricted use of the figure granted by the U.S. GRADE Network).
Parenchymal  Nonviable calcified NCC (CPN) Nodular calcifications less than 20 mm in diameter (often 1-5 mm) with or without surrounding edema and/or contrast enhancement.
Calcified granuloma with or without surrounding inflammation and/or gliosis.
Single, small enhancing (single enhancing lesions, SEL)
Cystic or nodular enhancing lesion < 2 cm in size.
Single parenchymal parasites in the process of degeneration with surrounding inflammation and variable opacification or absence of the cyst fluid. Viable parenchymal NCC Vesicular lesions often with evidence of associated contrast enhancement and/ or surrounding edema. The scolex is often visible on high-definition imaging. NCC = neurocysticercosis. * Patients with more than one form are classified with the form found lower on the chart with the exception that SEL that are also viable are grouped with SEL. Ocular cysticercosis is classified separately.
 Refers to cysticerci in the brain parenchyma. Small cysticerci in the gyri over the cerebral convexity behave clinically like parenchymal cysticerci and are grouped with parenchymal cysticerci. Rare forms of NCC include multiple inflamed parenchymal cysticerci with diffuse cerebral edema, termed cysticercal encephalitis, large parenchymal cysticerci (> 20 mm). * Two well-designed randomized trials demonstrated more rapid radiologic responses and fewer generalized seizures in patients treated with albendazole compared with placebo.  The combination of praziquantel and albendazole was superior to albdendazole alone in patients with more than two cysts, but was not better in those with one or two viable cysts.  The optimal anti-inflammatory regimen has not been clearly defined. A trial comparing 6 mg/day of dexamethasone for 10 days with 8 mg/day for 28 days followed by a taper noted fewer seizures in the higher dose group. Other studies have used prednisone 1-1.5 mg/kg/day during therapy.  There are no clear data on optimal duration of antiepileptic drugs. Risk factors for recurrent seizures include calcifications on follow-up CT, breakthrough seizures, and > 2 seizures during the course of the disease. In patients without any of these risk factors and no seizures in the prior 3 months, AEDs can be safely withdrawn within a few weeks of the resolution of the SCG on high resolution imaging studies.
k Two meta-analyses of randomized controlled trials have concluded that albendazole improved the outcome in patients with SEL due to NCC. { Albendazole should be given along with anti-inflammatory drugs. The optimal dose and duration has not been defined, but doses have included dexamethasone 0.1 mg/kg/day for the duration of therapy or 1-2 mg/kg/day of prednisone or prednisolone have been used.

# Management guidelines are similar to that in other patients with seizures. Many can be managed with a single drug. There are no data on relative efficacy of different antiepileptic drugs. ** A few case reports suggest when corticosteroids are lowered or stopped, rebound perilesional edema can occur. Therefore, anti-inflammatory drugs should be used cautiously, if at all, in patients presenting with perilesional edema around a calcified lesion.
  Antiparasitic agents can worsen cerebral edema and should generally be avoided in patients with increased intracranial pressure from either diffuse cerebral edema (cysticercal encephalitis) or untreated hydrocephalus. * The endoscopic surgical approach often requires ventriculomegaly. Cyst rupture is the norm and not associated with adverse consequences. The microsurgical approach is also facilitated by the presence of hydrocephalus.
 Alternative approaches include 1) CSF diversion along with medical management or 2) craniotomy with microsurgical excision.  Shunts are initially efficacious acutely for hydrocephalus, but there is a very high rate of shunt malfunction in patients with NCC. Shunt failure may be lower when combined with corticosteroids and antiparasitic treatment.
 SAN should be aggressively treated with antiparasitic and anti-inflammatory drugs. Hydrocephalus should be addressed before antiparasitic therapy. k Untreated hydrocephalus is a contraindication to antiparasitic therapy and needs to be treated first. Some cases respond to anti-inflammatory treatment, but most cases require CSF diversion. { Generally, 1 mg/kg/day of prednisone or 0.2-0.4 mg/kg/day of dexamethasone are administered 3-4 days before and during antiparasitic treatment. The dose is slowly decreased, depending on the intensity of the inflammatory response. Methotrexate or antibody to TNF can be used as steroid-sparing agents.
[table] TABLE 1: Classification of NCC based on location and appearance of the parasite and surrounding host tissue on neuroimaging [/table]