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

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.