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Seizures in a Non-communicative Child
DEPARTMENT
Case Study—Acute & Specialty Care
Seizures in a Non-communicative Child Anna Tielsch-Goddard, MSN, CPNP-PC
KEY WORDS Seizures, autism, neurocysticercosis, pork tape worm, parasite
CHIEF COMPLAINT The child’s mother reports that her child had a seizure and has been irritable. HISTORY OF PRESENT ILLNESS A 5-year-old non-verbal child with autism whose case is followed by the neurology department comes to the neurology clinic because of new-onset seizures. The patient was in a rural area of Mexico visiting his grandparents during the summer for several months. Section Editors Andrea Kline Tilford, MS, RN, CPNP-PC/AC, CCRN, FCCM Corresponding Editor Riley Hospital for Children Indianapolis, Indiana Terea Giannetta, MSN, RN, CPNP California State University, Fresno Fresno, California Karin Reuter-Rice, PhD, RN, CPNP Rady Children’s Hospital San Diego, California Anna Tielsch-Goddard, Pediatric Nurse Practitioner, Pediatric Neurology, Children’s Medical Center Dallas, Dallas, TX. Conflicts of interest: None to report. Correspondence: Anna Tielsch-Goddard, MSN, CPNP-PC, Advance Practice Services, 1935 Medical District Dr, Dallas, TX 75235; e-mail: [email protected]. J Pediatr Health Care. (2010) 24, 270-274. 0891-5245/$36.00 Copyright Q 2010 by the National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved. doi:10.1016/j.pedhc.2010.03.006
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When he returned to the United States 2 weeks ago, his mother reported a first-time seizure that she described as total body jerks with teeth clenched and drooling from the corner of his mouth. This episode lasted for approximately 2 to 3 minutes. She does not know if these seizures were happening while the child was in Mexico. The mother reports that the patient has had increased irritability and has been holding his head when rocking in a chair at night for the past few weeks. The mother reports that this behavior is new. She also has noticed that he holds his head as he walks around the house and has been sensitive to bright lights. When the mother noticed a second seizure and postictal vomiting 1 week ago, she brought the child in for an examination. HISTORY This child’s case has been followed by the neurology department since he first presented with symptoms of autism at 2 years of age. He demonstrated early signs of pervasive developmental disorder at age 2 years and continued to have marked impairment in verbal communication, although he has some language skills and can communicate with his parents through rudimentary sign language. He avoids eye contact and frequently repeats words he hears from his parents. Consistent with the autistic spectrum, the child exhibits repetitive body movements and behavior patterns and has sensory integration difficulties for which he receives occupational therapy. He has not shown any signs of aggression toward himself or others. He was born full term with an unremarkable birth and neonatal history. The patient showed delays with gross and fine motor skills as well as language and social and behavioral skills at 18 months. His mother denies a history of head or spinal cord trauma, and he has never had meningitis, encephalitis, or varicella. He is not toilet trained, and his mother reports no diarrhea or constipation. He does not take any medication and has no known allergies. His immunizations are up to date and he has never had surgery or been under anesthesia. Journal of Pediatric Health Care
Early childhood intervention therapies have been provided at school. He lives at home with his mom, dad, and an older male sibling in Texas. His diet consists of macaroni and cheese, hot dogs, hamburgers, and sausage. He requires his food to be cut into small pieces or ground. He is minimally active outside and enjoys watching cooking shows on television with his grandparents. He has visited his grandparents, who live in a rural area in Mexico, for several weeks every summer since the age of 2 years. The family history is negative for any neurologic symptoms or disease including epilepsy, cerebral palsy, neurodegenerative disorders, neurofibromatosis, tuberous sclerosis, or headaches. PHYSICAL EXAMINATION The patient’s temperature was 36.9°C (98.4°F), his respiratory rate was 26 breaths per minute, his pulse was 100 beats per minute, and his blood pressure was 128/86 mm Hg. On examination, he appeared well nourished and in no acute distress. His neck was supple with no lymphadenopathy. He became irritated with anyone in close proximity of his head, mouth, and ears and had poor eye contact, which is consistent with children who have autism. An eye examination was done under papoose restraint. His pupils were equal and reactive to light, with his extraocular muscles intact. He had sharp disc margins. His tongue was midline. His lungs were clear upon auscultation, and an examination of the cardiovascular system revealed no murmurs, rubs, or gallops and a regular heart rate and rhythm. Decreased tone was noted in his upper extremities, and he was sensitive to touch with monofilament fiber on examination. Tip-toe walking was noted, which is frequently seen in children with autism and is part of his normal behavior. He had negative Gower’s and Romberg’s signs. Gower’s sign indicates proximal
muscle weakness and describes patients who use their hands to ‘‘walk’’ up the body from a squatting position. Gower’s sign is seen in persons with muscular dystrophy, paraplegia, and conditions with proximal muscle weakness or atrophy. Romberg’s test indicates sensory ataxia and is performed by having patients stand with their feet together and their eyes closed while the examiner observes for swaying or unsteady gait with the eyes closed. His gait was awkward and had not changed since the previous examination. Deep tendon reflexes were 2+ in all extremities. Cranial nerves I-XII were grossly intact. DIAGNOSTIC TESTING A complete blood cell count with differential, serum electrolytes (including glucose, calcium, and magnesium), blood lactate and pyruvate, blood ammonia, urinalysis, and stool hemoccult with culture were sent to identify a possible underlying etiology. Laboratory tests showed mild leukocytosis of 15.5 109/L (normal, 4.4-10.8 109/L). Other laboratory findings were within normal limits. Because of this patient’s recent travel to Mexico, a stool sample for hemoccult and ova and parasites was ordered. The sample was negative for mucosa damage, heme, and Giardia antigen. Because of the new onset of seizures, an electroencephalogram to evaluate epileptic activity and a magnetic resonance imaging (MRI) scan for brain imaging were obtained. The electroencephalogram showed high amplitude waves and an erratic pattern of electrical activity lasting for several minutes in the frontal and parietal regions of the brain, which indicates abnormal activity and is diagnostic for generalized grand-mal seizure activity. The MRI scan showed viable cystic lesions in the left frontal and parietal lobes with a small hyper-dense nodule, representing the scolex of a tapeworm.
