Acta Neurochir (2015) 157:1339–1343 DOI 10.1007/s00701-015-2466-9 CASE REPORT - BRAIN TUMORS Cerebral sparganosis: case report and review of the European cases Anna Lo Presti 1 & Daniel T. Aguirre 1 & Pablo De Andrés 1 & Lina Daoud 2 & José Fortes 2 & Jesús Muñiz 1 Received: 16 March 2015 / Accepted: 1 June 2015 / Published online: 18 June 2015 # Springer-Verlag Wien 2015 Abstract Sparganosis is a severe parasitic infection caused by the larvae of Spirometra mansoni, also called Bsparganum.^ In human hosts, the Spirometra mansoni larva commonly targets the subcutaneous tissue or muscle. Sometimes it can also migrate into the brain, resulting in cerebral sparganosis, mainly characterized by focal neurological symptoms such as seizures and radiological Bwandering lesions^ on magnetic resonance images (MRIs). Clinical cases of cerebral sparganosis have been reported worldwide, mainly in Asian countries, but also in North America, South America and Australia. Only two cases have been previously reported in Europe. A 29-year-old male from Bolivia, who lived in Spain, presented to our service for seizures and a multicystic brain lesion, initially suspected to be a dysembryoplastic neuroepithelial tumor (DNET). He underwent gross total resection of the mixed solid/ cystic lesion. Pathology revealed gliosis, multiple interconnected cystic cavities with fibrous walls, inflammatory cell infiltration and no necrotizing granulomatous reaction. Inside the cavities, a parasitic form was identified as the larva of the cestode Spirometra mansoni. At 1-year follow-up, the patient had no deficits and was seizure free. Clinicians should be alerted to the possible * Anna Lo Presti annaloprestivega@gmail.com 1 Department of Neurosurgery, Fundación Jiménez Díaz (IIS-FJD), Autonomous University, Av. Reyes Católicos 2, 28040 Madrid, Spain 2 Department of Pathology, Fundación Jiménez Díaz (IIS-FJD), Autonomous University, Madrid, Spain existence of this rare entity in Europe, especially in patients from endemic areas with a possible infection history as well as Bwandering lesions^ on the MRI. Keywords Human sparganosis . Cerebral sparganosis . Europe . Stereotactic aspiration . Praziquantel Introduction Sparganosis is a severe parasitic infection caused by the larvae of Spirometra mansoni, also called Bsparganum^ [4, 5, 8]. Adult Spirometra mansoni are mainly parasitized in the intestines of cats and dogs, producing eggs that pass out with feces and reach fresh water. Coracidia hatched from these eggs are then ingested by copepods (first intermediate host), developing into larvae that are ingested by frogs, snakes, birds or mammals in which procercoids mature into plerocercoid larvae [4]. Humans are infected with sparganum by drinking water contaminated with infected copepods, eating undercooked meat of snakes or frogs infected with sparganum, or by using poultices of frog or snake flesh or skin on open wounds [4, 8, 11]. The survival period of sparganum in the human body ranges from 5 to 20 years, commonly targeting the subcutaneous tissue or muscle. However, it can also migrate into the brain, resulting in the most harmful pattern of the disease: cerebral sparganosis [8, 16]. In the past decades, clinical cases of cerebral sparganosis have been reported worldwide, mainly in Asian countries, especially in China, South Korea, Japan and Thailand [2, 8, 17, 20], but also occasionally from North America [1, 3, 10, 12], South America [9] and Australia [13]. However, to date only two cases of cerebral sparganosis have been reported in Europe [6, 1340 19]. We present the first case of cerebral sparganosis reported in Spain and review the European cases previously published in the literature. Acta Neurochir (2015) 157:1339–1343 cystic cavity inside on the cortical surface. Upon opening the cystic cavity, there was an outflow of clear liquid. A block resection of the solid part was performed. Postoperative course Case report History and examination A 29-year-old immunocompetent male from Bolivia, who had been living in Spain the past 3 years, presented to our service for seizures and a multicystic brain lesion. He had experienced the first seizures 4 years ago in his original country, but had never been examined for