NEUROCYSTICERCOSIS. in Pregnancy Not Just Another Headache. About Cysticercosis

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NEUROCYSTICERCOSIS in Pregnancy Not Just Another Headache Elizabeth Gardner, DNP, RN

Chaney, EdD, RN, FNP-C, FAANP Cysticercosis is a parasitic infection caused by the

have suffered from tapeworm infestations for more than 170,000 years (Buzzle.

Even during the modern era, the pork tapeworm is a significant cause of illness,

2005). Box 1 provides a case example of a patient with cysticercosis.

Although relatively uncommon in those countries where pork is screened prior to

Ramaratnam, & Ranganathan, 2010; Garcia & Del Brutto).

Latin America, Asia and Africa (Garcia & Del Brutto, 2005; Garcia et al.

There has been a significant increase in neurocysticercosis in the United States,

(Del la Garza et al., 2005; Hawk, Shahlaie, Kim, & Theis, 2005).

neglected infection of poverty (NIP), because it s found mostly in people with

The patient in our case example (see Box 1) had lived in rural areas in Mexico before

Abstract: Infection with pork tapeworm, or Taenia solium, affects approximately 50

The most important and potentially devastating form of the infestation,

2 NEUROCYSTICERCOSIS in Pregnancy Not Just Another Headache Elizabeth Gardner, DNP, RN / Myong Chang, MSN, RN, WHNP Peggy Mancuso, PhD, CNM / Susan E. Chaney, EdD, RN, FNP-C, FAANP Cysticercosis is a parasitic infection caused by the larval stage of the pork tapeworm Taenia solium (Garcia, Gonzalez, Evans, & Gilman, 2003). Recent genetic evidence suggests that humankind and the ancestors of modern humans have suffered from tapeworm infestations for more than 170,000 years (Buzzle.com, 2011). Even during the modern era, the pork tapeworm is a significant cause of illness, disability and death, affecting 50 million people worldwide (Garcia & Del Brutto, 2005). Box 1 provides a case example of a patient with cysticercosis. When the pork tapeworm larva lodges in the human brain, neurocysticercosis develops. Although relatively uncommon in those countries where pork is screened prior to slaughter, neurocysticercosis is the leading cause of seizures in the world (Abba, Ramaratnam, & Ranganathan, 2010; Garcia & Del Brutto). About Cysticercosis Cysticercosis is most common in the underdeveloped countries of Latin America, Asia and Africa (Garcia & Del Brutto, 2005; Garcia et al., 2004; Serpa, Yancey, & White, 2006). There has been a significant increase in neurocysticercosis in the United States, especially in areas where there are large immigrant populations from endemic areas (Del la Garza et al., 2005; Hawk, Shahlaie, Kim, & Theis, 2005). The Centers for Disease Control and Prevention (CDC) considers cysticercosis to be a neglected infection of poverty (NIP), because it s found mostly in people with limited resources and limited access to medical care (CDC, 2010). The patient in our case example (see Box 1) had lived in rural areas in Mexico before she came to the United States. Abstract: Infection with pork tapeworm, or Taenia solium, affects approximately 50 million people worldwide. The most important and potentially devastating form of the infestation, neurocysticercosis, occurs when the parasite invades the central nervous system. There has been a significant increase in the number of cases in the United States due to immigration from endemic areas. This case study of a pregnant woman in the 35th week of gestation exemplifies the serious consequences of this infection in pregnancy, and discusses an evidence-based approach to the diagnosis, treatment and eradication of this preventable disease. DOI: /j X x Keywords: Taenia solium pork tapeworm cysticercosis neurocysticercosis pregnancy