CASE STUDY QUESTIONS 1. 2. 3. 4. 5. 6.
What differential diagnoses are you considering for this child? How is Taenia solium diagnosed? What key historical finding leads you to consider neurocysticercosis? What is the appropriate treatment for neurocysticercosis? What is the recommended follow-up for neurocysticercosis? What patient education would you provide for the family?
CASE STUDY ANSWERS 1. What differential diagnoses are you considering for this child? Differential diagnoses for this patient includes autism with new onset of epilepsy, parasitic or infectious etiology, or a possible cerebrovascular event because of the focal neurologic signs reported by the mother. Other possible causes include tuberous sclerosis,
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a brain abscess or neoplasm, tuberculosis, cytomegalovirus infection, mycosis, arteriovenous malformation, or even toxoplasmosis. Laboratory and radiologic findings are key to the diagnosis. Autistic patients often have a dual diagnosis of epilepsy (Canitano, 2006). A recent study showed that one of three patients with autism also had some form
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of epilepsy and that autism is significantly associated with seizure activity (Danielsson, Gillberg, Billstedt, Gillberg, & Olsson, 2005). An MRI or computed tomography (CT) scan is frequently ordered with new-onset seizures. In this case an MRI scan revealed small hyperdense areas consistent with the scolex of a tapeworm, or Taenia solium (pig tapeworm) infestation. An MRI scan is more accurate than a CT scan in detecting the scolex, which can be used to confidently diagnosis neurocysticercosis (Khosla, 2008). An MRI scan can detect other tapeworms such as the beef tapeworm, Taneia saginate, which is often found in North America, and the fish tapeworm, Diphyllobothrium latum, which is common in Asia. The pork tapeworm, T. solium, is most commonly found in Latin America. The recent visit to Mexico and the patient’s diet of pork and beef products supports the suspicion of a parasitic infection such as Taenia saginata, as well as Diphyllobothrium latum. Stool testing was negative for parasites and is not diagnostic for neurocysticercosis (Del Brutto et al., 2001). Demonstrating T. solium eggs in the stool only allows diagnosis of taeniasis and not active cysticerci in the brain (John & Petri, 2006). Enzyme-linked immunoblot assay (ELISA) of the serum was positive for T. solium, demonstrating detection of anti-cysticercal antibodies. ELISA has the greatest sensitivity and specificity for diagnosing neurocysticercosis (Kraft, 2007). 2. How is Taenia solium diagnosed? MRI visualization of the scolex, or sucking parts of the larva, is diagnostic for neurocysticercosis (Kraft, 2007). CT or MRI neuroimaging are the most useful methods for diagnosing neurocysticercosis (Del Brutto et al., 2001). CT scans show calcified and uncalcified cysts. Unlike CT scans, MRI scans can detect intraventricular cysts. This child’s MRI scan demonstrated intraventricular multiple calcified cystic lesions. Single non-enhancing cystic lesions occur with hydatid disease, arachnoid cysts, cystic astrocytoma, and colloid cysts. Multiple non-enhancing cystic lesions occur with multiple metastasis, hydatid disease, tuberculosis, mycosis, toxoplasmosis, early gliomas, and arteriovenous malformations. Calcifications of these lesions may occur with tuberous sclerosis, tuberculosis, cytomegalovirus infection, and toxoplasmosis (Kraft, 2007). A brain lesion biopsy of T. solium as well as a direct visualization of sub-retinal parasites by funduscopic examination also may be used to diagnose neurocysticercosis. Absolute criteria for diagnosis involves a brain or spinal cord biopsy of the lesion, but other clinical and laboratory data should be used first to avoid invasive diagnostic tests (Burneo, Plener, & Garcia, 2009; Del Brutto et al., 2001; Kraft, 2007). With patients who are severely developmentally delayed or have cognitive deficits, such as autistic patients, eye examinations and radiologic imaging often must be done under sedation. 272
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The ELISA has been reported to have 98% sensitivity and 100% specificity. The serologic diagnosis of T. solium may be done with the enzyme-linked immune-transfer blot for detection of any circulating antibodies. A negative serologic result, however, does not exclude cysticercosis (Khosla, 2008). The sensitivity of serologic testing is decreased in patients with calcified lesions (Khosla, 2008). 3. What key historical finding leads you to consider neurocysticercosis? Contact with T. solium must occur for infection. Travel to an endemic area or any endemic exposure to a person from or living in an area with cysticercosis raises suspicion for this diagnosis. T. solium is transmitted mainly in areas where pigs have access to untreated human sewage or feces (Kraft, 2007). The parasite has been eradicated in the more developed countries with improved sanitation and control of Neurocysticercosis domestic pig farming. is a parasitic T. solium is endemic in China, Southeast infection caused by Asia, India, sub-Sahara the pork tapeworm Africa, and Latin AmerT. solium. ica (Kraft, 2007). In Mexico alone, 3.1% to 3.9% of people carry the parasite (World Health Organization [WHO], 2009). In the United States, the disease