|Year : 2022 | Volume
| Issue : 6 | Page : 916-921
Case series on the clinical profile of neurocysticercosis in pediatric age group
Shiji S Chalipat, Sanjay Chavan, Sudhir D Malwade, Sharad R Agarkhedkar, Soni Kumari
Department of Pediatrics, Dr. D. Y. Patil Medical College and Research Centre, Pimpri, Pune, Maharashtra, India
|Date of Submission||23-Mar-2022|
|Date of Decision||15-Apr-2022|
|Date of Acceptance||15-Apr-2022|
|Date of Web Publication||23-Dec-2022|
Dr. Soni Kumari
Department of Paediatrics, Dr. D.Y. Patil Medical College Hospital and Research Centre, Pimpri, Pune, Maharashtra - 4111018
Source of Support: None, Conflict of Interest: None
Introduction: Neurocysticercosis (NCC) is a common infection of the nervous system caused by ingestion of Tenia solium eggs in undercooked pork or contaminated water. Most children present with seizures particularly focal seizures with or without signs of raised intracranial pressure (ICP). Diagnosis is made by computed tomography/magnetic resonance imaging (CT/MRI) scan which shows single/multiple ring-enhancing lesions with perilesional edema. Management involves the use of anticonvulsants for seizures and steroids for edema. Objective: To establish a clinical profile of NCC patient in pediatric age groups. Material and Methods: The Department of Pediatric Neurology at our institute conducted a retrospective study of 15 children with ring-enhancing lesions on MRI who were managed at this centre. The observations were further analyzed. Results: Nearly 14 children presented with seizures, 50% of these children also showed signs of raised ICP. Only 1 child had presented with signs of raised ICP. All the children were treated with anti-cysticidal, anti-epileptic with pre-treatment steroids. At 6 months all children were seizure-free. Conclusion: NCC should be ruled out in children presenting with focal seizures, especially in a country like India where the disease is endemic. Apart from antiepileptics, anthelmintic – Albendazole and Praziquantel are very effective against NCC with pre-treatment with corticosteroids and have been very effective in managing the disease.
Keywords: Neurocysticercosis, pediatric seizures, seizures
|How to cite this article:|
Chalipat SS, Chavan S, Malwade SD, Agarkhedkar SR, Kumari S. Case series on the clinical profile of neurocysticercosis in pediatric age group. Med J DY Patil Vidyapeeth 2022;15:916-21
|How to cite this URL:|
Chalipat SS, Chavan S, Malwade SD, Agarkhedkar SR, Kumari S. Case series on the clinical profile of neurocysticercosis in pediatric age group. Med J DY Patil Vidyapeeth [serial online] 2022 [cited 2023 Jan 30];15:916-21. Available from: https://www.mjdrdypv.org/text.asp?2022/15/6/916/365004
| Introduction|| |
Neurocysticercosis (NCC) is the most common infection of the central nervous system caused by cestode – Tenia Solium. Humans become infected after consuming raw or undercooked, infected pork, or food and water contaminated with tapeworm eggs, or due to poor sanitation leading to feco-oral transmission. This is an endemic zoonosis in the developing world, including India. The World Health Organization (WHO) considers cysticercosis among one of the “Neglected tropical zoonotic disease.” According to the WHO 30% of all epilepsy cases in endemic countries and 3% of epileptic cases globally may be due to NCC. NCC is considered as the most common preventable cause of epilepsy in developing world. While developing countries have endemicity of this disease, Incidence of NCC has increased in the recent years as more people are immigrating from endemic areas. Diagnosis of NCC is primarily neuro radiologic (computed tomography/magnetic resonance imaging – CT/MRI). The main radiologic findings suggestive of NCC include: cystic lesion with scolex in the center (dot in a hole sign); ring or disc enhancing lesions; punctate calcifications brain parenchyma. Management of NCC in children generally includes anti-helminthic, antiepileptics, and corticosteroids.
| Patients and Methods|| |
We performed a retrospective descriptive study that included 15 patients with NCC who presented at the Pediatric Neurology Division, Department of Pediatrics, at our institute in the past 1 year (January 2021 to December 2021). The Institutional Ethics has approved data collection and their publishing for all the patients. All cases which have been diagnosed and treated as “seizures with ring-enhancing lesions” by Pediatric neurology were identified in phase 1. In phase 2, their medical charts, clinical profile, and radiological and other investigation were reviewed.
Children in the age group of 2–12 years diagnosed with NCC who were admitted to the pediatric ward. Diagnosis of NCC was based on clinical presentation and MRI scans which were consistent with NCC. These included:
- Solitary well-defined thin-walled cystic or ring-enhancing lesion usually <2 cm in size with mild perilesional edema in a typical gray-white matter junction or basal ganglia.
- Multiple lesions in different stages.
Children with tuberculomas or ring-enhancing lesions secondary to other etiologies were excluded. MRI indication of tuberculoma included the presence of hyperintense central core lesions. Children with calcified granuloma were also excluded. Patients whose data of at least 6 months follow-up was not available were excluded.
