Echinococcus Infestation of the Central Nervous System as the Primary and Solitary Manifestation of the Disease: Case Report and Literature Review

Main Article Content

Dimitrios Panagopoulos Maria Gavra Georgios Stranjalis Efstathios Boviatsis Stefanos Korfias Ploutarchos Karydakis Marios Tmemistocleous

Abstract

Introduction: Hydatid disease of the central nervous system, especially when there is no involvement of other organs, is a rare occurrence and it accounts for 2–3% of all the hydatid cyst cases that are recorded worldwide. Echinococcus is considered as an endemic disease, mainly encountered in sheep and cattle-raising regions, with a large proportion of patients living in Mediterranean countries, as well as Turkey and Syria. Pediatric neurosurgeons in nonendemic countries are facing a differential diagnostic challenge when a patient with hydatid cyst of the central nervous system is admitted to their department, mainly due to lack of awareness.


Materials and Methods: We report the case of an eleven years old patient, coming from a village, who was admitted to our hospital due to progressive hemiparesis, hemianopsia, headache and episodes of vomiting. A thorough laboratory and neuroimaging investigation was performed, which revealed the presence of a hydatid cyst at the right fronto-parietal region. An en-block total resection was performed and the patient neurological signs and symptoms were immediately relieved.


Conclusions: The clinical features related to hydatid cyst involving the central nervous system are largely nonspecific. Nevertheless, MRI has proved to be a very important diagnostic tool, significantly enhancing the diagnostic aid of neuroimaging and supporting our pre-operative diagnostic accuracy. Laboratory investigation with immunological tests may be indicative, but negative results should be interpreted with caution. En block removal of the cyst without interruption of its wall and avoidance of any spillage of content is our ultimate therapeutic goal. Infection with Echinococcus granulosus should be included in the differential diagnosis for non-specific neurologic symptoms such as progressive headache, especially in pediatric patients who are coming from regions which are considered as endemic for such parasitic diseases. In addition, the adjuvant therapy with albendazole is strongly indicated. The majority of misdiagnoses or delayed diagnoses may have resulted from confusing clinical features and atypical radiographic findings.

Article Details

How to Cite
PANAGOPOULOS, Dimitrios et al. Echinococcus Infestation of the Central Nervous System as the Primary and Solitary Manifestation of the Disease: Case Report and Literature Review. Medical Research Archives, [S.l.], v. 11, n. 1, jan. 2023. ISSN 2375-1924. Available at: <https://esmed.org/MRA/mra/article/view/3510>. Date accessed: 20 apr. 2024. doi: https://doi.org/10.18103/mra.v11i1.3510.
Section
Case Reports

