To review imaging and management in a rare case of multifocal intracranial biopsy-confirmed neurenteric cysts.

Neurenteric cysts are a rare developmental abnormality derived from endoderm, often benign, and typically appear in or around the spinal cord. Intracranial neurenteric cysts are relatively uncommon, the majority of which occur in the posterior fossa; supratentorial neurenteric cysts are even rarer.

Case report

We present the case of a 42-year-old female born in Mexico with intraparenchymal cystic disease of unclear etiology since infancy. Patient needed ventriculoperitoneal shunt (VPS) placement in her first year of life with multiple subsequent revisions. Since moving to the US in the early 2000s, she has been seen numerous times by neurology, neurosurgery, and infectious disease, including parasitology and neurocysticercosis (NCC) specialists for workup of her multifocal cystic lesions. Patient was initially diagnosed with NCC, but multiple rounds of empiric anti-helminthic therapy did not help and antibody testing was repeatedly negative.  Due to high concern for an infectious process neurosurgery was reticent to perform biopsy.

She ultimately presented to our hospital with acute on chronic worsening headaches, dizziness, gait instability, and diplopia. High resolution MRI brain with contrast redemonstrated multifocal supra- and infra-tentorial extra-axial cystic lesions. Taken together with her negative NCC workup, poor treatment response, and remote history of histology showing epithelial cells, the patient was taken for cyst fenestration. Histology was positive for MCK and EMA, suggestive of neurenteric cysts. Patient’s symptoms nearly completely resolved after fenestration.

An interesting topic of discussion in this case is the multifocality of the cysts, which has rarely been documented in the literature. A hypothesis to consider is that during her numerous neurosurgical procedures early in life, there may have been seeding of neurenteric cyst cells to several parts of her brain. This was proven via histopathology of her cysts.