NEUROCYSTICERCOSIS Komal Antil Master of Sciences in Nursing (Neurosciences Nursing) All India Institute of Medical Sciences, New Delhi
Introduction Neurocysticercosis (NCC) is the infection of the CNS by the larval stage of the pork tapeworm Taenia solium : Infection may develop in any organ- CNS (parenchyma, subarachnoid spaces, ventricles and spinal cord), eyes and muscles are the most commonly involved. The most common manifestation is epilepsy but can have several other neurological manifestations.
Epidemiology NCC is endemic in most Latin American countries, sub-Saharan Africa, and large regions of Asia (including the Indian subcontinent and China). Rare in developed countries, can occur in travellers/immigrants. A study from North India found a point prevalence of NCC - 4.5/1000. In pig farming communities it is up to 15 %. The proportion of NCC among patients with epilepsy is estimated @ 29 %. In those with partial seizures, it was >50 %.
Life Cycle of Taenia Solium ✔ Taenia solium requires two hosts to complete its life cycle. ✔ Pigs ( intermediate host) contain the cysticerci, primarily in muscle. ✔ Humans ( definitive host) infected by consuming undercooked pork containing live T.solium cysticerci. ✔ Cysticerci develop into adult tapeworm which releases eggs in human faeces. ✔ Eggs contaminate soil/vegetation, when ingested by pigs/humans eggs develop into larvae which pass through the intestinal mucosa and reach various tissues.
Growth stages of Taenia solium: A-Infective T.solium eggs B-Larva or cysticercus C-Evaginating cysticercus D-Tapeworm scolex E-Tapeworm strobila
Modes of infection Sources of infection- persons with Taeniasis (acquired from pork). Transmission ways - not spread from person to person directly persons with Taeniasis will shed tapeworm eggs in their bowel movements Infection can happen by accidentally swallowing pork tapeworm eggs Through- drinking contaminated water or food by putting contaminated fingers to mouth (external autoinfection) by internal autoinfection
Disease spectrum of T. Solium ✔ Taeniasis = adult tapeworm in the small intestine • Usually asymptomatic (eggs or proglottids in faeces) • Vague abdominal symptoms ✔ Cysticercosis = T. solium larvae in human tissues (eg. muscle) • Usually asymptomatic • Painless subcutaneous nodules in arms and chest ✔ Neurocysticercosis (NCC) = cysts in the central nervous system • Most severe manifestation
Types of Neurocysticercosis 1) Intraparenchymal NCC The most common form is seen at the grey-white matter junction . Single or Multiple. Range in size from a few mm to 1 to 2 cm. Commonly seen in children >5 years but can occur in toddlers and infants. Seizures are the most common manifestation of intraparenchymal NCC. 1/3 rd may have associated headache and vomiting. Papilledema occurs in 2 to 7 %. Neurological deficits saw in 4 to 6 %. Seizures respond well to monotherapy. Cysticercus Encephalitis - results from a large number of cysticerci in brain parenchyma with diffuse inflammation and edema.
The parenchymal cysts evolve through 4 stages- V esicular cyst Colloidal stage Granular nodular stage Nodular calcified stage
2. Extraparenchymal NCC Ventricular NCC - can obstruct CSF flow causing hydrocephalus. CT may reveal only hydrocephalus and no cysticerci. Subarachnoid NCC - can occur in the gyri, fissures, and basilar cisterns.
Other forms of Cysticercosis Spinal Cysticercosis (intramedullary/extramedullary) Ocular Cysticercosis In Muscles In Subcutaneous tissue Other organs
Clinical Manifestations ✔ Asymptomatic ✔ The most common manifestation is Seizure (focal, secondary generalized or generalized). (80%) ✔ Headache is common (unilateral/bilateral)- may reflect raised ICT or vasculitis. ✔ Focal neurological deficits (16%) ✔ Symptoms/signs of raised ICT-nausea, vomiting, altered mental status, visual changes, dizziness, cerebral edema. (12%) ✔ Neurocognitive defects- learning disability, psychosis, depression. (5%)
Imaging CT ✔ Parenchymal cysts- usually appear as single, small (<20mm) with ring/disk enhancement and eccentric hyperdense scolex. Multiple lesions give a ‘starry sky’ appearance. (colloidal stage). ✔ Enhancement indicates inflammation. Live vesicular cysts are non-enhancing. ✔ Extra-parenchymal cysts- may show hydrocephalus, enhancement of tentorium and basal cisterns and occasionally infarcts.
Imaging MRI ✔ Superior to CT in detecting cysts in ventricles, posterior fossa, brainstem, And small cysts. ✔ Small calcified lesions may be missed. ✔ Magnetization transfer images (MT) and magnetization transfer ratio (MTR), recovery (FLAIR) and fast imaging employing steady-state acquisition (FIESTA) sequences for lesions not visible on routine MRI.
