Isolated intracerebral cystic echinococcosis. Unusual presentation of a rare disease

Abstract

A 46-year-old woman was admitted with a first generalised tonic-clonic seizure. Past medical history was unremarkable. She reported no travels abroad within the past five years. Brain MRI demonstrated disseminated cerebral and cerebellar focal lesions. Cerebrospinal fluid analysis showed a mild eosinophilic pleocytosis. Extensive imaging investigations and serological tests revealed no hints for a systemic parasitic disease. A stereotactic brain biopsy detected no pathogenic agent. 10 months following the initial admission, brain MRI showed multiple cystic lesions with ring-like enhancement following Gd-DTPA administration. At that time serological tests were positive for an infection with Echinococcus granulosus. The diagnosis was based on positive sequential serological tests and the typical MRI findings in conjunction with an eosinophilic cerebrospinal fluid pleocytosis. An antihelmintic therapy with Albendazole was initiated. Following two-months of oral Albendazole administration, brain MRI revealed a clear reduction in size and number of the hydatid lesions. The patient was free of complaints and the clinical examination was unremarkable. Isolated intracerebral manifestation of cystic echinococcosis is very rare. Compared to the typical presentation with a solitary cerebral hydatid cyst, the dissemination of hydatid cysts is quite uncommon in cystic echinococcosis. Diagnosis should be based on the typical MRI findings and serological tests. The present case demonstrates that the later may be negative over a long period within the clinical course of the disease offering a real challenge to the clinician.