In this case of sparganosis in HIV/AIDS patient, we found out that the pathogenesis of sparganosis in this immunocompromised patient was the same as in other reported cases of immunocompetent patients.
In this case, the patient had lived in two endemic areas in China; Guanxi province- her home town and Guangdong province where she used to work. The most probable source of infection could be drinking infested water as per epidemiologic history. Sometimes the epidemiological history is atypical (2,3).
Cerebral sparganosis is a diagnosis of exclusion and is misdiagnosed in most cases. This case wasn’t an exception as it was first diagnosed and treated as secondary epilepsy for 5 years. Wang et al found up to 11 years of misdiagnosis. In their study, all 24 cases were first misdiagnosed as glioma, brain abscess, brain tuberculosis and primary epilepsy (3). In a review of 6 European cases, the misdiagnosis rate was 100%. They were diagnosed as neurocysticercosis, cerebral abscess or dysembryoplastic neuroepithelial tumour (4). The clinical manifestations of sparganosis are atypical and vary depending on the site of infection. Cerebral sparganosis commonly involves the frontal lobe. It can also invade other parts such as the parietal lobe, temporal lobe, occipital lobe, basal ganglia or cerebellum. The main clinical symptoms are seizures, limb weakness, headache, or physical disturbances (2, 3). In severe cases, it can lead to intracranial hypertension, visual impairment, consciousness disorder, or sudden death.
In terms of auxiliary examination, the patient’s peripheral blood white blood cell count and eosinophils count were not elevated but IgE was elevated. Apart from the raised opening pressure and glucose, there was no obvious abnormality in the routine examination of cerebrospinal fluid. The reported cases of cerebral sparganosis in non-HIV patients have shown some variability in the cerebral spinal fluid analysis with most cases presenting with raised white cells and proteins while others having normal values. Peripheral blood and cerebrospinal fluid sparganosis antibody test has high specificity and sensitivity and is an important auxiliary diagnostic method (3). Combined with head imaging, it can confirm the diagnosis and improve the diagnosis rate. Studies have reported immunologic tests to have cross-reactivity with other cestodes but when combined with a thorough history and radiological imaging especially MRIs, it plays a significant diagnostic role.
In particular, enhanced MRI of the head has significant advantages in the diagnosis of the disease, it shows the following features: multiple small nodules in the brain with low signal on T1WI and high signal on T2WI, which can be a small ring, bead-like, orbital or tubular enhancement. There is a large area of cerebral oedema around the lesion. The larva can migrate across the lobes of the brain and across the midline, and brain atrophy occurs in the primary lobe after the migration (2,5-6). This patient had multiple intracranial lesions and obvious oedema bands around the lesions, which showed linear, nodular, and ring-enhancing lesions after enhancement, which is consistent with the literature.
Surgical removal is the first choice for the treatment of cerebral sparganosis. Image-guided stereotactic aspiration is currently an ideal surgical option as it is highly effective and less traumatic (7).
For patients whose lesions are located in major functional areas, those who don’t want invasive treatment or whose general conditions cannot tolerate surgery, medical treatment needs to be considered. Medical treatment with high dose praziquantel has shown to be effective in treating cerebral sparganosis in inoperable patients (2,8). In this case, we opted for pharmacological therapy in consideration of the compromised immunity in HIV/AIDS patients and the high risk of the operator's occupational exposure. At present, there is no unified medical treatment standard guide. The definitive treatment of cerebral sparganosis with medical therapy is still controversial. Some authors have declared it as effective as surgical treatment while others have declared it ineffective. A study involving 96 patients showed no significant difference between long term high dose praziquantel therapy and surgical treatment (9). Some patients often require multiple cycles of treatment, and there are still some patients, who cannot be cured with medical treatment (8).
This patient received four cycles of high dose praziquantel 25mg/kg tds for 10 days with and each cycle was a month apart therapy with significant improvement on clinical and radiological features. There was no seizure after the completion of the first cycle up to date, the patient is still being followed up every three months when she attends her routine HIV clinic.
The incidence of cerebral sparganosis is low and AIDS combined with cerebral sparganosis is even rarer. There are currently no reports of similar cases. We searched the CNKI, Wan Fang, Web of Science and Pub Med database with "HIV/AIDS + cerebral sparganosis" as the keywords but there were no related articles.
The clinical characteristics of this patient are similar to those of non-AIDS patients with cerebral sparganosis, which may be due to the high CD4+T cell count. Due to the immunosuppression of AIDS patients, parasitic infections in low immunity patients may have different characteristics. It is not excluded that patients with AIDS and cerebral sparganosis with low CD4+ T cell counts have more atypical intracranial lesions and their clinical manifestations may be more complicated and insidious, the discovery of more cases of cerebral sparganosis in HIV/AIDS patients may confirm this further. There is currently no consistent standard for the timing and choice of antiretroviral treatment for patients with HIV/AIDS existing with sparganosis, and there are no clinically available data on the merits of each regimen. In this case, we chose the regimen of Zidovudine + Lamivudine + Nevirapine as the first line mainly based on its high permeability of the blood-brain barrier.
Although cerebral sparganosis with HIV/AIDS is rare with no reported cases at present, it should be suspected especially in patients from endemic areas with possible epidemiologic history and radiological features.
When deciding the mode of treatment in an immunocompromised patient these factors need to be considered; the ability of the patient to withstand surgery, the surgeon's occupational risk and the post-operative recovery. Although surgical removal of the sparganum is the first choice of treatment, high dose praziquantel therapy has proved to be effective in inoperable cases. Clinical progress and follow up radiological images are of great use to ensure the death of the worms in the brain. Since there is currently no uniform standard for deworming and antiviral treatment guidelines, this case calls for immediate action on the matter.
2. Hong, Xie, Zhu, Wan, Xu, Wu, “Cerebral sparganosis”, 1515-1516.
3. Lei, Fei, et al, “Analysis”, 699-700.
4. Presti, Aguirre, Andrés, Doud, Fortes, Muñiz, “Cerebral Sparganosis”, 1342.
5. Liao, Li, Zhou, Liu, Li, Liu, Wu, Zhu, Tan, “Imaging”, 378-379.
6. Li, Ramsahye, Yin, Zhang, Geng, Zee, “Migration”, 328-329.
7. Deng, Xiong, Qian, “Diagnosis”, 1422.
8. Zhang, Zou, Yu, Wang, Lv, Liu, Ding, Zhang, Zhao, Yang, Wang, “Follow-up”, 3, 10-11.
9. Hong, Xie, Wan, An, Xu, Zhang, “Efficacy”, 10.