3 Taenia solium infestation begins with a human tapeworm carrier who defecates T. solium eggs, which are then eaten by the pig that serves as the intermediate host of the parasite. Three to eight weeks after ingestion, small cysts (cysticeri) develop in the pig, usually in muscle or brain tissue. The human then eats the flesh of the pig, and the adult tapeworm develops and lives in the human intestine. The disease caused by infestation with the adult tapeworm in the intestine is termed taeniasis. Taeniasis is a bothersome, but not usually fatal, infestation that can be prevented by cooking pork thoroughly. In this stage, treatment is with niclosamide or praziquantel (Van Voorthis & Weller, 2010). The situation worsens when a human ingests the pork tapeworm s eggs (perhaps through self-inoculation). The larval cysticerci can develop in any human tissue, with brain or muscle tissue being the most likely site. This condition is called cysticercosis, or neurocysticercosis if the infestation is within the brain. Neurocysticercosis can be life threatening, especially if the lesions inhibit the flow of cerebrospinal fluid (see Get the Facts box for a link to a CDC graphic illustrating the life cycle of the pork tapeworm). Disease Progression During the initial phase of infection, the living tapeworm larva cysts do not cause extensive inflammation within surrounding tissues. The living tapeworm larva seems to be able to elude the human immune system response to foreign tissue invasion. When the larva cysts die, however, they degenerate and either resolve or form a calcified granuloma. Calcifications are associated with recurrent seizures in the host. The specific mechanisms of calcification-associated seizure activity are not known. Seizures may occur as a result of inflammation, intermittent parasitic antigen release or scarring (Nash et al., 2004). Calcified and cystic lesions may be present simultaneously. The highest prevalence of symptomatic neurocysticercosis occurs in developing countries, where inadequate sanitation enables pigs to access and consume human feces. Humans acquire the parasite when they ingest food contaminated with infected feces (Hawk et al., 2005). Gastrointestinal tapeworm infections occur when people consume undercooked pork containing cysticerci in swine tissues. Alternately, autoinfection, which can cause neurocysticercosis from worm larva cysts, can Elizabeth Gardner, DNP, RN, is a family nurse practitioner in Fort Worth, TX; Myong Chang, MSN, RN,WHNP, is a women s health nurse practitioner at Parkland Hospital in Dallas, TX; Peggy Mancuso, PhD, CNM, is director of the Doctor of Nursing Practice Program and an associate professor at Texas Woman s University College of Nursing in Dallas, TX; Susan E. Chaney, EdD, RN, FNP-C, FAANP, is professor at Texas Woman s University College of Nursing in Dallas, TX. The authors report no conflicts of interest or relevant financial relationships. Address correspondence to: lizbgardner@sbcglobal.net. occur via unwashed hands that facilitate fecal-oral ingestion of the worm eggs. Neurocysticercosis Neurocysticercosis is the most serious manifestation of tapeworm infestation (Carpio, 2002). Sometimes people with neurocysticercosis are asymptomatic for many years until they suddenly present with nonspecific neurologic manifestations, such as headache, confusion, ataxia, seizures and meningismus (Carpio). Fever is not typically present. The onset of symptomatic neurocysticercosis has been estimated to peak at 3 to 5 years after infection, but symptomatic neurocysticercosis can also be delayed for more than 30 years before the human host becomes symptomatic (Del la Garza et al., 2005; Garcia & Del Brutto, 2005). The most common clinical manifestation of neurocysticercosis is a seizure disorder, which affects nearly 80 percent of people with tapeworm-induced neurologic lesions (Rajshekhar, 2010). Other serious neurologic complications include intracranial hypertension, hydrocephalus, chronic meningitis and cranial nerve abnormalities (CDC, 2010b). Symptoms are varied, however, and depend on the anatomic location, size, number and stage of the larval cysts, as well as the host response to the parasite (Garcia & Del Brutto, 2005; Singh & Singh, 2004). Diagnosing Neurocysticercosis During Pregnancy During pregnancy, diagnosis of neurocysticercosis is confounded because many of its associated symptoms (headache, nausea and vomiting, reduced visual acuity, impaired consciousness, seizures) may occur as discomforts of uncomplicated pregnancy or are characteristic of pregnancy complicated by pre-eclampsia or eclampsia. Neurocysticercosis may also be misdiagnosed as meningitis or as a postdural puncture headache (Grondin, D Angelo, Thomas, & Pan, 2006). Following the varied clinical presentation of neurocysticercosis, radiographic imaging is very helpful and facilitates diagnosis (Serpa et al., 2006). Serologic tests can be helpful but are not always necessary. CT and MRI provide objective evidence concerning the number and location of cysticerci and the viability and severity of the host inflammatory reaction against the parasites (Garcia et al., 2005). CT scanning is an excellent method to detect calcifications, and, in most cases, is sufficient to make the diagnosis. It s also less expensive than MRI. MRI is useful for detecting relatively small lesions, and for evaluating degenerative changes and edema around calcified lesions (Castillo, 2004). A lumbar puncture for cerebrospinal fluid examination is usually not necessary for the diagnosis of neurocysticercosis, but may be helpful for excluding other possible differential diagnoses , AWHONN