this. The month before consulting our service, he had two more seizures consisting of involuntary movements in the upper and lower limbs, which improved after antiepileptic drugs (AED) treatment. He had enjoyed good health, and his medical history was not remarkable. He was unaware of having skin lesions and denied ingestion of raw food, but used to drink water from lakes and rivers. Physical examination and blood tests were unremarkable. The magnetic resonance image (MRI) showed a multicystic intraaxial lesion involving the left temporal lobe, hypointense on T1-weighted images and hyperintense on T2weighted images with ring enhancement after contrast administration (Fig. 1). No ventricular dilatation was evident. Functional MRI did not show significant left expressive and receptive language activation, but erratic contralateral activation was observed. Perfusion-weighted MRI did not show an increase in the relative cerebral blood volume (rCBV). With these findings the first suspected diagnosis was a dysembryoplastic neuroepithelial tumor (DNET), and a craniotomy was proposed as the first line treatment to remove the lesion. Operation A left temporal craniotomy was performed, followed by dural opening, showing a non-infiltrating solid gray area with a Fig. 1 a–b Preoperative axial FLAIR and T2WI MRI showing a cystic cavity with perilesional edema. c Preoperative axial postcontrast MRI with a ring enhancement pattern The final pathology report from the surgical resection revealed gliosis, multiple interconnected cystic cavities with fibrous walls, inflammatory cell infiltration and no necrotizing granulomatous reaction. Inside the cavities, a parasitic form was identified as the larva of the cestode Spirometra mansoni (Figs. 2 and 3). Postoperative MRI showed the expected postoperative changes with total resection of the lesion and no complications. The patient was discharged with a baseline examination and was free of seizures on AED treatment and tapering doses of steroids. On the 30th postoperative day the patient presented with a superficial wound infected by Propionibacterium acnes, which was treated with antibiotics and had a good evolution. At the 1-year follow-up after surgery in the outpatient clinic, the patient had no deficits and was seizure-free. Follow-up MRI showed an area of encephalomalacia involving the temporal lobe with subcortical gliosis without enhancement after contrast administration (Fig. 4). Discussion The first published case of human sparganosis in Europe was a 40-year-old female from Genoa, Italy, in whom the sparganum was located subcutaneously in the right forearm [14, 15]. Since then, very few cases of human sparganosis have been reported in Europe, most of them from Italy and usually affecting subcutaneous tissue [14]. Besides the current case, only two prior cases of cerebral sparganosis have been reported in Europe, one in Switzerland and the other in Germany (Table 1). Diagnosis of cerebral sparganosis is usually one of exclusion and usually relies on Acta Neurochir (2015) 157:1339–1343 1341 Fig. 2 The surgical excision revealed a 2-cm, white, encapsulated multicystic specimen (a, b) with a mural nodule (c) and intracystic clear fluid histopathological verification. The differential diagnosis includes brain tumors and other infectious diseases such as neurocysticercosis and tuberculosis [16, 18]. Although it is difficult to confirm the disease at the preoperative stage, there are some characteristics that may alert physicians to this rare entity. A history of drinking untreated water, eating raw or uncooked fish, frogs or snakes, or using frog flesh as a poultice for an open wound in endemic regions can be identified in the history [8]. However, some patients have no definite risk factors for infection [8]. The two patients with cerebral s p a rg an o s i s r ep or t e d i n E ur op e w e r e f r om A s i a (Bangladesh) and Africa (Malawi), but no information about their epidemiological history was reported. In the current case, the patient was from South America (Bolivia), and a history of drinking water from lakes and rivers in his country was presented. Besides a dependable history, evidence of a previous or active infestation of other organs may also be