Pediatric Neurocysticercosis 

  • Author: Vinod K Dhawan, MD, FACP, FRCP(C); Chief Editor: Russell W Steele, MD   more...
 
Updated: Jul 22, 2011
 

Background

Neurocysticercosis has been increasingly recognized as a major cause of neurologic disease worldwide and an important problem among immigrant populations in the United States. This is largely due to the widespread availability of sensitive neuroimaging techniques. Neurocysticercosis is the most common parasitic infection of the CNS. Tissue-invading larval forms of the pork tapeworm Taenia solium cause the disease.

Historically, neurocysticercosis was endemic to only Latin America, Asia, and Africa, although it has become increasingly frequent in the United States since the 1980s. Because of this epidemiologic change, all general pediatricians should become familiar with this disease process. The disease has been the subject of several recent reviews.[1, 2, 3, 4, 5, 6]

See the images below.

Case 1: Coronal image MRI of a 6-year-old boy fromCase 1: Coronal image MRI of a 6-year-old boy from Peru with single right frontal cyst. Case 1: Axial image MRI of a 6-year-old boy from PCase 1: Axial image MRI of a 6-year-old boy from Peru with single right frontal cyst.
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Pathophysiology

The larvae of T solium (Cysticercus cellulosae) cause neurocysticercosis. This pork tapeworm can vary in size but is notable for a scolex (head) with approximately 25 hooklets, 4 suckers, and a body with 700-1000 proglottids. The ova of the tapeworm are spread via the fecal-oral route and are approximately 40 microns in diameter with a radially striated shell. The intermediate host is the pig, which harbors the larvae after eating ova, and the definitive host is the human being.

If pig products infected with larvae are ingested, a tapeworm infection in the intestines ensues; however, if ova are ingested, neurocysticercosis may occur in this normally intermediate host. The ingested ova develop into larvae (cysticerci) and lodge in soft tissues, especially skin, muscle, and brain. Cysticerci are fluid-filled oval cysts, approximately 1-2 cm in diameter, with an internal scolex.

The eggs are found in human feces because humans are the only definitive hosts. Greatest risk for infection occurs in regions where plants in gardens or farms are fertilized with human feces and humans are exposed to contaminated soil.

In the CNS, T solium is deposited in the cerebral parenchyma, meninges, spinal cord, and eyes. Unless large numbers of cysts are present, the body's immune system does not act to destroy the organism, and cysts can live for many years undetected. A live cyst can go undetected for as long as 5 years before dying or causing symptoms in the host.

Neurologic symptoms arise when the encysted worm dies and the human mounts an associated inflammatory response. If the cyst lodges in the ventricular system (especially the fourth ventricle), hydrocephalus can occur.

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Epidemiology

Frequency

United States

Neurocysticercosis is more common among immigrants from endemic areas or children in contact with these immigrants.[7] [8] Several studies report approximately 1000 cases annually in adults and children. One study reported neurocysticercosis as the cause of 2% of the neurologic and neurosurgical admissions in southern California.[9] With increasing immigration from Mexico and other endemic areas, incidence in the southern United States has been increasing. In a recent meta-analysis of the US medical literature, 1494 cases have been reported from 1980-2004.[10]

International

Neurocysticercosis is highly endemic in Latin America, Mexico, Eastern Europe, Asia, Africa, and Spain. The estimated serologic prevalence in Mexican adults is 3.6%, with positive confirmation by autopsy in 1.9%. Approximately 50 million people worldwide are infected. World Health Organization estimates that more than 50,000 individuals die each year from neurocysticercosis.[11]

Mortality/Morbidity

Neurocysticercosis is typically benign, and most lesions spontaneously resolve within 2-3 months; however, mortality is highly dependent on whether the disease process is simple or complicated.

Simple neurocysticercosis occurs in children with only a single exposure to cysts. These children tend to have solitary cysts and fewer complications. Often, children can be treated symptomatically and have a favorable prognosis. This type of neurocysticercosis tends to be observed in the United States and other nonendemic areas.

Complicated neurocysticercosis occurs in children in endemic areas who are repeatedly exposed to ova. Because of complications from increased intracranial pressure and difficulty controlling seizures, these children may have a less favorable prognosis. Complications can arise from uncontrolled seizures, hydrocephalus, papilledema, and occasionally, headaches and emesis, although all of these complications are rare.

Race

Neurocysticercosis tends to be diagnosed more frequently in Hispanics because of the prevalence of the organism in the countries of origin, rather than an innate property of the host child.