4 should be considered if patients experience seizures during this medication-free period. Antiparasitic Medications photo by Roberto J. Galindo / Wikimedia Commons Although there are antiparasitic drugs available, their use in pregnant women remains controversial and is not often initiated. Possible benefits of antiparasitic therapy include resolution of active cysts, decreased risk of seizures following resolution and parasite death and inhibition of hydrocephalus recurrence (Nash et al., 2006). Nevertheless, antiparasitic therapy often exacerbates neurologic symptoms because the degenerating cyst promotes increased inflammation, particularly in those people with a large number of lesions. Increased neurological inflammatory processes with accompanying seizure activity can result in permanent disability or death (Garcia et al., 2004). In pregnant women, prolonged seizures may kill the fetus if placental profusion decreases significantly. With the antiparasitic medications currently available, albendazole is preferred over praziquantel because of albendazole s lower cost THE PORK TAPEWORM IS A SIGNIFICANT CAUSE OF ILLNESS, DISABILITY AND DEATH, AFFECTING 50 MILLION PEOPLE WORLDWIDE Treatment Antiseizure Management With any person who is infected with neurocysticercosis, the treatment plan is complicated. Different management option choices depend on clinical presentation, extent of infection, site of larval cysts and stages of larval development. With patients like the one in our case example who are experiencing symptoms such as seizure activity, initial management must focus on seizure control using antiepileptic drugs and treatment of increased intracranial pressure, if present (Grondin et al., 2006). Antiepileptic medications, such as phenytoin, carbamazepine, levetiracetam or topiramate should be administered (Singh et al., 2006). In pregnancy, clinicians must use the safest effective drug or drug combination that will not harm the fetus. The optimal duration of antiepileptic drug therapy is uncertain, but most experts favor 6 to 12 months of antiseizure medication following radiographic resolution of active parasitic infection. Following this time period, a medication-free trial period may be warranted to see if seizures reoccur. Long-term antiseizure medications April May 2012 and fewer side effects (Rajshekhar, 2010). If health care providers determine that antiparasitic therapy is indicated, corticosteroids are given at the same time to reduce inflammation associated with the dying worm larva (Nash et al., 2006). Nevertheless, albendazole is contradicted in pregnancy, and treatment with this drug is almost always delayed until after birth (Asnis et al., 2009; Takayanagui, 2004). Surgery Surgery is infrequently required in the management of patients with neurocysticercosis. The most common indication for surgery is to excise intraventricular cysts. Endoscopic surgery is minimally invasive and is the procedure of choice for excision of intraventricular cysticercal cysts. The placement of a shunt to relieve hydrocephalus is the next most common indication for surgery (Rajshekhar, 2010). Implications of Maternal Cysticercosis on Fetal Health There is no evidence that cysticercosis can be transmitted to Nursing for Women s Health 121

BOX 1 Case Study When Rosa (not her real name) presented to obstetric triage complaining of a headache, she seemed like a typical pregnant woman at 35 weeks gestation.