is usually found in immigrants from Mexico and Central and South America (WHO, 2009). Furthermore, the presence of multifocal calcifications on an MRI scan is highly suggestive of cysticercosis, especially in patients from endemic areas (Khosla, 2008). Neurocysticercosis is a parasitic infection caused by the pork tapeworm T. solium. Infection occurs in humans when ingesting eggs or larvae from undercooked pork containing the larvae or from food or water contaminated with fecal matter (John & Petri, 2006). After ingestion, the ova release oncospheres that eventually reach the bloodstream and are able to develop into cysticerci in any organ but are most common in the brain and eyes (John & Petri, 2006). Clinical symptomatology depends on the number and location of the cysts and the immune system’s response to the parasite. Most patients present with clinical signs and symptoms, such as seizures or other neurologic signs such as headaches and increased intracranial pressure (Khosla, 2008). Patients will present any time from 2 to 10 years from when cysts become mature (Davis, 2005). Because of the long incubation period, children do not account for a large percentage of clinical presentations of neurocysticercosis, and seropositive results tend to increase with age (Khosla, 2008). Neurocysticercosis is a leading cause of seizures and epilepsy in developing countries (DeGiorgio, Medina, Reynalds, & Pietsch-Escueta, 2004). Chief symptoms are often seizures (70% to 90%) and headaches, but patients also may present with nausea, vomiting, Journal of Pediatric Health Care
behavioral disturbances, or memory impairment (DeGiorgio et al., 2004). Cysts and disease in the brain develop in almost 60% of patients with cysticerci (John & Petri, 2006). 4. What is the appropriate treatment for neurocysticercosis? Antihelmintic drugs should be considered only after a thorough ophthalmic examination to rule out any intraocular cysts before treatment with a cysticercoidal drug. Cysticidal drugs cause cyst degeneration and can result in visual impairment if cysts are present in the eye (Garcia et al., 2002). This patient did not have any ocular involvement as evidenced by an ophthalmologic examination under sedation, so cysticidal medication was indicated. Surgery is indicated for ophthalmic cysticercosis, and antihelminth drugs should be avoided until ocular lesions are removed (Garcia et al., 2002). Albendazole and praziquantel are the principal cysticidal drugs (DeGiorgio et al., 2004; Sotelo & Del Brutto, 2002). Albendazole is preferred over praziquantel because it is less costly and has a better clinical response for seizure control in persons with neurocysticercosis (Burneo et al., 2009; Matthaiou et al., 2008). Albendazole should be given at 15 mg/ kg/day (up to 400 mg twice daily) for 4 weeks to reduce seizure recurrence and existing lesions (Bureno, 2009; Matthaiou, Panos, Adamidi, & Falagas, 2008). Adverse effects include nausea, headache, cerebral edema, gastrointestinal upset, seizures, and increased intracranial pressure (Carpio, 2002). Steroids commonly are used with cysticercosis in the brain because cysticidal drugs damage the parasite and release antigens that often trigger inflammatory response (Burneo et al., 2009). Corticosteroids may decrease the seizure frequency (Yancey, Diaz-Marchan, & White, 2005). An openlabel randomized trial found a reduced seizure rate when prednisone therapy, 1 mg/kg/day for 10 days, was followed by a 4-day tapering of the drug (Mall, Agarwall, Garg, Kar, & Shukla, 2003). 5. What is the recommended follow-up for neurocysticercosis? Neuroimaging should be repeated in 3 months, after completion of therapy with albendazole. If there is only a partial resolution of the cystic lesion, a repeat MRI scan of the head should be repeated in 3 months (Garcia et al., 2002). A second course of albendazole may be needed if the lesions are persistent after 6 months (Garcia et al., 2002). Some patients will require long-term antiepileptic medications such as phenytoin (Dilantin) and carbamazepine (Tegretol) for seizure control (Kraft, 2007). Referral to a pediatric neurologist or epilepsy clinic is indicated. Seizure recurrence depends on the number of cyst calcifications on an MRI scan and the cyst burden of the brain. Prognosis varies from patient to patient and the specific clinical findings in each case. The patient should www.jpedhc.org
follow up with a neurologist for this condition every 6 months, and the patient’s clinical course and outcome should be communicated to the patient’s primary care provider. 