Two Cases of Neurocysticercosis in Indigenous Papuans: Diagnostic and Public Health Challenges

Introduction

Neurocysticercosis (NCC) is a neglected tropical disease that remains a significant public health challenge in many low- and middle-income countries where Taenia solium transmission is endemic. NCC results from infection with the larval stage of T. solium in the central nervous system [1]. Humans, the definitive hosts, acquire intestinal tapeworms by consuming undercooked pork containing larvae [2]. Parasite eggs passed into the environment via human feces are ingested by pigs who develop cysts throughout their bodies, including in muscle. Similar to pigs, humans can also become infected with the larval stage of T. solium through the fecal-oral route [3].

NCC is a major cause of acquired epilepsy and other neurological disorders in endemic regions. Clinical manifestations associated with NCC include seizures, focal neurological deficits, intracranial hypertension, cognitive decline, headaches, stroke, and involuntary movements [1]. Indonesia is recognized as an endemic country for T. solium [4]. However, documentation of human NCC in Papua remains limited. The first confirmed case in Papua was reported in a 35-year-old indigenous man from Irian Jaya (now Papua) in 1973 who sustained burns during a seizure [5]. Since then, only 2 additional reports have documented NCC in Papua, 3 confirmed cases in Mimika Regency in 2014, and a 2020 report of suspected NCC in a Papuan-born man residing in Manado [6,7]. This report describes 2 computed tomography (CT)-confirmed cases of NCC presenting with rarer clinical manifestations and stresses the urgent need to strengthen diagnostic capacity for NCC in this underreported endemic region.

Case Reports

CASE 1:

In July 2023, an 11-year-old girl from Lanny Jaya Regency presented to Wamena Regional General Hospital (WRGH) in Jayawijaya Regency with visual hallucinations and progressive blindness. Upon physical examination, she was conscious, with normal vital signs: blood pressure (BP) 110/70 mmHg, heart rate (HR) 90 bpm, respiratory rate (RR) 34 bpm, and temperature 36°C. She had a history of seizure onset starting in 2020, prompting a CT scan in Makassar (South Sulawesi Province), where she was initially diagnosed with NCC. In February 2020, a follow-up multi-slice computed tomography (MSCT) scan of the head was conducted in Jayapura (Papua Province) using axial, sagittal, and coronal reformatting. Imaging revealed multiple small, circular, and diffusely distributed hypodense lesions with calcification in the cortical and subcortical areas of both cerebral hemispheres, as well as in the cerebellar parenchyma. No enhancement was observed following contrast administration. Additionally, physiological calcifications were noted in the pineal gland and bilateral choroid plexus. The findings were interpreted as NCC with encephalitis and vasogenic edema, but treatment was not initiated.

In 2023, the neurologist at WRGH confirmed the diagnosis of NCC based on a re-evaluation of the February 2020 MSCT scan, noting cerebral edema and multiple dead or calcified cysts without evidence of midline shift. The patient was prescribed carbamazepine (100 mg twice daily for 15 days) and methylprednisolone (4 mg twice daily for 10 days), with instructions to return for follow-up after 2 weeks. Unfortunately, the patient did not return for follow-up evaluation, and the progression of her condition after therapy remains unknown. CT scan images are not included in this report due to image display challenges associated with the utilized scanner.

CASE 2:

In February 2023, a 20-year-old man from Nduga Regency was referred to WRGH’s Neurology Department after initial psychiatric assessment for hebephrenic schizophrenia. At the initial psychiatric consultation, the patient appeared calm but uncooperative, with limited eye contact, generalized weakness, and excessive salivation. He was prescribed 50 mg chlorpromazine, 10 mg aripiprazole, and 1 mg haloperidol daily for 2 weeks. During the second consultation, approximately 1 month later, he showed minimal verbal and visual interaction, flat affect, excessive salivation, and decreased psychomotor activity. The psychiatrist continued management with chlorpromazine. At the third visit, worsening neuropsychiatric symptoms, including disorganized behavior, generalized tonic-clonic seizures (GTCS), aphasia, and slurred speech, led to a neurological referral. Laboratory testing revealed positive hepatitis B surface antigen (HBsAg), but no T. solium serology was performed.

A non-contrast MSCT scan of the head performed in February 2023 using axial, sagittal, and coronal reformatting demonstrated multiple small, circular calcifications (less than 1 cm in diameter) distributed extensively throughout the cerebral parenchyma (Figure 1). A hypodense lesion with water density (13 HU) was identified in the right maxillary sinus, along with an 8° rightward nasal septum deviation. Bilateral orbits and mastoid processes were normal, and the calvaria was intact without osteodestruction. Cerebral sulci, gyri, ventricles, cisterns, pons, cerebellum, and cerebellopontine angles were normal. The neurologist confirmed NCC and noted the appearance of viable cysts on MSCT. The patient was prescribed phenytoin (100 mg twice daily for 15 days) and methylprednisolone (4 mg twice daily for 15 days), in addition to his existing psychiatric medications. He was instructed to return for follow-up every 2 weeks, but he did not attend further consultations, and the progression of his condition after NCC therapy remains unknown.

