Abstract
Neuronal intranuclear inclusion disease is a neurodegenerative disorder pathologically characterized by eosinophilic hyaline intranuclear inclusions. A high-intensity signal along the corticomedullary junction on DWI has been described as a specific MR imaging finding of the cerebrum in neuronal intranuclear inclusion disease. However, MR imaging findings of the cerebellum in neuronal intranuclear inclusion disease have not been fully evaluated. Here, we review MR imaging findings of the cerebellum in a series of 8 patients with pathologically confirmed neuronal intranuclear inclusion disease. The MR imaging results showed cerebellar atrophy (8/8 patients) and high-intensity signal on FLAIR images in the medial part of the cerebellar hemisphere right beside the vermis (the "paravermal area") (6/8) and in the middle cerebellar peduncle (4/8). The paravermal abnormal signals had a characteristic distribution, and they could be an indicator of the diagnosis of neuronal intranuclear inclusion disease even when using the results of past MR imaging examinations in which DWI findings were not examined.
Highlights
We reviewed the medical records of the 8 patients including their medical history and neurologic examination findings, Mini-Mental State Examination (MMSE), Frontal Assessment Battery (FAB), and CSF results
We evaluated the following findings: 1) high-intensity signal along the corticomedullary junction on DWI, 2) diffuse high-intensity signal of cerebral white matter on FLAIR images, 3) atrophy of the cerebellum, 4) high-intensity signal in the medial part of the cerebellar hemisphere right beside the vermis on FLAIR images (Fig 1), and
The relationship between the extent of the high-intensity signal of the cerebral white matter and DWI high- lesion and the severity of ataxia could not be assessed, but all 3 patients with ataxia presented with an abnormal signal in the paravermal area
Summary
We have presented the MR imaging features of the cerebellum in 8 patients with pathologically confirmed adult-onset NIID.
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