6. What patient education would you provide for the family? Emphasizing the importance of cooking pork thoroughly or keeping unused portions frozen along with close inspection of the meat should be taught to people who frequently travel or live in endemic areas. Consumption of raw or undercooked meat Neurocysticercosis should be avoided. can present in The WHO stresses the importance of treating many forms or vaccinating pigs depending on cyst against cysticercosis location and (WHO, 2007). Cysticercosis can be prevented disease by routine inspection progression. of meat and destruction of infected meat by the local government. However, in areas where food is scarce, cyst-infected meat is still eaten, which is one of the reasons cysticercosis has not been eliminated in endemic area (WHO, 2007). Family care and support is important for patients with neurocysticercosis infection. Case management may be necessary to coordinate infectious disease follow-up, neurology visits, or possible referral to an epileptologist. The patient’s medication regimen and rationale should be reviewed with the parents, and the importance of compliance with follow-up diagnostic imaging and the health care provider must be emphasized. The family also can be given useful references to explore, such as the Neurocysticercosis and Epilepsy Research Network and the Centers for Disease Control Working Group on Parasitic Diseases at http://whqlibdoc.who.int/hq/2007/WHO_ CDS_NTD_2007.3_eng.pdf Neurocysticercosis can present in many forms depending on cyst location and disease progression. It should be high on the list of differential diagnoses in children presenting with new-onset seizures who have traveled to endemic areas. Neurocysticercosis is a leading cause of epilepsy in the developing world (DeGiorgio et al., 2004). Treatment with albendazole improves the prognosis for recurrent tonic-clonic seizures in highly selected patients. The WHO is developing and enforcing global strategies for control (DeGiorgio, 2004). INTERPRETING A MEDICAL HISTORY WHEN YOUR PATIENT HAS DIFFICULTY COMMUNICATING Autistic children have impaired communication skills and sometimes are unable to verbalize symptoms of July/August 2010
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pain such as headaches. The patient presented with increased irritability, per the mother’s report. Providers who are taking care of children with any disorder involving impaired communication need to pay close attention to the caregiver’s report of changes in behavior or activity. This patient presented with possible headaches, as suggested by the way he repetitively held his head and had increased irritability and sensitivity to lights. Patients with any pervasive developmental disorder or who present on the autistic spectrum present the practitioner with an added challenge in compiling a medical history. SUMMARY This patient presented to a specialty clinic that routinely monitored him for autism. An electroencephalogram and MRI scan were ordered as part of a chief complaint for new-onset seizures. The scolex of a tapeworm was an incidental finding that is diagnostic for tapeworm. Pork tapeworm is the most common tapeworm parasite in Mexico. Cysticidal medication was indicated because the eye examination was within normal limits. The patient should receive close follow-up from the neurology team with ongoing communication to the primary care provider. REFERENCES Burneo, J., Plener, I., & Garcia, H. (2009). Neurocysticercosis and epilepsy research network. Canadian Medical Association, 180(6), 639-642. Canitano, R. (2006). Epilepsy in autism spectrum disorders. European Child and Adolescent Psychiatry, 16(1), 61-66. Carpio, A. (2002). Neurocysticercosis: An update. The Lancet Infectious Diseases, 2, 751-762. Danielsson, S., Gillberg, C., Billstedt, E., Gillberg, C., & Olsson, I. (2005, June). Epilepsy in young adults with autism: A prospec-
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tive population-based follow-up study of 120 individuals diagnosed in childhood. Epilepsia, 46(6), 918-923. Davis, L. E. (2005). Neurocysticercosis. In C. Power & R. T. Johnson (Eds.), Emerging neurological infections (pp. 261-287). Boca Raton, FL: Taylor & Francis Group. DeGiorgio, C. M., Medina, M. T., Reynalds, Z., & Pietsch-Escueta, S. P. (2004, May). Neurocysticercosis. Epilepsy Currents, 4(3), 107-111. Del Brutto, O. H., Rajshekhar, V., White, A. C., Tsang, W., Nash, T. E., Takayanagui, O. M., & Garcia, H. H. (2001). Proposed diagnostic criteria for neurocysticercosis. Neurology, 57, 177-183. Garcia, H. H., Evans, C. W., Nash, T., Takayanagui, O. M., Clinton White, A., Botero, D., & Del Brutto, O. H. (2002, Oct). Current consensus guidelines for treatment of neurocysticercosis. Clinical Microbiology Reviews, 15(4), 747-756. Khosla, A. (2008). Cysticercosis CNS. eMedicine Radiology. Retrieved from http://emedicine.medscape.com/article/339654. Kraft, R. (2007, July 1). Cysticercosis: An emerging parasitic disease. American Family Physician, 76(1), 91-96. Mall, R. K., Agarwal, A., Garg, R. K., Kar, A. M., & Shukla, R. (2003). Short course of prednisolone in Indian patients with solitary cysticercus granuloma and new-onset seizures. Epilepsia, 44(11), 1397-1401. John, D. T., & Petri, W. A. (2006). Markell and Voge’s medical parasitology (9th ed.). Philadelphia, PA: Saunders. Sotelo, J., & DelBrutto, O. H. (2002). Review of neurocysticercosis. Neurosurgical FOCUS, 12, 1. Matthaiou, D. K., Panos, G., Adamidi, E. S., & Falagas, M. E. (2008, March 12). Albendazole versus praziquantel in the treatment of neurocysticercosis: A meta-analysis of comparative trials. PLoS Neglected Tropical Disease, 2(3), e194. World Health Organization. (2007, March). Global plan to combat neglected tropical diseases 2008-2015. Retrieved from http://whqlibdoc.who.int/hq/2007/WHO_CDS_NTD_2007 .3_eng.pdf World Health Organization. (2009). Taeniasis/cysticercosis. Retrieved from http://www.who.int/zoonoses/diseases/taeniasis/ en/index.html Yancey, L. S., Diaz-Marchan, P. J., & White, A. C. (2005). Cysticercosis: Recent advances in diagnosis and management of neurocysticercosis. Current Infection Disease Report, 7(1), 39-47.
