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May 07, 2019; 92 (19) Resident & Fellow Section

Teaching NeuroImages: The zigzag edging sign of adult-onset neuronal intranuclear inclusion disease

Lizhang Chen, Anshan Chen, Song Lei, Li He, Muke Zhou
First published May 6, 2019, DOI: https://doi.org/10.1212/WNL.0000000000007464
Lizhang Chen
From the Departments of Neurology (L.C., L.H., M.Z.) and Pathology (S.L.), West China Hospital, Sichuan University, Chengdu; and Department of Neurology (A.C.), Dazhu County People's Hospital, Sichuan, China.
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Anshan Chen
From the Departments of Neurology (L.C., L.H., M.Z.) and Pathology (S.L.), West China Hospital, Sichuan University, Chengdu; and Department of Neurology (A.C.), Dazhu County People's Hospital, Sichuan, China.
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Song Lei
From the Departments of Neurology (L.C., L.H., M.Z.) and Pathology (S.L.), West China Hospital, Sichuan University, Chengdu; and Department of Neurology (A.C.), Dazhu County People's Hospital, Sichuan, China.
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Li He
From the Departments of Neurology (L.C., L.H., M.Z.) and Pathology (S.L.), West China Hospital, Sichuan University, Chengdu; and Department of Neurology (A.C.), Dazhu County People's Hospital, Sichuan, China.
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Muke Zhou
From the Departments of Neurology (L.C., L.H., M.Z.) and Pathology (S.L.), West China Hospital, Sichuan University, Chengdu; and Department of Neurology (A.C.), Dazhu County People's Hospital, Sichuan, China.
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Teaching NeuroImages: The zigzag edging sign of adult-onset neuronal intranuclear inclusion disease
Lizhang Chen, Anshan Chen, Song Lei, Li He, Muke Zhou
Neurology May 2019, 92 (19) e2295-e2296; DOI: 10.1212/WNL.0000000000007464

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This article has a correction. Please see:

  • Teaching NeuroImages: The zigzag edging sign of adult-onset neuronal intranuclear inclusion disease - August 27, 2019
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A 66-year-old woman presented with dementia, urinary incontinence, and episodic coma for 3 years without remarkable family history. Brain MRI showed leukoencephalopathy and a high signal intensity along the corticomedullary junction on diffusion-weighted imaging (DWI) (zigzag edging sign) (figure 1). Skin biopsy revealed intranuclear inclusion bodies in sweat glands and perivascular cells (figure 2). FMRI CGG permutation was not present. Finally, the diagnosis of neuronal intranuclear inclusion disease (NIID) was confirmed.

Figure 1
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Figure 1 Brain MRI

Brain MRI shows bilateral cerebral white matter lesions, hypointensity on T1 (A), hyperintensity on the T2 (B) and fluid-attenuated inversion recovery (C), and high signal intensity along the corticomedullary junction on diffusion-weighted imaging (D).

Figure 2
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Figure 2 Skin biopsy

Electron microscope shows intranuclear inclusion bodies in sweat gland (A, B) and perivascular cells (C, D) (arrows).

Adult-onset NIID is a clinically heterogeneous neurodegenerative disorder. The zigzag edging sign on DWI is a strong diagnostic clue.1 A definite diagnosis requires skin biopsy revealing intranuclear inclusions and genetic evaluation ruling out fragile X syndrome.1,2

Study funding

No targeted funding reported.

Disclosure

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

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Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Teaching slides links.lww.com/WNL/A876

  • © 2019 American Academy of Neurology

References

  1. 1.↵
    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
  2. 2.↵
    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

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