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March 30, 2021; 96 (13) Resident & Fellow Section

Teaching Video NeuroImages: A Triad of Tremor, Ataxia, and Cognitive Impairment

Lisa W.C. Au, View ORCID ProfileKo Ho, Joshua J.X. Li, Vincent C.T. Mok
First published December 4, 2020, DOI: https://doi.org/10.1212/WNL.0000000000011288
Lisa W.C. Au
From the Departments of Medicine and Therapeutics (L.W.C.A., K.H., V.C.T.M.) and Anatomical and Cellular Pathology (J.J.X.L.), The Chinese University of Hong Kong, Prince of Wales Hospital; and Gerald Choa Neuroscience Centre, Margaret K.L. Cheung Research Centre for Management of Parkinsonism (L.W.C.A., K.H., V.C.T.M.), Therese Pei Fong Chow Research Centre for Prevention of Dementia, Hong Kong.
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Ko Ho
From the Departments of Medicine and Therapeutics (L.W.C.A., K.H., V.C.T.M.) and Anatomical and Cellular Pathology (J.J.X.L.), The Chinese University of Hong Kong, Prince of Wales Hospital; and Gerald Choa Neuroscience Centre, Margaret K.L. Cheung Research Centre for Management of Parkinsonism (L.W.C.A., K.H., V.C.T.M.), Therese Pei Fong Chow Research Centre for Prevention of Dementia, Hong Kong.
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  • ORCID record for Ko Ho
Joshua J.X. Li
From the Departments of Medicine and Therapeutics (L.W.C.A., K.H., V.C.T.M.) and Anatomical and Cellular Pathology (J.J.X.L.), The Chinese University of Hong Kong, Prince of Wales Hospital; and Gerald Choa Neuroscience Centre, Margaret K.L. Cheung Research Centre for Management of Parkinsonism (L.W.C.A., K.H., V.C.T.M.), Therese Pei Fong Chow Research Centre for Prevention of Dementia, Hong Kong.
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Vincent C.T. Mok
From the Departments of Medicine and Therapeutics (L.W.C.A., K.H., V.C.T.M.) and Anatomical and Cellular Pathology (J.J.X.L.), The Chinese University of Hong Kong, Prince of Wales Hospital; and Gerald Choa Neuroscience Centre, Margaret K.L. Cheung Research Centre for Management of Parkinsonism (L.W.C.A., K.H., V.C.T.M.), Therese Pei Fong Chow Research Centre for Prevention of Dementia, Hong Kong.
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Citation
Teaching Video NeuroImages: A Triad of Tremor, Ataxia, and Cognitive Impairment
Lisa W.C. Au, Ko Ho, Joshua J.X. Li, Vincent C.T. Mok
Neurology Mar 2021, 96 (13) e1802-e1803; DOI: 10.1212/WNL.0000000000011288

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A 67-year-old woman presented with a 2-year history of forgetfulness and unsteadiness. She scored 17 on the Montreal Cognitive Assessment. She had tremor of head and upper limbs (video 1) since early adulthood, which was diagnosed as essential tremor. Brain MRI showed leukoencephalopathy and high signal intensity along the corticomedullary junction on diffusion-weighted images (figure 1). The diagnosis of adult-onset neuronal intranuclear inclusion disease (NIID) was confirmed by skin biopsy showing eosinophilic intranuclear inclusions (figure 2).1 NIID is a clinically heterogeneous rare neurodegenerative disease.2 Its characteristic MRI pattern should prompt confirmation of the diagnosis by skin biopsy.

Video 1

Video recorded at age 72 years. The patient showed tremor of head and upper limbs that were present at rest and worsened on posture and movement. She failed tandem walking. Download Supplementary Video 1 via http://dx.doi.org/10.1212/011288_Video_1

Figure 1
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Figure 1 MRI Brain

Axial MRI brain performed at age 72 years showed bilateral subcortical white matter lesions (arrows) on axial fluid-attenuated inversion recovery images (A–C) and high signal intensity along the corticomedullary junction (arrows) on diffusion-weighted images (D–F).

Figure 2
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Figure 2 Skin Biopsy

Hematoxylin & eosin stain of fibroblasts (A) and ubiquitin immunostain of sweat gland cells (B) showing intranuclear inclusions (arrows); the inclusions consisted of electron-dense filamentous materials without membrane structure on electron microscopy (C).

Study Funding

No targeted funding reported.

Disclosure

The authors report no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.

Appendix Authors

Table

Footnotes

  • Go to Neurology.org/N for full disclosures.

  • Teaching slides links.lww.com/WNL/B293

  • © 2020 American Academy of Neurology

References

  1. 1.↵
    1. Sone J,
    2. Tanaka F,
    3. Koike H, et al
    . Skin biopsy is useful for the antemortem diagnosis of neuronal intranuclear inclusion disease. Neurology 2011;76:1372–1376.
    OpenUrlAbstract/FREE Full Text
  2. 2.↵
    1. Sone J,
    2. Mori K,
    3. Inagaki T, et al
    . Clinicopathological features of adult-onset neuronal intranuclear inclusion disease. Brain 2016;139:3170–3186.
    OpenUrlCrossRefPubMed

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