Journal of Pediatric Health Care
Case Study—Acute & Specialty Care
Seizures in a Non-communicative Child Anna Tielsch-Goddard, MSN, CPNP-PC
KEY WORDS Seizures, autism, neurocysticercosis, pork tape worm, parasite
CHIEF COMPLAINT The child’s mother reports that her child had a seizure and has been irritable. HISTORY OF PRESENT ILLNESS A 5-year-old non-verbal child with autism whose case is followed by the neurology department comes to the neurology clinic because of new-onset seizures. The patient was in a rural area of Mexico visiting his grandparents during the summer for several months. Section Editors Andrea Kline Tilford, MS, RN, CPNP-PC/AC, CCRN, FCCM Corresponding Editor Riley Hospital for Children Indianapolis, Indiana Terea Giannetta, MSN, RN, CPNP California State University, Fresno Fresno, California Karin Reuter-Rice, PhD, RN, CPNP Rady Children’s Hospital San Diego, California Anna Tielsch-Goddard, Pediatric Nurse Practitioner, Pediatric Neurology, Children’s Medical Center Dallas, Dallas, TX. Conflicts of interest: None to report. Correspondence: Anna Tielsch-Goddard, MSN, CPNP-PC, Advance Practice Services, 1935 Medical District Dr, Dallas, TX 75235; e-mail: [email protected]. J Pediatr Health Care. (2010) 24, 270-274. 0891-5245/$36.00 Copyright Q 2010 by the National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved. doi:10.1016/j.pedhc.2010.03.006
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When he returned to the United States 2 weeks ago, his mother reported a first-time seizure that she described as total body jerks with teeth clenched and drooling from the corner of his mouth. This episode lasted for approximately 2 to 3 minutes. She does not know if these seizures were happening while the child was in Mexico. The mother reports that the patient has had increased irritability and has been holding his head when rocking in a chair at night for the past few weeks. The mother reports that this behavior is new. She also has noticed that he holds his head as he walks around the house and has been sensitive to bright lights. When the mother noticed a second seizure and postictal vomiting 1 week ago, she brought the child in for an examination. HISTORY This child’s case has been followed by the neurology department since he first presented with symptoms of autism at 2 years of age. He demonstrated early signs of pervasive developmental disorder at age 2 years and continued to have marked impairment in verbal communication, although he has some language skills and can communicate with his parents through rudimentary sign language. He avoids eye contact and frequently repeats words he hears from his parents. Consistent with the autistic spectrum, the child exhibits repetitive body movements and behavior patterns and has sensory integration difficulties for which he receives occupational therapy. He has not shown any signs of aggression toward himself or others. He was born full term with an unremarkable birth and neonatal history. The patient showed delays with gross and fine motor skills as well as language and social and behavioral skills at 18 months. His mother denies a history of head or spinal cord trauma, and he has never had meningitis, encephalitis, or varicella. He is not toilet trained, and his mother reports no diarrhea or constipation. He does not take any medication and has no known allergies. His immunizations are up to date and he has never had surgery or been under anesthesia. Journal of Pediatric Health Care
Early childhood intervention therapies have been provided at school. He lives at home with his mom, dad, and an older male sibling in Texas. His diet consists of macaroni and cheese, hot dogs, hamburgers, and sausage. He requires his food to be cut into small pieces or ground. He is minimally active outside and enjoys watching cooking shows on television with his grandparents. He has visited his grandparents, who live in a rural area in Mexico, for several weeks every summer since the age of 2 years. The family history is negative for any neurologic symptoms or disease including epilepsy, cerebral palsy, neurodegenerative disorders, neurofibromatosis, tuberous sclerosis, or headaches. PHYSICAL EXAMINATION The patient’s temperature was 36.9°C (98.4°F), his respiratory rate was 26 breaths per minute, his pulse was 100 beats per minute, and his blood pressure was 128/86 mm Hg. On examination, he appeared well nourished and in no acute distress. His neck was supple with no lymphadenopathy. He became irritated with anyone in close proximity of his head, mouth, and ears and had poor eye contact, which is consistent with children who have autism. An eye examination was done under papoose restraint. His pupils were equal and reactive to light, with his extraocular muscles intact. He had sharp disc margins. His tongue was midline. His lungs were clear upon auscultation, and an examination of the cardiovascular system revealed no murmurs, rubs, or gallops and a regular heart rate and rhythm. Decreased tone was noted in his upper extremities, and he was sensitive to touch with monofilament fiber on examination. Tip-toe walking was noted, which is frequently seen in children with autism and is part of his normal behavior. He had negative Gower’s and Romberg’s signs. Gower’s sign indicates proximal
muscle weakness and describes patients who use their hands to ‘‘walk’’ up the body from a squatting position. Gower’s sign is seen in persons with muscular dystrophy, paraplegia, and conditions with proximal muscle weakness or atrophy. Romberg’s test indicates sensory ataxia and is performed by having patients stand with their feet together and their eyes closed while the examiner observes for swaying or unsteady gait with the eyes closed. His gait was awkward and had not changed since the previous examination. Deep tendon reflexes were 2+ in all extremities. Cranial nerves I-XII were grossly intact. DIAGNOSTIC TESTING A complete blood cell count with differential, serum electrolytes (including glucose, calcium, and magnesium), blood lactate and pyruvate, blood ammonia, urinalysis, and stool hemoccult with culture were sent to identify a possible underlying etiology. Laboratory tests showed mild leukocytosis of 15.5 109/L (normal, 4.4-10.8 109/L). Other laboratory findings were within normal limits. Because of this patient’s recent travel to Mexico, a stool sample for hemoccult and ova and parasites was ordered. The sample was negative for mucosa damage, heme, and Giardia antigen. Because of the new onset of seizures, an electroencephalogram to evaluate epileptic activity and a magnetic resonance imaging (MRI) scan for brain imaging were obtained. The electroencephalogram showed high amplitude waves and an erratic pattern of electrical activity lasting for several minutes in the frontal and parietal regions of the brain, which indicates abnormal activity and is diagnostic for generalized grand-mal seizure activity. The MRI scan showed viable cystic lesions in the left frontal and parietal lobes with a small hyper-dense nodule, representing the scolex of a tapeworm.
