32-year-old lady presented with complaints of oligomenorrhoea since 9 years; tremor of both hands since 9 months; intermittent right frontal headache, drop attacks, urinary incontinence, somnolence and irrelevant laughing since 6 months. On examination, there was bilateral secondary optic atrophy. No other cranial nerve involvement. Power was grade 3/5 in both upper limbs and grade 4+/5 in both lower limbs. Deep tendon reflexes were brisk. Bilateral cerebellar signs were present.\nMRI [] showed a suprasellar lesion elevating the floor of the fourth ventricle and extending into the interpeduncular fossa posteriorly. It appeared iso- to mildly hyperintense on T1 weighted images and hyperintense on T2 weighted images. There was facilitated diffusion in the lesion with high ADC values. There was no blooming on gradient echo images. There was no obvious enhancement on administration of contrast. On MR spectroscopy, there was a prominent peak at 2.02 ppm corresponding to NAA like compound. The third and lateral ventricles were dilated with periventricular hyperintensities suggestive of transependymal seepage of CSF.\nThe patient underwent right frontotemporal craniotomy, single peice orbitozygomatic osteotomy and subtotal excision of the lesion. Histopathology showed ciliated columnar, pseudostratified columnar, stratified squamous, mucosal glands, clusters of Rosenthal fibres suggestive of neuroepithelial cyst. Based on the immunohistochemical properties, a final diagnosis of neuroenteric cyst was made.
A 7 month old girl presented with complaints of poor feeding, excessive crying and downgaze preference since 1 month. MRI [] showed a CSF-intensity cystic lesion of 7 × 5.7 × 5.8 cm size in left side of posterior fossa compressing the brainstem and cerebellar hemisphere to right with obstructive hydrocephalus and periventricular hyperintensities. The child underwent midline suboccipital craniotomy and decompression of cyst. On histopathology, the cyst wall was lined by pseudostratified ciliated cuboidal to flattened epithelium (black arrow) with a subepithelium composed of fibrocollagenous tissue with dilated vascular channels. On microscopic examination, the findings were suggestive of a neuroepithelial cyst, but based on the immunohistochemical properties, a final diagnosis of neuroenteric cyst was made.