a useful tool in the diagnosis. Clinical manifestations of cerebral sparganosis are closely associated with the site of the lesion [8]. The most commonly observed symptom is seizures, followed by hemiparesis, headache, body sensory disturbance and alteration in consciousness [4]. One characteristic helpful in the diagnosis of this disease is a possible alteration in the clinical manifestations with the migration of the larva through the brain [4]. Routine laboratory tests Fig. 3 Histological study showed a multicystic lesion (a–b) with a parietal granulomatous reaction (c) containing the larval form of Spirometra mansoni (a). In the parenchyma of the worm we observed loose stroma, calcareous bodies and smooth muscle (d–e). The body wall of the worm consisted of a tegument with microvilli, layers of smooth muscle and tegumental cells (f) sometimes show eosinophilia. Although the cerebrospinal fluid (CSF) in cerebral sparganosis usually has a raised white cell count with lymphocyte predominance and raised protein levels, findings can be variable, and protein and white cell counts have been reported as normal [10]. The immunological diagnostic methods are of limited use because of cross-reactivity with other cestode infestations. However, strong immunopositivity to Spirometra mansoni in both serum and CSF samples may also be a useful tool [8]. Neuroimaging, especially MRI, also plays an important role in the diagnosis and differential diagnosis of cerebral sparganosis. The primary characteristic of this entity on MRI is the tunnel sign seen on post-contrast sequences because of the migration of the live worm with an undulating motion. The second characteristic is the conglomerated ring or bead-shaped enhancement, which represents an inflammatory granuloma. Finally, the alternate change of varying stages in the same image is common because of the long course of the disease. In addition, unilateral ventricular dilation, focal cerebral cortical atrophy, calcification and white matter edema are also characteristic [18]. As in the current case, in our review the two patients presented with multiple or single ring-enhancing lesions, but only one showed the tunnel sign [6]. 1342 Acta Neurochir (2015) 157:1339–1343 Fig. 4 a–b Postoperative axial MRI showing an area of encephalomalacia involving the temporal lobe with subcortical gliosis without enhancement after contrast administration The treatment of choice for cerebral sparganosis is to remove the sparganum from the infested site in the brain. Lei Deng et al. proposed in 2011 that stereotactic surgery provides the most effective approach for this purpose, because this surgery creates the smallest wound and prevents broken larvae [4]. They recommended that the most enhanced feature on the CT scan or MRI should be selected as the targeting point. Aspiration should be performed in multiple directions radiating from the origin, and carefully examination of the integrity of the removed larva should be achieved, paying particular attention to the scolex. In case the larva cannot be taken out after repeated aspirations, a craniotomy should be performed [4]. On the other hand, medication with antiparasitic drugs is generally believed to be ineffective for treating cerebral sparganosis [7]. However, some authors have suggested high-dose praziquantel may be considered in inoperable cases of cerebral sparganosis following a good outcome [6, 8]. Like the case reported in Germany, in the current case the preoperative diagnosis was not cerebral sparganosis. Instead, the authors considered a brain abscess and DNET, respectively, which led to a craniotomy as the first line approach to remove the lesion. Conclusion Although cerebral sparganosis is rarely reported in Europe, it should be suspected in patients from endemic areas with a possible infection history as well as Bwandering lesions^ on the MRI. To prevent the sequelae of the infection, histopathological verification should be attempted early by removing the sparganum from the infested site of the brain, either by stereotactic aspiration or craniotomy. In addition, praziquantel has been proposed as an effective treatment in inoperable cases. Clinical and imaging follow-up should be performed after treatment