Intramedullary neurenteric cyst without spinal dysraphism

Clinical History

A 49-year-old female patient with no comorbidities presented with bilateral lower limb weakness for the past 15 years. The patient underwent surgery.

Imaging Findings

An intramedullary lobulated lesion was found in the dorsal cord, which was high on T2W (Fig. 1), iso to low on T1W (Fig. 3), showing no post contrast enhancement (Fig. 2, 4a). Additional sequence DWI (Fig. 4b) showed no diffusion restriction.

Discussion

Discussion: Intramedullary neurenteric cyst without spinal dysraphism is a rare entity. Particularly the intramedullary location is seen in 10-15% of cases. It belongs to the subgroup of split notochord syndrome spectrum and there is an abnormal connection between primitive endoderm and ectoderm during the 3rd embryonic week with incomplete separation of the notochord layer from the endoderm. [1]
Clinical Perspective: Patients present with motor weakness with or without sensory symptoms [2]. Imaging is needed to confirm the diagnosis since it is a benign condition.
Imaging perspective: Intramedullary lesion, which has a dumbbell and CSF signal on all sequences without contrast enhancement is the key diagnostic feature.
MRI with contrast is the modality of choice.
Outcome: Treatment of choice is surgery with marsuplization of the cyst.
Take home message, Teaching points: Not all intramedullary lesions of the cord are neoplastic.

Differential Diagnosis List

Final Diagnosis

Intramedullary neurenteric cyst without spinal dysraphism, confirmed on biopsy.

URL:	https://www.eurorad.org/case/12439
DOI:	10.1594/EURORAD/CASE.12439
ISSN:	1563-4086