References

1. Padayachy LC, Dattatraya M. Hydatid disease (Echinococcus) of the central nervous system. Childs Nerv Syst. 2018;34(10):1967-1971. doi: 10.1007/s00381-018-3883-x. Epub 2018 Jun 30.
2. Eckert J, Deplazes P. Biological, epidemiological and clinical aspects of echinococcosis, a zoonosis of increasing concern. Clin Microbiol Rev 2004;(17):107–135.
3. Beggs I. The radiology of hydatid disease. Am J Roentgenol 1985;(145):639–648.
4. Tuzun M, Hekimoglu B. Hydatid disease of the CNS: imaging features. Am J Roentgenol 1998;(171):1497–150.
5. Benzagmout M, Maaroufi M, Chakour K, Chaoui ME. Atypical radiological findings in cerebral hydatid disease. Neurosciences (Riyadh). 2011;16(3):263-6.
6. Arana-Iniguez R. Hydatid echinococcosis of the nervous system. In: Spillane JD, editor. Tropical neurology. London, Oxford (UK): University Press; 1973. p. 408-417.
7. Tlili-Graiess K, El-Ouni F, Gharbi-Jemni H, Arifa N, Moulahi H, Mrad-Dali K, Guesmi H, Abroug S, Yacoub M, Krifa H. [Cerebral hydatid disease: imaging features]. J Neuroradiol 2006; (33): 304-318. French
8. Furtado S.V, Visvanathan K, Nandita G, Reddy K, Hegde A.S. Multiple fourth ventricular hydatidosis. J Clin Neurosci 2009;16(1):110-2. doi: 10.1016/j.jocn.2007.09.031.
9. Taslakian B, Darwish H. Intracranial hydatid cyst: imaging findings of a rare disease. BMJ Case Rep. 2016;(12);2016: bcr2016216570. doi: 10.1136/bcr-2016-216570.
10. Kitis O, Calli C, Yunten N. Report of diffusion-weighted MRI in two cases with different cerebral hydatid disease. Acta Radiol 2004;45(1):85-7. doi: 10.1080/02841850410000737.
11. Senturk S, Oguz K.K, Soylemezoglu F, Inci S. Cerebral alveolar echinoccosis mimicking primary brain tumor. AJNR Am J Neuroradiol 2006;(27) (2):420-2.
12. Tüzün M, Altinörs N, Arda IS, Hekimoğlu B. Cerebral hydatid disease CT and MR findings. Clin Imaging. 2002;26(5):353-7. doi: 10.1016/s0899-7071(02)00449-7.
13. Yaka U, Aras Y, Aydoseli A, Osman M, Sencer A.A, Imer M, Hepgul K. Primary multiple cerebral hydatid disease: still symptomatic despite pathologically confirmed death of the cyst. Turk Neurosurg 2013;23(4):505-8. doi: 10.5137/1019-5149.JTN.5826-12.1.
14. Khalatbari M.R, Brunetti E, Shobeiri E, Moharamzad Y. Calcified Mass on Brain CT in a Teenager with Refractory Seizures. Neuroradiol J 2014;(27) (6):691-6. doi: 10.15274/NRJ-2014-10101. Epub 2014 Dec 1.
15. Luo K, Luo DH, Zhang TR, Wen H. Primary intracranial and spinal hydatidosis: a retrospective study of 21 cases. Pathog Glob Health. 2013;(107) (2):47-51. doi: 10.1179/2047773213Y.0000000072.
16. Ersahin Y, Mutluer S, Guzelbag E. Intracranial hydatid cysts in children. Neurosurgery 1993; (33):219–224
17. Al Zain T.J, Al-Witry S.H, Khalili H.M, Aboud S.H, Al Zain Jr F.T. Multiple intracranial hydatosis. Acta Neurochir 2002;(144):1179–1185
18. Khaldi M, Mohamed S, Kallell J, Khouja N. Brain hydatosis: report on 117 cases. Childs Nerv Syst 2000;(16):765–769.
19. Guzel A, Tatli M, Maciaczyk J, Altinors N. Primary cerebral intraventricular hydatid cyst: a case report and review of the literature. J Child Neurol. 2008;23(5):585-8 doi: 10.1177/0883073807309791.
20. Du G, Li Y, Wu P, Wang X, Su R, Fan Y, Geng D. Diagnosis, treatment, and misdiagnosis analysis of 28 cases of central nervous system echinococcosis. Chin Neurosurg J 2021;(21);7(1):30. doi: 10.1186/s41016-021-00248-y.
21. Altinors N, Bavbek M, Caner H, Erdogan B. Central nervous system hydatidosis in Turkey: a cooperative study and literature survey analysis of 458 cases. J Neurosurg. 2000;93(1):1–8. https://doi.org/10.3171/jns.2000.93.1.0001.
22. Lunardi P, Missori P, Di Lorenzo N, Fortuna A. Cerebral hydatidosis in childhood: a retrospective survey with emphasis on long-term follow-up. Neurosurgery 1991;29(4):515–8 https://doi.org/10.1227/00006123-19911 0000-00005.
23. Nurchi G, Floris F, Montaldo C, Mastio F, Peltz T, Coraddu M. Multiple cerebral hydatid disease: case report with magnetic resonance imaging study. Neurosurgery 1992;30(3):436–8. https://doi.org/10.1227/00006123-1 99203000-00023.
24. Khaldi M, Mohamed S, Kallel J, Khouja N. Brain hydatidosis: report on 117 cases. Child’s Nervous System. 2000;16(10-11):765–9. https://doi.org/10.1007/ s003810000348.
25. Akdemir G, Dağlıoğlu E, Seçer M, Ergüngör F. Hydatid cysts of the internal acoustic canal and jugular foramen. J Clin Neuroscience. 2007;(14):394–6.
26. Ersahin Y, Mutluer S, Dermirtas E. Taşkin Yurtseven: A case of thalamic hydatid cyst. Clin Neurol Neurosurg. 1995;97(4):321–3. https://doi.org/10.101 6/0303-8467(95)00052-L.
27. Ciurea AV, Fountas KN, Coman TC, Machinis TG, Kapsalaki EZ, Fezoulidis NI, Robinson JS. Long-term surgical outcome in patients with intracranial hydatid cyst. Acta Neurochir (Wien). 2006;148(4):421 https://doi.org/10.1007/s00701-005-0679-z.
28. Onal C, Erguvan-Onal R, Yakinci C, Karayol A, Atambay M, Daldal N. Can the requirement of a diversion procedure be predicted after an uncomplicated intracranial hydatid cyst surgery? Pediatr Neurosurg. 2006;42(6):383–6 https://doi.org/10.1159/000095570.
29. Bükte Y, Kemaloglu S, Nazaroglu H, Ozkan U, Ceviz A, Simsek M. Cerebral hydatid disease: CT and MR imaging findings. Swiss Med Wkly. 2004;134(31-32):459–67 DOI: 2004/31/smw-10711.
30. El-Shamam O, Amer T, El-Atta MA. Magnetic resonance imaging of simple and infected hydatid cysts of the brain. Magn Reson Imaging 2001;(19): 965-974.
31. Razek AA, El-Shamam O, Wahab A.N. Magnetic resonance appearance of cerebral cystic echinococcosis: World Health Organization (WHO) classification. Acta Radiol 2009; (50): 549-554.