CASE STUDY QUESTIONS 1. 2. 3. 4. 5. 6.
What differential diagnoses are you considering for this child? How is Taenia solium diagnosed? What key historical finding leads you to consider neurocysticercosis? What is the appropriate treatment for neurocysticercosis? What is the recommended follow-up for neurocysticercosis? What patient education would you provide for the family?
CASE STUDY ANSWERS 1. What differential diagnoses are you considering for this child? Differential diagnoses for this patient includes autism with new onset of epilepsy, parasitic or infectious etiology, or a possible cerebrovascular event because of the focal neurologic signs reported by the mother. Other possible causes include tuberous sclerosis,
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a brain abscess or neoplasm, tuberculosis, cytomegalovirus infection, mycosis, arteriovenous malformation, or even toxoplasmosis. Laboratory and radiologic findings are key to the diagnosis. Autistic patients often have a dual diagnosis of epilepsy (Canitano, 2006). A recent study showed that one of three patients with autism also had some form
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of epilepsy and that autism is significantly associated with seizure activity (Danielsson, Gillberg, Billstedt, Gillberg, & Olsson, 2005). An MRI or computed tomography (CT) scan is frequently ordered with new-onset seizures. In this case an MRI scan revealed small hyperdense areas consistent with the scolex of a tapeworm, or Taenia solium (pig tapeworm) infestation. An MRI scan is more accurate than a CT scan in detecting the scolex, which can be used to confidently diagnosis neurocysticercosis (Khosla, 2008). An MRI scan can detect other tapeworms such as the beef tapeworm, Taneia saginate, which is often found in North America, and the fish tapeworm, Diphyllobothrium latum, which is common in Asia. The pork tapeworm, T. solium, is most commonly found in Latin America. The recent visit to Mexico and the patient’s diet of pork and beef products supports the suspicion of a parasitic infection such as Taenia saginata, as well as Diphyllobothrium latum. Stool testing was negative for parasites and is not diagnostic for neurocysticercosis (Del Brutto et al., 2001). Demonstrating T. solium eggs in the stool only allows diagnosis of taeniasis and not active cysticerci in the brain (John & Petri, 2006). Enzyme-linked immunoblot assay (ELISA) of the serum was positive for T. solium, demonstrating detection of anti-cysticercal antibodies. ELISA has the greatest sensitivity and specificity for diagnosing neurocysticercosis (Kraft, 2007). 2. How is Taenia solium diagnosed? MRI visualization of the scolex, or sucking parts of the larva, is diagnostic for neurocysticercosis (Kraft, 2007). CT or MRI neuroimaging are the most useful methods for diagnosing neurocysticercosis (Del Brutto et al., 2001). CT scans show calcified and uncalcified cysts. Unlike CT scans, MRI scans can detect intraventricular cysts. This child’s MRI scan demonstrated intraventricular multiple calcified cystic lesions. Single non-enhancing cystic lesions occur with hydatid disease, arachnoid cysts, cystic astrocytoma, and colloid cysts. Multiple non-enhancing cystic lesions occur with multiple metastasis, hydatid disease, tuberculosis, mycosis, toxoplasmosis, early gliomas, and arteriovenous malformations. Calcifications of these lesions may occur with tuberous sclerosis, tuberculosis, cytomegalovirus infection, and toxoplasmosis (Kraft, 2007). A brain lesion biopsy of T. solium as well as a direct visualization of sub-retinal parasites by funduscopic examination also may be used to diagnose neurocysticercosis. Absolute criteria for diagnosis involves a brain or spinal cord biopsy of the lesion, but other clinical and laboratory data should be used first to avoid invasive diagnostic tests (Burneo, Plener, & Garcia, 2009; Del Brutto et al., 2001; Kraft, 2007). With patients who are severely developmentally delayed or have cognitive deficits, such as autistic patients, eye examinations and radiologic imaging often must be done under sedation. 272
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The ELISA has been reported to have 98% sensitivity and 100% specificity. The serologic diagnosis of T. solium may be done with the enzyme-linked immune-transfer blot for detection of any circulating antibodies. A negative serologic result, however, does not exclude cysticercosis (Khosla, 2008). The sensitivity of serologic testing is decreased in patients with calcified lesions (Khosla, 2008). 3. What key historical finding leads you to consider neurocysticercosis? Contact with T. solium must occur for infection. Travel to an endemic area or any endemic exposure to a person from or living in an area with cysticercosis raises suspicion for this diagnosis. T. solium is transmitted mainly in areas where pigs have access to untreated human sewage or feces (Kraft, 2007). The parasite has been eradicated in the more developed countries with improved sanitation and control of Neurocysticercosis domestic pig farming. is a parasitic T. solium is endemic in China, Southeast infection caused by Asia, India, sub-Sahara the pork tapeworm Africa, and Latin AmerT. solium. ica (Kraft, 2007). In Mexico alone, 3.1% to 3.9% of people carry the parasite (World Health Organization [WHO], 2009). In the United States, the disease is usually found