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

Abstract

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.