Multinodular and vacuolating neuronal tumor of the cerebrum is a recently reported benign, mixed glial neuronal lesion that is included in the 2016 updated World Health Organization classification of brain neoplasms as a unique cytoarchitectural pattern of gangliocytoma. We report 33 cases of presumed multinodular and vacuolating neuronal tumor of the cerebrum that exhibit a remarkably similar pattern of imaging findings consisting of a subcortical cluster of nodular lesions located on the inner surface of an otherwise normal-appearing cortex, principally within the deep cortical ribbon and superficial subcortical white matter, which is hyperintense on FLAIR. Only 4 of our cases are biopsy-proven because most were asymptomatic and incidentally discovered. The remaining were followed for a minimum of 24 months (mean, 3 years) without interval change. We demonstrate that these are benign, nonaggressive lesions that do not require biopsy in asymptomatic patients and behave more like a malformative process than a true neoplasm.

Discovery of genetic abnormalities associated with neurodegeneration with brain iron accumulation (NBIA) has led to use of a genetic-based NBIA classification schema. Most NBIA subtypes demonstrate characteristic imaging abnormalities. While clinical diagnosis of NBIA is difficult, analysis of both clinical findings and characteristic imaging abnormalities allows accurate diagnosis of most of the NBIA subtypes. This article reviews recent updates in the genetic, clinical, and imaging findings of NBIA subtypes and provides a practical step-by-step clinicoradiological algorithm toward clinical diagnosis of different NBIA subtypes.

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W e have read with great interest the article by Sugiyama et al 1 published in the November 2017 edition of the American Journal of Neuroradiology, which described typical brain MR imaging findings in adult-onset neuronal intranuclear inclusion disease (NIID). The authors highlighted the paravermal signal changes on FLAIR sequences as well as the high-intensity signal on DWI along the corticomedullary junction as typical neuroimaging findings in NIID.According to Sone et al, 2 who described clinical features, MR imaging findings, and pathologic features in a larger series of 57 patients with adult-onset NIID, the pathophysiology of fragile X-associated tremor/ataxia syndrome (FXTAS) and NIID overlaps, and it is not reliable for distinguishing both disorders based on either clinical presentation, imaging findings, or family history. Moreover, pathologic changes are also similar because FXTAS and NIID usually present with intranuclear eosinophilic inclusions. The study of Sugiyama et al 1 did not evaluate the CGG repeat length of the FMR1 gene in their patients, which is a crucial step to support their conclusions. When we reviewed our institutional records, 3 recent patients were clinically evaluated and genetic studies confirmed FXTAS. Most interesting, our 3 patients with FXTAS presented with imaging findings (Figs 1 and 2) very similar to the those in patients described by Sugiyama et al, whose diagnoses were NIID.Research in neuroradiology must concentrate on predicting specific neurologic disorders, identifying either radiophenotypes and/or useful algorithms for clinical practice. Considering that FXTAS seems to be more common than adult-onset NIID, it is reasonable that the algorithm using genetic studies (FMR1 gene premutation) proposed by Sone et al 2 remains unpredictable because the pathologic changes and the imaging findings reported by Sugiyama et al 1 may occur in both disorders.

Formerly called dysembryoplastic neuroepithelial tumour (DNET) of the septum pellucidum, myxoid glioneuronal tumour (MGT) was recently recognized as a distinct entity. We report three cases of presumed MGT with typical location and image features.