5 BOX 1 Case Study When Rosa (not her real name) presented to obstetric triage complaining of a headache, she seemed like a typical pregnant woman at 35 weeks gestation. The previous week her first prenatal visit had been uneventful, with ultrasound indicating normal fetal development. Rosa told the triage nurse that her headache began the previous day and was now worse, with severe radiating pain focused at the top of her skull. She rated the pain as 10 on a scale of 1 to 10. Rosa denied headache history, fever or neurologic problems. Her blood pressure was 119/71 and she was afebrile. Dipstick urine indicated large ketones and no protein. Intravenous (IV) therapy was initiated, and a complete blood count (CBC), aspartate aminotransferase (AST) and creatinine were obtained to rule out gestational hypertension. Neither acetaminophen nor Midrin (acetaminophen, dichloralphenazone and isometheptene) decreased Rosa s pain. Rosa s condition worsened rapidly. She developed nuchal rigidity, fever of 100.4ºF and an elevated white blood cell count. Neurology was consulted to assess for meningitis or intracranial venous thrombosis. Emergent lumbar puncture was followed by IV dexamethasone, vancomycin and ceftriaxone. Magnetic resonance venography and imaging (MRV/MRI) was negative for venous thrombosis, but positive for a cystic mass within the body of the right lateral ventricle concerning for intraventricular neurocysticercosis. Rosa had a parasitic worm larva (cysticercus) in her brain. To avoid risk to her fetus, Rosa s physicians planned to treat her infestation after birth. On the second day of hospitalization, Rosa s mental status changed, and she developed status epilepticus. She was intubated and treated with levetiracetam, lorazepam and fosphenytoin. Emergency computerized tomography (CT) scan revealed hydrocephalous. An external ventricular drain (EVD) was inserted to reduce intracranial pressure. Rosa s fetus had been continuously assessed throughout hospitalization via electronic fetal monitoring. Baseline fetal heart rate remained between 110 and 160 beats per minute with moderate variability and no decelerations, consistent with normal fetal acid-base status. Rosa s medical team planned cesarean section delivery at 37 weeks gestation, followed by immediate removal of the intraventricular cystericus. Before this plan could be initiated, Rosa experienced spontaneous rupture of membranes with clear fluid and rapid onset of labor. Epidural anesthesia was placed, and labor was augmented with oxytocin. Second-stage labor management avoided excessive maternal pushing efforts that could increase intracranial pressure. Rosa was allowed to labor down. Rosa delivered a vigorous 2,884-gram male with Apgar scores of 7 and 9. She held her son briefly before she was transferred to the intensive care unit. Post-delivery CT and MRI did not indicate intercranial hemorrhage or fluid collection. The cysticercus in Rosa s third ventricle was endoscopically removed a week later. Because there was no evidence of other cysticerci, Rosa did not require antiparasitic medication. Rosa was discharged home in stable condition on levetiracetam, dexamethasone and hydrocodone. She never had another seizure, and her neurosurgeon discontinued seizure medications after 2 months. Rosa s final CT scan indicated the presence of bifrontal punctuate calcifications, likely the sequela of remote neurocysticercosis. Both mother and baby were well at the time this article was written. 122 Nursing for Women s Health Volume 16 Issue 2

