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Published on: September 25, 2009
Neuronal intranuclear inclusion disease complicated by acute middle cerebral artery infarction: A case report
Xueming Li1, Weijiang Ding2, Tao Jiang3
1Department of General Medicine, The Second Affiliated Hospital of Nanchang University, No 1 Minde Road, Donghu District, Nanchang 330006, Jiangxi Province, China.
Background:
Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disorder with no prior reports linking it to acute large-vessel cerebral infarction.
Methods:
A 65-year-old man with progressive limb numbness and acute neuropsychiatric symptoms underwent MRI, skin biopsy, and genetic testing.
Results:
MRI revealed corticomedullary "ribbon signs" and right middle cerebral artery (MCA) stenosis. Follow - up imaging not only confirmed the occlusion of the MCA accompanied by an infarction in the basal ganglia but also indicated an enlargement of the "ribbon signs". Skin biopsy showed p62-positive intranuclear inclusions; genetic testing identified a NOTCH2NLC GGC expansion (>41 repeats). Despite treatment, the patient died of pneumonia three months post-infarction.
Conclusions:
This single-case, first-reported observational study of NIID with large-vessel stenosis illustrates a potential vasculopathic link. While robust histopathological validation is pending, the temporal correlation between NIID progression and vascular events warrants further investigation.