Discussion

NCC diagnosis relies on meeting standardized criteria, such as those proposed by Del Brutto et al (2017) (Table 1) [8]. Case 1 demonstrated parenchymal brain calcifications with no enhancement. According to the Del Brutto criteria, typical parenchymal brain calcifications (single or multiple, solid, and generally <10 mm in diameter) are a major neuroimaging criterion of NCC. The combination of calcifications with 2 supporting clinical/exposure features (residence in an endemic area and NCC-compatible neurological manifestations) supports a definitive diagnosis. The CT scan for Case 2 revealed multiple cystic lesions, some without a clearly discernible scolex and others exhibiting the classic “hole-with-dot” sign, suggesting viable cysts, which is a pathognomonic sign of NCC and an absolute criterion confirming NCC [8].

Case 1 presented with recurrent seizures, visual hallucinations, and progressive bilateral vision loss. Seizures and epilepsy are the most common neurological manifestations of NCC, reported in 78.8% of patients (95% CI: 65.1–89.7) [9]. Visual hallucinations followed by progressive blindness are rarely reported in NCC, with a systematic review providing a pooled prevalence estimate of 5.6% (95% CI: 1.1–13.5) [9]. These symptoms may suggest occipital lobe involvement, intracranial hypertension, or optic pathway compression, although lesion localization could not be confirmed without follow-up imaging [10].

Case 2 displayed a more complex neurological and psychiatric profile, including disorganized behavior resembling hebephrenic schizophrenia, GTCS, and aphasia. GTCS often results from cortical irritation due to multiple parenchymal cysts [11]. Psychiatric symptoms, such as schizophrenia-like behavior, are rarer with NCC. Aphasia and slurred speech are also considered rare NCC presentations [12]. Harshitsha et al (2023) documented Broca’s aphasia as an isolated manifestation, likely due to a cyst affecting the speech motor cortex [13]. Whether the psychiatric symptoms in Case 2 arose directly from NCC or were coincidental remains unclear. Further neuroimaging and neuropsychological assessment would be required to clarify causality.

Neither of our 2 patients received antiparasitic therapy. According to World Health Organization recommendations, antiparasitic treatment is contraindicated in patients with extensive cystic lesions and elevated intracranial pressure [14]. In such cases, corticosteroids alone are advised until inflammation subsides [15]. In Case 1, the presence of cerebral edema justified corticosteroid therapy without antiparasitic agents. In Case 2, although edema was not evident, the extensive parenchymal cyst burden raised a concern for potential inflammatory reactions; thus, clinicians deferred antiparasitic therapy until further reassessment could confirm stability. Unfortunately, the patient did not return after the initial course of corticosteroid and anti-epileptic therapy.

These 2 cases represent relatively strong diagnostic approaches in the local context, as both used CT scans to support the clinical diagnosis of NCC. However, access to neuroimaging is extremely limited in Papua; CT scanners are confined to a few urban hospitals and often have prolonged downtime. Geographic isolation and socioeconomic challenges exacerbate diagnostic and treatment gaps. In 2019, the Highland Papua region accounted for nearly 50% of the province’s 926 630 low-income residents (earning below USD 62.11 per month) [16].

Both patients discontinued follow-up care, preventing assessment of treatment outcomes or disease progression. There is often reluctance for NCC patients to seek follow-up care, and patients may discontinue treatment or avoid returning due to long travel distances, limited awareness of possible severe clinical outcomes, and sociocultural or geopolitical conditions. Regular follow-up consultations can serve as opportunities to apply confirmative neuroimaging criteria over time, such as demonstrating cyst resolution after antiparasitic treatment. Addressing these diagnostic and treatment gaps will require expanding training for healthcare providers, enhancing referral systems between rural and tertiary centers, and improving access to antiparasitic and essential medicines. Public health interventions must also target transmission prevention through improved sanitation, elimination of open defecation, and better pig husbandry practices [17]. Ultimately, eliminating NCC in endemic regions like rural Papua will require ensuring that even the most remote communities have access to accurate diagnosis, effective care, and the basic tools for prevention.

Conclusions

Two CT-confirmed cases of NCC with atypical features, including blindness, visual hallucinations, and psychiatric manifestations, were reported from a hospital in Highland Papua. Neuroimaging revealed both calcified and viable cysts. Both patients received symptomatic management without follow-up. These cases highlight the occurrence of rarer clinical forms of NCC and the urgent need to strengthen diagnostic capacity and evidence-based clinical management in rural Papua.