Out of 21 children with ring-enhancing lesions, A total of 15 cases were selected, their clinical presentation, radiological features, treatment received, response to treatment, seizure-free period till the last follow-up, and their status at 6 months of follow-up were all recorded and analyzed. Resolution of scolices on follow-up MRI was defined by the absence of perilesional edema. Isointense lesion core surrounded by edema meant partial resolution. The treatment protocol followed at our institute included corticosteroids, cysticidal drugs, and anti-seizure medications in children with acute symptomatic seizures. The choice of Corticosteroid was Prednisolone given as 2 mg/kg/day for 1 week followed by tapering over the next 1 week. Cysticidal drugs included Albendazole given as 15 mg/kg/day in two divided doses for 28 days; Praziquantel at 50 mg/kg/day in 3 divided doses for 14 days. Anti-seizures medications used included Oxcarbazepine (15 mg/kg/day) and levetiracetam (20 mg/kg/day).
| Results|| |
The demographic and clinical details of the patients are presented in [Table 1]. A total of 15 patients were included in the study. All the patients belonged to the age group of 6–12 years. Number of males was two times more than the number of females (male: female ratio, 2:1). Of them, 66% of the individuals were vegetarian and none of them had a history of pork consumption. All the 15 patients were from poor socioeconomic background. Nearly 14 out of 15 children had presented with seizures as primary complaints. One patient had no seizure, but presented with complaints of headache and vomiting with signs of raised intracranial tension (ICT). Of the 14 patients who presented with seizure 7 had symptoms of raised ICT – headaches, nausea, and vomiting. Most common seizure type was focal onset motor seizures progressing to bilateral convulsive seizure 13 980%. Electroencephalogram (EEG) was done in ten patients, out of which three children had abnormal findings like focal slowing and focal sharp waves.
|Table 1: Demographic and clinical details of 15 patients included in the study|
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All children had undergone MRI brain plain plus contrast study, which showed solitary lesions in nine (60%) children and multiple lesions in six (40%) children. Majority of the 14 (94%) children had supra tentorial lesions and only 1 (6%) child had both supra tentorial and infra tentorial lesions. The lesions were predominantly distributed over the frontal lobe in ten (70%) children, followed by the parietal lobe in 3 (20%) children and then in occipital lobe. One child had lesions in Basal Ganglia and Thalamus [Figure 1]. Perilesional edema with mass effect was noted in 12 (80%) cases.
|Figure 1: MRI scan showing multiple parenchymal cystic lesions involving the parietal, occipital frontal and the basal ganglia [case 4]|
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All the children were treated with oral steroids for 48 h before starting cysticidal drugs, corticosteroids were continued till 1 weeks, following which it was tapered off over 1 week. Cysticidal drugs – albendazole was given for 28 days to all children with single lesions. While six children who had multiple lesions were treated with albendazole and praziquantel for 28 days. Antiepileptic drugs used were levetiracetam and oxcarbazepine. At 6 months follow-up, all the children were seizure-free for more than 3 months. Follow-up MRI was done for one patient who had extra-parenchymal involvement, and it showed successful resolution of the cysts.
| Discussion|| |
Cysticercosis is caused by ingestion of the eggs of Tenia solium. Dissemination of cysticerci in the central nervous tissue causes NCC. The cysticerci in the brain undergo four stages of involution. Stage 1: vesicular stage – characterized by a cyst with translucent walls and a viable scolex. Stage 2: the colloidal stage, which is characterized by a cyst with a thick wall. Stage 3: The granular stage is a stage with a cyst with a thicker wall, degenerated scolex. Stage 4: Final stage is calcific stage, the cyst transforms into a coarse calcified nodule.
NCC is one of the major causes of epilepsy in the pediatric population in India and is prevalent in almost all of the states in India. The prevalence varies between the states. NCC has been classically associated with consumption of improperly cooked pork. Surprisingly more than 95% patients in India were vegetarian by diet, pointing toward poor hygiene practices as a root cause of transmission and endemicity of the disease. Poor hygiene practices both personal and that of the community, contaminated water supply may all have a role in transmission. About two-third of our cases were vegetarian by diet and none were consumers of pork. All of them belonged to poor socioeconomic status.
Clinical features of NCC vary widely in children and continue to pose a challenge in clinical practice. The presentations may be myriad depending on the stage, number, viability, and location of the cyst. Most common symptoms are focal seizures, generalized seizures, focal neurological deficit, and signs of raised ICT. A study of 500 children with NCC in India reported that almost 95% of the cases had seizures, of these 84% cases had focal seizures, while 30% of children showed symptoms of increased ICT and 4% showed neuro deficit. Two studies in India showed that generalized seizure to be a more common type of seizure associated with NCC., Presentation in extra parenchymal NCC is pleomorphic, and depending on the location it may include vasculitis, stroke, dorsal midbrain syndrome, ptosis, dystonia, and neurocognitive deficits., In our case series most of the children (14 out of 15) had seizures, while one case presented with persistent headache, the fundoscopy revealed signs of papilledema. Of those 14 cases 7 also had features of raised ICP like vomiting and headaches. The most common seizure type was focal onset seizures and only one child presented with generalized seizure with unknown onset.