6 opmental issues and obtain early intervention should any concern arise. Conclusion For the patient in our case example, a successful outcome was attained following the experience of a frightening, life-threatening acute illness. Cysticercosis is a disease associated with poverty, poor hygiene, lack of sanitation and a poorly regulated pork industry in some less developed nations. Prevention is the single most important factor in reducing the frequency of cysticercosis (Serpa et al., 2006). The transmission cycle of cysticercosis could be interrupted by improving sanitary conditions and eliminating the presence of the parasite in human hosts. The treatment of all T. solium carriers could prevent cysticercosis by reducing the excretion of eggs and subsequent infection in other humans or autoinoculation within the original host. Currently, the major strategies for control of cysticercosis are public health education campaigns emphasizing the need for hygienic practices. Simple hygiene, such as washing hands before eating and after using the bathroom, drinking boiled water, properly storing CURRENTLY, THE MAJOR STRATEGIES FOR CONTROL OF CYSTICERCOSIS ARE PUBLIC HEALTH EDUCATION CAMPAIGNS EMPHASIZING THE NEED FOR HYGIENIC PRACTICES the fetus via the placenta. The greater danger is due the complications of neurocysticercosis, particularly when the mother has seizures, causing fetal hypoxia. A short episode of hypoxia from a single maternal seizure usually causes no harm, but prolonged fetal hypoxia results in neonatal morbidity and death. Up to 50 percent of all fetuses do not survive an episode of maternal status epilepticus (Bienstock & Fox, 2004). In the long term, fetal hypoxia may cause mental retardation, seizure disorders and cerebral palsy (Delivoria-Papadopoulous & Mishra, 2000). In the case example, ongoing electronic fetal monitoring never detected any evidence of fetal distress, and fortunately, the baby was born without any apparent neurologic deficits. The baby s pediatric provider has access to a complete prenatal history through the electronic health record. This will alert the provider to carefully assess the infant for neurologic and devel- April May 2012 and cooking pork and improving the sanitary infrastructure would markedly decrease the incidence of this parasitic infection (Serpa et al., 2006). Efforts should be made to identify and treat asymptomatic tapeworm carriers, especially among the household contacts of neurocysticercosis patients. These tapeworm carriers seem to constitute a major source of infection (Serpa et al.). Eradication of neurologic disease produced by the pork tapeworm is possible, and only socioeconomic barriers prevent achievement of this goal in the near future, even in underdeveloped regions of the world. Although the patient in our case example had a happy conclusion, the costs physical, emotional and financial were significant. There is no reason for Rosa, her newborn, or any other human to experience life-threatening disease induced by this parasitic worm. NWH Nursing for Women s Health 123

References Abba, K., Ramaratnam, S., & Ranganathan, L. N. (2010). Antihelminthics for people with neurocysticercosis. Cochrane Database Systematic Reviews (3).doi:10.1002/14651858 CD000215.

