Neuronal intranuclear inclusion disease (NIID): investigations
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Neuronal intranuclear inclusion disease (NIID): investigations. Leukoencephalopathy, Cerebral atrophy. Fully referenced neurology checklist.
Magnetic resonance imaging (MRI) brain: features
- Leukoencephalopathy
- Cerebral atrophy
- Cerebellar atrophy
- Paravermal medial cerebellar hemisphere high signal
- Middle cerebellar peduncle high signal
- Cortico-medullary junction high signal
- Focal cortical lesions: localised cortical oedema or atrophy
Neurophysiological tests
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Tissue biopsy
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Treatment
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References
- Nakamura M, Ueki S, Kubo M, et al. Two cases of sporadic adult-onset neuronal intranuclear inclusion disease preceded by urinary disturbance for many years. J Neurol Sci 2018; 392:89-93.
- Sugiyama A, Sato N, Kimura Y, et al. MR imaging features of the cerebellum in adult-onset neuronal intranuclear inclusion disease: 8 cases. Am J Neuroradiol 2017; 38:2100-2104.
- Han X, Han M, Liu N, et al. Adult-onset neuronal intranuclear inclusion disease presenting with typical MRI changes. Brain Behav 2019; 9:e01477.
- Xiao F, Tian X, Wang XF. Cerebral atrophy and leukoencephalopathy in a young man presenting with encephalitic episodes. JAMA Neurol 2018; 75:1563-1564.
- Sone J, Mori K, Inagaki T, et al. Clinicopathological features of adult-onset neuronal intranuclear inclusion disease. Brain 2016; 139:3170-3186.
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