To highlight the presence of various neurological symptoms in patients presumed to have multinodular and vacuolating neuronal tumor (MVNT).

MVNT is a recently recognized distinct entity in the 2016 World Health Organization Tumors of the Central Nervous System. Characteristic imaging features include multiple, nodular (1-5 mm) T2/FLAIR hyperintensities clustering in the cortex or juxtacortex without mass effect or diffusion restriction, and rare contrast enhancement. Available literature suggests dysplastic/ malformative etiology than neoplastic. Despite our radiographic and histologic understanding of the condition, there has been a paucity of data on clinical syndromes associated with MVNT.

We describe 3 cases of patients who presented with debilitating neurologic complaints and subsequently found to have MVNT on imaging. Patient 1 presented with severe headache and persistent left hemi-paresthesias. Patient 2 presented with intermittent vertigo, right hemi-paresthesias and whole-body tremulousness. Patient 3 presented with severe headache and dizziness. Their symptoms did not fully localize to the MVNT lesions, and the objective neurologic exam was non-focal for all three patients. A thorough neurological workup including video electroencephalogram, cerebrospinal fluid analysis, neoplastic, and paraneoplastic studies were unremarkable.

Not applicable

Apart from seizures, reports on the clinical syndromes associated with MVNT are rare. The majority of MVNT cases previously described were discovered as part of workup for nonspecific symptoms. Although MVNT may be a radiographically isolated condition, it is feasible that the clinical features may be part of a yet-to-be-described MVNT syndrome. One possible mechanism could be an immune-mediated response from antibodies produced by the dysplastic cells. Future studies that systematically examine the clinical, imaging and pathologic correlations would help advance our understanding of such new entity.