7 References Abba, K., Ramaratnam, S., & Ranganathan, L. N. (2010). Antihelminthics for people with neurocysticercosis. Cochrane Database Systematic Reviews (3).doi: / CD pub4 Asnis, D., Kazakov, J., Toronjadze, T., Bern, C., Garcia, H. H., McAuliffe, I., John, H. H. (2009). Neurocysticercosis in the infant of a pregnant mother with a tapeworm. American Journal of Tropical Medicine and Hygiene, 81(3), Bienstock, J. L., & Fox, H. E. (2004). Comorbid diseases in pregnancy. In J. E. Tintinall (Ed.), Emergency medicine: A comprehensive study guide (6th ed., Chapter 105). New York: McGraw-Hill Companies. Buzzle.com. (2011). Human tapeworm Intestinal parasite infection. Costa Mesa, CA: Author. Retrieved from buzzle.com/articles/human-tapeworm-intestinal-parasiteinfection.html Carpio, A. (2002). Neurocysticercosis: An update. Lancet Infectious Disease, 2(12), doi: /s (02) Castillo, M. (2004). Imaging of neurocysticercosis. Seminars in Roentgenology, 39(4), doi: /j.ro Centers for Disease Control and Prevention. (2010). Parasites Cysticercosis. Atlanta, GA: Author. Retrieved from cdc.gov/parasites/cysticercosis/ Delivoria-Papadopoulous, M., & Mishra, O. M. (2000). Mechanisms of perinatal cerebral injury in fetus and newborn. Annals of the New York Academy of Sciences, 900, doi: /j tb06226.x/pdf Del la Garza, Y., Graviss, E. A., Daver, N. G., Gambarin, K. J., Shandera,W. X., Schantz, P. M., & White, A. C. (2005). Epidemiology of neurocysticercosis in Houston, Texas. The American Journal of Tropical Medicine and Hygiene, 73(4), Garcia, H. H., & Del Brutto, O. H. (2005). Neurocysticercosis: Updated concepts about an old disease. Lancet Neurology, 4 (10), doi: /s (05) Garcia, H. H., Del Brutto, O. H., Nash, T. E., White, A. C., Tsang, V. C., & Gilman, R. H. (2005). New concepts in the diagnosis and management of neurocysticercosis. The American Journal of Tropical Medicine and Hygiene, 72(1), 3 9. Garcia, H. H., Gonzalez, A. E., Evans, C. A., & Gilman, R. H. (2003). Taenia solium cysticercosis. The Lancet, 362 (9383), doi: /s (03) Garcia, H. H., Pretell, E. J., Gilman, R. H., Martinez, M., Moulton, L. H., Del Brutto, O. H., Gonzalez, A. E. (2004). A trial of antiparasitic treatment to reduce the rate of seizures due to cerebral cysticercosis. New England Journal of Medicine, 3(350), doi: /nejmoa Grondin, L., D Angelo, R., Thomas, J., & Pan, P. H. (2006). Neurocysticercosis masquerading as eclampsia. Anesthesiology, 105(5), doi: / Hawk, M. W., Shahlaie, K., Kim, K. D., & Theis, J. H. (2005). Neurocysticercosis: A review. Surgical Neurology, 63(2), doi: /j.surneu Nash, T. E., Del Brutto, O., Butman, J. A., Corona, T., Delgodo-Escueta, A., Duron, R. M., Garcia, H. H. (2004). Calcific neurocysticercosis and epileptogenesis. Neurology, 62(11), doi: /01.wnl Get the Facts General Information on Cysticercosis Resources for Health Professionals health_professionals/index.html Illustration: Life Cycle of the Pork Tapeworm Cysticercosis_LifeCycle.gif Nash, T. E., Singh, G., White, A. C., Rajshekhar, V., Loeb, J. A., Proano, J. V., Garcia, H. H. (2006). Treatment of neurocysticercosis: Current status and future research needs. Neurology, 67(7), doi: /01.wnl a Rajshekhar, V. (2010). Surgical management of neurocysticercosis. International Journal of Surgery, 8(2), doi: /j. ijsu Serpa, J. A., Yancey, L. S., & White, A. C. (2006). Advances in the diagnosis and management of neurocysticercosis. Expert Review of Anti-infective Therapy, 4(6), doi: / Singh, P., & Singh, S. (2004). Neurocysticercosis in children. Journal of Child Neurology, 19(7), doi: / Singh, G., Singh, P., Singh, I., Rani, A., Kaushal, S., & Avasthi, G. (2006). Epidemiologic classification of seizures associated with neurocysticercosis: Observations from a sample of seizure disorders in neurologic care in India. Acta Neurologica Scandinavica, 113(4), doi: /j x Takayanagui, O. M. (2004). Therapy for neurocysticercosis. Expert Review Neurotherapy, 4(1), doi: / Van Voorthis, W. C., & Weller, P. F. (2010). Helminthic infections. In E. G. Nabel & D. D. Federman (Eds.), Infectious diseases: The clinician s guide to diagnosis, treatment, and prevention. New York: Decker Intellectual Properties. 124 Nursing for Women s Health Volume 16 Issue 2

PARASITOLOGY CASE HISTORY 5 (HISTOLOGY) (Lynne S. Garcia)

PARASITOLOGY CASE HISTORY 5 (HISTOLOGY) (Lynne S. Garcia) A 47 year-old male presented with symptoms that were consistent with possible late adult-onset epilepsy (frequent headaches and occasional seizures).