Objective: Our aim is to report an unusual Neuronal intranuclear inclusion disease (NIID) case of a woman who initially presented recurrent Transient Global Amnesia (TGA) like symptoms before the onset of any other typical neurological symptoms of NIID.

Background: Neuronal intranuclear inclusion disease (NIID) is a rare progressive neurodegenerative disease, which demonstrates heterogenous clinical symptoms. It is characterized by the presence of eosinophilic hyaline intranuclear inclusions in the central and peripheral nervous systems and visceral organs. The most common neurological signs include ataxia, extrapyramidal signs, and lower motor neuron signs, and most NIID patients show dementia or Parkinson’s disease-like symptoms in the advanced stage.

Method: Single case report and description of clinical characteristics.

Results: A 65-year-old Korean woman was referred to our neurology department complaining of transient amnestic symptoms. And then, she had presented two more times of similar amnesia as like clinically TGA for the next 9 years. Although her amnesia symptoms showed early improvements, her following brain imaging by diffusion MRI showed gradually progressing hyperintensities in cortico-medullary junctions, and skin biopsy confirmed eosinophilic intranuclear inclusions. These neuro-radiological and neuro-pathological findings confirmed the defined final diagnosis of NIID.

Conclusion: To our knowledge, this is the first case report of a definite NIID patient, initially presenting as like clinical recurrent TGA after severe stress and fatigue. As like this case, the diagnosis of NIID should be considered if there is a history of clinically recurrent TGA preceding dementia or Parkinson’s disease-like symptoms.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/unusual-neuronal-intranuclear-inclusion-disease-niid-with-initial-presentation-mimicking-recurrent-transient-global-amnesia-tga/