Neuronal intranuclear inclusion disease (NIID): clinical features
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Neuronal intranuclear inclusion disease (NIID): clinical features. Adult onset: dementia dominant. Fully referenced neurology checklist.
Onset types
- Adult onset: dementia dominant
- Juvenile onset: leg weakness dominant
- Childhood-onset
Clinical patterns
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Encephalopathic features
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Neuromuscular features
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Movement disorders
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Autonomic features
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Other features
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Differential diagnosis
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Acronym
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References
- Sone J, Mori K, Inagaki T, et al. Clinicopathological features of adult-onset neuronal intranuclear inclusion disease. Brain 2016; 139:3170-3186.
- Tai H, Wang A, Zhang Yet al. Clinical features and classification of neuronal intranuclear inclusion disease. Neurol Genet 2023; 9:e200057.
- Furuta M, Sato M, Kasahara H, et al. Clinical, radiological, and molecular analyses of neuronal intranuclear inclusion disease with polyglycine inclusions. J Neurol Sci 2023; 448:120618.
- Wiltshire KM, Dunham C, Reid S, Auer RN, Suchowersky O. Neuronal intranuclear inclusion disease presenting as juvenile parkinsonism. Can J Neurol Sci 2010; 37:213-218.
- Deng J, Gu M, Miao Y, et al. Long-read sequencing identified repeat expansions in the 5'UTR of the NOTCH2NLC gene from Chinese patients with neuronal intranuclear inclusion disease. J Med Genet 2019; 56:758-764.
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