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Neuronal intranuclear inclusion disease manifesting with new‐onset epilepsy in the elderly

We report the case of a 72‐year‐old woman with a first epileptic seizure at the age of 70 years. Neuronal intranuclear inclusion disease was subsequently diagnosed based on characteristic magnetic resonance imaging findings and intranuclear inclusions on skin biopsy histology. Neuronal intranuclear inclusion disease is a rare neurodegenerative disease with a diverse range of clinical manifestations, including epilepsy. It is categorized into three categories: infantile, juvenile and adult forms. Epileptic seizure is the third most common neurological disorder in the elderly after stroke and dementia. To our knowledge, this is the first case of a patient presenting with epileptic seizures as a manifestation of the adult form of neuronal intranuclear inclusion disease. We further showed intranuclear inclusions in keratinocytes by skin biopsy histology. We report neuronal intranuclear inclusion disease as a cause of new‐onset epilepsy in the elderly.