Case Report: Neuronal intranuclear inclusion disease mimicking recurrent stroke in the setting of intracranial stenosis - Takeaways - MDSpire

Case Report: Neuronal intranuclear inclusion disease mimicking recurrent stroke in the setting of intracranial stenosis

  • By

  • Jingmin Zhao

  • Guangxun Shen

  • Lumei Chi

  • May 20, 2026

  • 0 min

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  • 1

    Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disorder that can mimic recurrent stroke-like episodes.

  • 2

    A 64-year-old woman with recurrent neurological deficits was diagnosed with NIID after a skin biopsy revealed characteristic intranuclear inclusions.

  • 3

    The 'ribbon sign' on diffusion-weighted imaging is a key neuroimaging feature that suggests NIID, even in the presence of intracranial stenosis.

  • 4

    Genetic testing for NIID can be negative, necessitating tissue biopsy for definitive diagnosis when imaging is suggestive.

  • 5

    Management of NIID differs from stroke; antiplatelet therapy is not recommended, as episodes are linked to cerebral amyloid angiopathy.

Original Source(s)

Case Report: Neuronal intranuclear inclusion disease mimicking recurrent stroke in the setting of intracranial stenosis

  • by Jingmin Zhao, Guangxun Shen, Lumei Chi

  • May 20, 2026

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