in immigrants from Mexico and Central and South America (WHO, 2009). Furthermore, the presence of multifocal calcifications on an MRI scan is highly suggestive of cysticercosis, especially in patients from endemic areas (Khosla, 2008). Neurocysticercosis is a parasitic infection caused by the pork tapeworm T. solium. Infection occurs in humans when ingesting eggs or larvae from undercooked pork containing the larvae or from food or water contaminated with fecal matter (John & Petri, 2006). After ingestion, the ova release oncospheres that eventually reach the bloodstream and are able to develop into cysticerci in any organ but are most common in the brain and eyes (John & Petri, 2006). Clinical symptomatology depends on the number and location of the cysts and the immune system’s response to the parasite. Most patients present with clinical signs and symptoms, such as seizures or other neurologic signs such as headaches and increased intracranial pressure (Khosla, 2008). Patients will present any time from 2 to 10 years from when cysts become mature (Davis, 2005). Because of the long incubation period, children do not account for a large percentage of clinical presentations of neurocysticercosis, and seropositive results tend to increase with age (Khosla, 2008). Neurocysticercosis is a leading cause of seizures and epilepsy in developing countries (DeGiorgio, Medina, Reynalds, & Pietsch-Escueta, 2004). Chief symptoms are often seizures (70% to 90%) and headaches, but patients also may present with nausea, vomiting, Journal of Pediatric Health Care
behavioral disturbances, or memory impairment (DeGiorgio et al., 2004). Cysts and disease in the brain develop in almost 60% of patients with cysticerci (John & Petri, 2006). 4. What is the appropriate treatment for neurocysticercosis? Antihelmintic drugs should be considered only after a thorough ophthalmic examination to rule out any intraocular cysts before treatment with a cysticercoidal drug. Cysticidal drugs cause cyst degeneration and can result in visual impairment if cysts are present in the eye (Garcia et al., 2002). This patient did not have any ocular involvement as evidenced by an ophthalmologic examination under sedation, so cysticidal medication was indicated. Surgery is indicated for ophthalmic cysticercosis, and antihelminth drugs should be avoided until ocular lesions are removed (Garcia et al., 2002). Albendazole and praziquantel are the principal cysticidal drugs (DeGiorgio et al., 2004; Sotelo & Del Brutto, 2002). Albendazole is preferred over praziquantel because it is less costly and has a better clinical response for seizure control in persons with neurocysticercosis (Burneo et al., 2009; Matthaiou et al., 2008). Albendazole should be given at 15 mg/ kg/day (up to 400 mg twice daily) for 4 weeks to reduce seizure recurrence and existing lesions (Bureno, 2009; Matthaiou, Panos, Adamidi, & Falagas, 2008). Adverse effects include nausea, headache, cerebral edema, gastrointestinal upset, seizures, and increased intracranial pressure (Carpio, 2002). Steroids commonly are used with cysticercosis in the brain because cysticidal drugs damage the parasite and release antigens that often trigger inflammatory response (Burneo et al., 2009). Corticosteroids may decrease the seizure frequency (Yancey, Diaz-Marchan, & White, 2005). An openlabel randomized trial found a reduced seizure rate when prednisone therapy, 1 mg/kg/day for 10 days, was followed by a 4-day tapering of the drug (Mall, Agarwall, Garg, Kar, & Shukla, 2003). 5. What is the recommended follow-up for neurocysticercosis? Neuroimaging should be repeated in 3 months, after completion of therapy with albendazole. If there is only a partial resolution of the cystic lesion, a repeat MRI scan of the head should be repeated in 3 months (Garcia et al., 2002). A second course of albendazole may be needed if the lesions are persistent after 6 months (Garcia et al., 2002). Some patients will require long-term antiepileptic medications such as phenytoin (Dilantin) and carbamazepine (Tegretol) for seizure control (Kraft, 2007). Referral to a pediatric neurologist or epilepsy clinic is indicated. Seizure recurrence depends on the number of cyst calcifications on an MRI scan and the cyst burden of the brain. Prognosis varies from patient to patient and the specific clinical findings in each case. The patient should www.jpedhc.org
follow up with a neurologist for this condition every 6 months, and the patient’s clinical course and outcome should be communicated to the patient’s primary care provider. 6. What patient education would you provide for the family? Emphasizing the importance of cooking pork thoroughly or keeping unused portions frozen along with close inspection of the meat should be taught to people who frequently travel or live in endemic areas. Consumption of raw or undercooked meat Neurocysticercosis should be avoided. can present in The WHO stresses the importance of treating many forms or vaccinating pigs depending on cyst against cysticercosis location and (WHO, 2007). Cysticercosis can be prevented disease by routine inspection progression. of meat and destruction of infected meat by the local government. However, in areas where food is scarce, cyst-infected meat is still eaten, which is one of the reasons cysticercosis has not been eliminated in endemic area (WHO, 2007). Family care and support is important for