Serological Tests ✔ Seropositivity depends on parasite load and endemicity. False positive and false negative results can occur. ✔ False-negative result: - Single lesions - Calcification ✔ False-positive: - Other parasitic infections - The high percentage of false positive for patients from endemic area
Serological Tests ✔ EITB assay- uses lentil lectin purified glycoprotein antigens (LLGP) to detect antibodies to T soliumin in serum. Sensitivity 98% (multiple parasites), 50-70 % (solitary cysticercus). ✔ Detection of anticysticercal antibodies in the CSF by ELISA. ✔ Detection of circulating parasitic antigens in serum by ELISA with monoclonal antibodies is experimental. ✔ In patients with a reliable diagnosis of NCC by imaging studies, the immunological test is not required, since a negative test will not discard an NCC.
Other lab tests... ✔ Eosinophilia may occur. ✔ CSF- - Usually done to rule out other causes. - Can be normal in inactive disease. - Moderate pleocytosis (mostly mononuclear cells; upto 300/mm 3 ), increased protein (50-300 mg/dl). Correlate with disease activity and whether or not the parasites are located in sub-arachnoid space.
Other lab tests... ✔ Biopsy of subcutaneous nodules. ✔ Radiographs of skeletal muscles. ✔ Specific coproantigen detection by ELISA for screening for T solium carriers. Stool examination for T.solium eggs has poor sensitivity.
Diagnostic Criteria Definitive diagnosis- one absolute criterion or two major plus one minor and one epidemiologic criteria Probable diagnosis- one major plus two minor criteria, one major plus one minor and one epidemiologic criteria, three minor plus one epidemiologic criteria.
Management Before the treatment, the viability of the cyst and the location of the parasite is to be confirmed. Treatment consists of : Symptomatic Specific anti cercus agent Surgical treatment
Management Emergency care ✔ Manage seizure activity ✔ Supportive care (A-B-C) ✔ Monitor, and correct metabolic abnormalities ✔ Anticonvulsants are effective. ✔ Evidence of increased ICP- Steroids, osmotic agents, and/or diuretics ✔ Initiate proper diagnostic procedures Blood work and imaging
Intraparenchymal NCC Symptomatic treatment, anti-parasite treatment or surgery ✔ Calcified cysts only - antiepileptic, analgesic, and anti-inflammatory drugs; for seizure relapses, repeat imaging looking for peri-calcification oedema. AED for at least 2yrs since the last seizure. No anti-parasite drugs. ✔ One or more cystic or degenerating lesions - antiepileptic, analgesic, and anti-inflammatory drugs; antiparasitic treatment under hospital conditions with steroid treatment. Discontinue AED in single lesions after they resolve (without calcification). *Level 1 evidence ✔ Cysticercus encephalitis - Manage intracranial hypertension; do not use antiparasitic drugs.
✔ Asymptomatic parenchymal lesions • Prophylactic AED is not justified in calcified lesions without seizures. • Viable cysts- Give prophylactic AED when antiparasite treatment is also planned. ✔ Repeat neuroimaging after 3-6 m to document lesion resolution. Repeat the course of cysticidal therapy if persistent lesion.
Drugs: Albendazole - 15mg/kg/day in 2-3 divided doses for 2-4 weeks. Shorter courses of 3 to 14 days tried in single lesions. Praziquintal - 50mg/kg/day, less effective than albendazole. Combinations of two antiparasitic drugs- increased cyst clearance in multiple lesions. Steroids- - Dexamethasone - 0.1 mg/kg/day i.v starting one day before antiparasite drugs, give for 1 to 2 weeks then taper. - Prednisolone- 1 to 2 mg/kg/day.
Extraparenchymal NCC
Management Other forms of NCC ✔ Spinal- Intramedullary cysts are treated with surgery, and albendazole with steroids is being tried. Subarachnoid cysts can migrate so imaging is done just before surgery. ✔ Ocular - Surgical management is the standard of care.
Indications for Surgery Extra-parenchymal NCC Intraventricular cysts Hydrocephalus due to racemose cysts Hydrocephalus due to ependymitis caused by NCC Spinal NCC Extramedullary Intramedullary
Nursing Management Observe the patient’s condition, vital signs and symptoms of increased intracranial pressure. Provide seizure precautions. Anticipate behavioural change and observe it. Give health education about disease conditions, treatment and prevention.
Prognosis ✔ Single lesions- good prognosis, disappears in >60%. ✔ Seizure recurrence is 10-20% with single lesions, multiple lesions have more frequent seizures. ✔ The prognosis is poorer in cysticerci encephalitis and extra parenchymal NCC.
Prevention ✔ T solium infection is one of a few diseases targeted for focal elimination and eventual eradication by the International Task Force for Disease Eradication. ✔ Public education, proper hygiene, and provision of toilets. ✔ Safe handling of meat, strict animal husbandry and meet inspection procedures. ✔ Mass deworming of the population with Niclosamide or Praziquantel. ✔ Mass vaccination of pigs and treatment of pigs with Oxfendazole. ✔ Community interventions reduce the rate of epilepsy in hyper-endemic areas.
Conclusion ✔ NCC is a common cause of seizures and other neurological manifestations and needs to be considered in D/D of many neurological conditions. ✔ Treatment with cycticidal therapy leads to a reduction in seizure frequency and faster resolution of lesions. ✔ Children with a single or few lesions have a good outcome. ✔ Prevention of NCC is important and feasible.