Computed tomography/magnetic resonance imaging
Routine diagnosis is made by CT/MRI scan which shows single/multiple ring-enhancing lesions with perilesional edema [Figure 1]. Both WHO guidelines and Indian guidelines agree that contrast-enhanced MRI remains the investigation of choice for NCC, CT can be done if MRI is unavailable, CT scan is preferred for delineating calcified lesions. The imaging characteristics of typical parenchymal NCC vary according to the pathological staging of the cyst. The commonest CT/MRI finding in India is “single small enhancing lesion” – a single, <20 mm, with perilesional edema. Multiple cysts in varying stages give rise to a “starry-sky” appearance. One of the patients in this series had both supra tentorial and infra tentorial lesions, along with lesions in thalamus and basal ganglia.
There is no EEG finding which is specific to NCC. Findings may range from absolutely normal EEG to periodic lateral epileptiform discharges, focal sharp waves or spikes, and/or slowing or epileptiform discharges. A study in India involving 115 cases reported that in cases with calcified granuloma EEG may be helpful in predicting recurrence of seizures. However, many authors have reported no evidence of association between EEG and clinical outcomes in NCC.,, In our series only ten patients had EEG done. Three patients had EEG changes including focal slowing and focal spikes, while seven were normal. The presence of EEG abnormalities did not seem to affect either clinical presentation or the final outcome. Also, presence or absence of EEG was not associated with the number of lesions. One of these three cases had single lesions, while two had multiple lesions.
There are no clear guidelines for managing and diagnosing the disease. Few consensus-based guidelines exist. The guidelines published in 2017 by Infectious Diseases Society of America (IDSA) and the American Society of Tropical Medicine and Hygiene (ASTMH) were not totally feasible for the management of this disease in developing nations. Association of Child Neurology of India has issued a consensus guideline, and the WHO too has issued consensus-based guidelines of their own in 2013. We do not do serological testing for NCC, their clinical use remains doubtful. The Indian consensus guidelines strongly opposed their routine use in managing and diagnosing.
There seems to be a common consensus in the use of anthelminthic, corticosteroids, and antiepileptics. Both the Indian and the WHO guidelines favor their use. A recent meta-analysis involving 16 trials and another involving 15 trials have concluded that albendazole was effective in early resolution and radiological clearance of lesions and also preventing seizures, Both albendazole and praziquantel had been found equally effective in radiological eradication of lesions on CT scan in a controlled trial. Corticosteroids are administered to reduce host inflammatory reaction and increase level of albendazole sulfoxide in plasma. Destruction of cysticerci releases antigens which induce an inflammatory host response. Adverse effects like fevers, seizures, headaches, nausea, altered sensorium, raised ICP have been observed after cysticidal therapy. Corticosteroids should be administered before commencing cysticidal therapy to reduce these adverse effects, especially in cases with multiple cysts, extra-parenchymal cysts, ventricular lesions, and disseminated lesions.
The antiepileptic drugs are no different in NCC than in other seizure disorders. We favor use of oxcarbazepine and levetiracetam and they have been very effective in preventing seizures in all patients of NCC that we treated. The duration for which we continued seizure prophylaxis was tailored to the patient. For patients with single lesions, we continued seizure prophylaxis till 6 months of seizure-free period. This is in line with the WHO and Indian guidelines. Continuing antiepileptics for 24 months is advised if there is calcification of the lesion or non-resolution of lesions on neuroimaging.
This study shows that NCC is endemic in India and is caused mostly due to unsanitary hygienic practices, and is therefore a public health problem also. The clinical features are pleomorphic, but NCC should be kept in the list of differentials for children presenting with seizures, raised ICT, or central neurological deficits, especially in a country like India where the disease is endemic. MRI with contrast is a great tool for reaching diagnosis and differentiating it from tuberculoma. Cysticidal drugs, with corticosteroids and anti-epileptics are very successful in resolution of lesions and gaining seizure free intervals. Neuroimaging on follow-up is not necessary, especially the financial burden it puts on the patients who often belong to economically disadvantaged sections of the society, and it should be reserved for cases with breakthrough seizures, or unremitting seizures.
| Conclusion|| |
NCC continues to be a major burden on the community. Though guidelines for management and diagnosis are few and subject to debate, but as evident in our findings that the consensus holds true to some extent. Focal seizures are the most common presentation, and NCC should be ruled out in children presenting with this, especially in a country like India where the disease is endemic. Contrast MRI is a valuable tool for diagnosing NCC. While most cases have single <2 cm ring enhancing lesions, there may be few cases with multiple lesions and even extra-parenchymal lesions which may present with a myriad focal neurological deficit. Apart from antiepileptics, anthelmintic – Albendazole and Praziquantel are very effective against NCC, but they should be started only after 48 h of systemic corticosteroids. We have got very favorable results with our regimen and by 6 months all our patients were seizure free.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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