patients with neurocysticercosis infection. Case management may be necessary to coordinate infectious disease follow-up, neurology visits, or possible referral to an epileptologist. The patient’s medication regimen and rationale should be reviewed with the parents, and the importance of compliance with follow-up diagnostic imaging and the health care provider must be emphasized. The family also can be given useful references to explore, such as the Neurocysticercosis and Epilepsy Research Network and the Centers for Disease Control Working Group on Parasitic Diseases at http://whqlibdoc.who.int/hq/2007/WHO_ CDS_NTD_2007.3_eng.pdf Neurocysticercosis can present in many forms depending on cyst location and disease progression. It should be high on the list of differential diagnoses in children presenting with new-onset seizures who have traveled to endemic areas. Neurocysticercosis is a leading cause of epilepsy in the developing world (DeGiorgio et al., 2004). Treatment with albendazole improves the prognosis for recurrent tonic-clonic seizures in highly selected patients. The WHO is developing and enforcing global strategies for control (DeGiorgio, 2004). INTERPRETING A MEDICAL HISTORY WHEN YOUR PATIENT HAS DIFFICULTY COMMUNICATING Autistic children have impaired communication skills and sometimes are unable to verbalize symptoms of July/August 2010
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pain such as headaches. The patient presented with increased irritability, per the mother’s report. Providers who are taking care of children with any disorder involving impaired communication need to pay close attention to the caregiver’s report of changes in behavior or activity. This patient presented with possible headaches, as suggested by the way he repetitively held his head and had increased irritability and sensitivity to lights. Patients with any pervasive developmental disorder or who present on the autistic spectrum present the practitioner with an added challenge in compiling a medical history. SUMMARY This patient presented to a specialty clinic that routinely monitored him for autism. An electroencephalogram and MRI scan were ordered as part of a chief complaint for new-onset seizures. The scolex of a tapeworm was an incidental finding that is diagnostic for tapeworm. Pork tapeworm is the most common tapeworm parasite in Mexico. Cysticidal medication was indicated because the eye examination was within normal limits. The patient should receive close follow-up from the neurology team with ongoing communication to the primary care provider. REFERENCES Burneo, J., Plener, I., & Garcia, H. (2009). Neurocysticercosis and epilepsy research network. Canadian Medical Association, 180(6), 639-642. Canitano, R. (2006). Epilepsy in autism spectrum disorders. European Child and Adolescent Psychiatry, 16(1), 61-66. Carpio, A. (2002). Neurocysticercosis: An update. The Lancet Infectious Diseases, 2, 751-762. Danielsson, S., Gillberg, C., Billstedt, E., Gillberg, C., & Olsson, I. (2005, June). Epilepsy in young adults with autism: A prospec-
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tive population-based follow-up study of 120 individuals diagnosed in childhood. Epilepsia, 46(6), 918-923. Davis, L. E. (2005). Neurocysticercosis. In C. Power & R. T. Johnson (Eds.), Emerging neurological infections (pp. 261-287). Boca Raton, FL: Taylor & Francis Group. DeGiorgio, C. M., Medina, M. T., Reynalds, Z., & Pietsch-Escueta, S. P. (2004, May). Neurocysticercosis. Epilepsy Currents, 4(3), 107-111. Del Brutto, O. H., Rajshekhar, V., White, A. C., Tsang, W., Nash, T. E., Takayanagui, O. M., & Garcia, H. H. (2001). Proposed diagnostic criteria for neurocysticercosis. Neurology, 57, 177-183. Garcia, H. H., Evans, C. W., Nash, T., Takayanagui, O. M., Clinton White, A., Botero, D., & Del Brutto, O. H. (2002, Oct). Current consensus guidelines for treatment of neurocysticercosis. Clinical Microbiology Reviews, 15(4), 747-756. Khosla, A. (2008). Cysticercosis CNS. eMedicine Radiology. Retrieved from http://emedicine.medscape.com/article/339654. Kraft, R. (2007, July 1). Cysticercosis: An emerging parasitic disease. American Family Physician, 76(1), 91-96. Mall, R. K., Agarwal, A., Garg, R. K., Kar, A. M., & Shukla, R. (2003). Short course of prednisolone in Indian patients with solitary cysticercus granuloma and new-onset seizures. Epilepsia, 44(11), 1397-1401. John, D. T., & Petri, W. A. (2006). Markell and Voge’s medical parasitology (9th ed.). Philadelphia, PA: Saunders. Sotelo, J., & DelBrutto, O. H. (2002). Review of neurocysticercosis. Neurosurgical FOCUS, 12, 1. Matthaiou, D. K., Panos, G., Adamidi, E. S., & Falagas, M. E. (2008, March 12). Albendazole versus praziquantel in the treatment of neurocysticercosis: A meta-analysis of comparative trials. PLoS Neglected Tropical Disease, 2(3), e194. World Health Organization. (2007, March). Global plan to combat neglected tropical diseases 2008-2015. Retrieved from http://whqlibdoc.who.int/hq/2007/WHO_CDS_NTD_2007 .3_eng.pdf World Health Organization. (2009). Taeniasis/cysticercosis. Retrieved from http://www.who.int/zoonoses/diseases/taeniasis/ en/index.html Yancey, L. S., Diaz-Marchan, P. J., & White, A. C. (2005). Cysticercosis: Recent advances in diagnosis and management of neurocysticercosis. Current Infection Disease Report, 7(1), 39-47.
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