to ensure that no larva residue remains in the brain. Table 1 Clinical features, radiological changes and treatment of patients with cerebral sparganosis reported in Europe Case no. Ref no. Country of Country of origin diagnosis Age Clinical Epidemiological MRI (years) presentation history 1 6 Bangladesh Switzerland 39 Seizures Not available 2 19 Malawi 3 Current case Bolivia Germany 25 Seizures Not available Spain 29 Seizures Yes DNET: Dysembryoplastic neuroepithelial tumor Initial suspected diagnosis Treatment Multiple ringNeurocysticercosis Only enhancing praziquantel lesions with perifocal edema. Tunnel sign Contrast-enhancing Cerebral abscess Craniotomy ring-shaped lesion in the right temporooccipital lobe Multicystic ringTumor (DNET) Craniotomy enhancing lesion Acta Neurochir (2015) 157:1339–1343 Acknowledgments All authors certify that they have NO affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript. Conflicts of interest None. References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. Anders K, Foley K, Stern E, Brown WJ (1984) Intracranial sparganosis: an uncommon infection. Case report. J Neurosurg 60:1282–1286 Chamadol W, Tangdumrongkul S, Thanaphaisal C, Sithithaworn P, Chamadol N (1992) Intracerebral hematoma caused by sparganum: a case report. J Med Assoc Thail 75:602–605 Cummings TJ, Madden JF, Gray L, Friedman AH (2000) Parasitic lesion of the insula suggesting cerebral sparganosis: case report. Neuroradiology 42:206–208 Deng L, Xiong P, Qian S (2011) Diagnosis and stereotactic aspiration treatment of cerebral sparganosis: summary of 11 cases. J Neurosurg 114:1421–1425 Fan KJ, Pezeshkpour GH (1986) Cerebral sparganosis. Neurology 36:1249–1251 Gonzenbach RR, Kong Y, Beck B, Buck A, Weller M, Semmler A (2013) High-dose praziquantel therapy for cerebral sparganosis. J Neurol 260:1423–1425 Holodniy M, Almenoff J, Loutit J (1991) Cerebral sparganosis: case report and review. Rev Infect Dis 13:155–159 Hong D, Xie H, Zhu M, Wan H, Xu R, Wu Y (2013) Cerebral sparganosis in mainland Chinese patients. J Clin Neurosci 20: 1514–1519 Jones MC, Agosti MR, D'Agustini M, Uriarte V, Drut R (2013) Cerebral sparganosis in a child: case report. Arch Argent Pediatr 111:1–4 Kwon JH, Kim JS (2004) Sparganosis presenting as a conus medullaris lesion—case report and literature review of the spinal sparganosis. Arch Neurol 61:1126–1128 Li MW, Song HQ, Li C, Lin HY, Xie WT, Lin RQ, Zhu XQ (2011) Sparganosis in mainland China. Int J Infect Dis 15:54–56 1343 12. Mitchell A, Scheithauer BW, Kelly PJ, Forbes GS, Rosenblatt JE (1990) Cerebral sparganosis. Case report. J Neurosurg 73:147–150 13. Munckhof WJ, Grayson ML, Susil BJ, Pullar MJ, Turnidge J (1994) Cerebral sparganosis in an East Timorese refugee. Med J Aust 161:263–264 14. Pampiglione S, Fioravanti ML, Rivasi F (2003) Human sparganosis in Italy: case report and review of the European cases. APMIS 111: 349–354 15. Pujatti D (1953) Un caso di sparganosi umana. Riv Parassitol 14: 213–217 16. Rengarajan S, Nanjegowda N, Bhat D, Mahadevan A, Sampath S, Krishna S (2008) Cerebral sparganosis: a diagnostic challenge. Br J Neurosurg 22:784–786 17. Shirakawa K, Yamasaki H, Ito A, Miyajima H (2010) Cerebral sparganosis: the wandering lesion. Neurology 74:180 18. Song T, Wang WS, Zhou BR, Mai WW, Li ZZ, Guo HC, Zhou F (2007) CT and MR characteristics of cerebral sparganosis. AJNR Am J Neuroradiol 28:1700–1705 19. Walker P, Cooper NK, Brandis A (2007) Cerebral sparganosis presenting as grand mal epilepsy. J R Army Med Corps 153:189–190 20. Wong CW, Ho YS (1994) Intraventricular haemorrhage and hydrocephalus caused by intraventricular parasitic granuloma suggesting cerebral sparganosis. Acta Neurochir 129:205–208 Patient Consent The patient consented to the submission of the case report Comments Lo Presti and co-workers describe a case of cerebral sparganosis, a severe parasitic infection mainly seen in Asia and only described twice—now three times—in Europe. Even though the present case is not really a European one—the 29-year-old male was from Bolivia, now living in Spain—the careful documentation of the case along with the aspect of enlarging our repertoire of differential diagnoses warrants its publication. Marcus Reinges Giessen, Germany