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. 2020 Sep 30;11:586606. doi: 10.3389/fneur.2020.586606

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Copyright © 2020 Elble.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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This flow diagram illustrates how Axis 1 and 2 classifications may change over time. This clinical scenario is based on the work of Chen et al. (40). A 54-year-old Chinese man presented with a 5-year history of tremor in the head and upper limbs. His family history was consistent with autosomal dominant inheritance. His initial Axis 1 classification was ET, and his Axis 2 classification was idiopathic familial. Over time, his Axis 1 classification changed from ET to ET plus mild cognitive impairment, and his Axis 1 classification ultimately changed to a combined tremor-dementia syndrome with antecedent ET. His MRI brain revealed diffusion-weighted abnormality in the subcortical U-fibers of both frontal lobes, and genetic testing revealed a GGC repeat expansion in NOTCH2NLC. Thus, his Axis 2 diagnosis was ultimately neuronal intranuclear inclusion disease that presented initially as ET.