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 17-year-old female patient presented with neck pain since two weeks which was aggravated by exercise and neck movements. She developed weakness of limbs for one week which started in left upper limb and then evolved to involve all four limbs. She also complained of numbness in both upper limbs and urgency of micturation. On neurological examination, tone was mildly decreased in both upper limbs and increased in both lower limbs. Power was 2/5 in left upper limbs and 4/5 in other limbs. Deep tendon reflexes were absent in upper limbs and exaggerated in lower limbs. Pain and touch sensation decreased by 30% below C2 level and vibration sense lost below T2 level.\nMRI [] showed a well circumscribed extra-axial cystic lesion of 3 × 1.8 × 2.4 cm size noted anterior and to the right of medulla extending through foramen magnum into upper cervical canal. It was hyperintense on T1 and T2 weighted images with no diffusion restriction. On right side hypoglossal nerve was displaced laterally by lesion.\nShe underwent suboccipital craniectomy and total excision of the lesion via right lateral approach. Histopathology showed a cyst wall is lined by cuboidal and columnar epithelium with goblet cells (arrow) suggestive of a neuroenteric cyst.\nPostoperatively, patient's power improved to 4/5 in all 4 limbs.