MR Imaging Features of Adult-Onset Neuronal Intranuclear Inclusion Disease May Be Indistinguishable from Fragile X–Associated Tremor/Ataxia Syndrome

I.G Padilha,R.H Nunes,J.L Pedroso,F.A Scortegagna,O.G.P Barsottini,M.R Rodrigues Gonçalves,A.J Da Rocha,V.H Marussi

doi:10.3174/ajnr.a5729

Abstract

We have read with great interest the article by Sugiyama et al[1][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

Highlights

Pathologic changes are similar because fragile X–associated tremor/ataxia syndrome (FXTAS) and neuronal intranuclear inclusion disease (NIID) usually present with intranuclear eosinophilic inclusions
When we reviewed our institutional records, 3 recent patients were clinically evaluated and genetic studies confirmed FXTAS
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

Summary

Introduction

MR Imaging Features of Adult-Onset Neuronal Intranuclear Inclusion Disease May Be Indistinguishable from Fragile X–Associated Tremor/Ataxia Syndrome We 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).

Results

Conclusion