Sex

No sexual predilection has been noted.

Age

Reports of cysticercosis are unlikely in children younger than 2 years because of the prolonged incubation period of T solium. Most often, the disease is recognized in children older than 7 years because of this incubation period.

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Contributor Information and Disclosures
Author

Vinod K Dhawan, MD, FACP, FRCP(C)  Professor, Department of Clinical Medicine, University of California, Los Angeles, David Geffen School of Medicine; Chief, Division of Infectious Diseases, Rancho Los Amigos National Rehabilitation Center, Downey, California.

Vinod K Dhawan, MD, FACP, FRCP(C) is a member of the following medical societies: American College of Physicians, American Society for Microbiology, American Society of Tropical Medicine and Hygiene, Infectious Diseases Society of America, and Royal College of Physicians and Surgeons of Canada

Disclosure: Pfizer Inc Honoraria Speaking and teaching

Coauthor(s)

Eric HW Kossoff, MD  Assistant Professor, Departments of Pediatrics and Neurology, Associate Director of Pediatric Neurology Residency Program, Johns Hopkins School of Medicine

Eric HW Kossoff, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Ashir Kumar, MD, MBBS, FAAP  Professor Emeritus, Department of Pediatrics and Human Development, Michigan State University College of Human Medicine

Ashir Kumar, MD, MBBS, FAAP is a member of the following medical societies: American Association of Physicians of Indian Origin and Infectious Diseases Society of America

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Leslie L Barton, MD  Professor Emerita of Pediatrics, University of Arizona College of Medicine

Leslie L Barton, MD is a member of the following medical societies: American Academy of Pediatrics, Association of Pediatric Program Directors, Infectious Diseases Society of America, and Pediatric Infectious Diseases Society

Disclosure: Nothing to disclose.

Robert W Tolan Jr, MD  Chief, Division of Allergy, Immunology and Infectious Diseases, The Children's Hospital at Saint Peter's University Hospital; Clinical Associate Professor of Pediatrics, Drexel University College of Medicine

Robert W Tolan Jr, MD is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, American Society for Microbiology, American Society of Tropical Medicine and Hygiene, Infectious Diseases Society of America, Pediatric Infectious Diseases Society, Phi Beta Kappa, and Physicians for Social Responsibility

Disclosure: GlaxoSmithKline Honoraria Speaking and teaching; MedImmune Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Sanofi Pasteur Honoraria Speaking and teaching; Baxter Healthcare Honoraria Speaking and teaching; Novartis Honoraria Speaking and teaching

Chief Editor

Russell W Steele, MD  Head, Division of Pediatric Infectious Diseases, Ochsner Children's Health Center; Clinical Professor, Department of Pediatrics, Tulane University School of Medicine

Russell W Steele, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American Pediatric Society, American Society for Microbiology, Infectious Diseases Society of America, Louisiana State Medical Society, Pediatric Infectious Diseases Society, Society for Pediatric Research, and Southern Medical Association

Disclosure: Nothing to disclose.

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Case 1: Coronal image MRI of a 6-year-old boy from Peru with single right frontal cyst.
Case 1: Axial image MRI of a 6-year-old boy from Peru with single right frontal cyst.
Case 2: MRI of a 40-year-old patient with a single parietal calcified cyst.
Case 2: CT scan of a 40-year-old patient with a single parietal calcified cyst.
Case 3: MRI of a 47-year-old man with 2 right parietal cysts, one with edema.
Case 3: MRI of a 47-year-old man with 2 right parietal cysts, one with edema, after the larger cyst had involuted.
Case 4: CT scan of 28-year-old woman with occipital headaches and diplopia; imaging reveals a superior cerebellar cyst, mild ventricular dilatation, and old calcifications in the right insular region. Image courtesy of Gholam Motamedi, MD.
Case 4: MRI of 28-year-old woman with occipital headaches and diplopia; MRI discerns prepontine and suprasellar lesions, as well as the superior cerebellar cyst. Image courtesy of Gholam Motamedi, MD.
MRI of multiple cysts. Image courtesy of the Centers for Disease Control and Prevention.
MRI of an 87-year-old patient from Europe with bitemporal lesions found incidentally. Image courtesy of Jon Poling, MD.
Two parietal lesions observed on autopsy specimen.
MRI of a 40-year-old woman with severe epilepsy and a left temporal single cyst.
MRI of a 21-year-old woman with left temporal lobe epilepsy and a single cyst.
 
 
 
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