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Prolonged Conscious Disturbance in a Patient with Neuronal Intranuclear Inclusion Disease Masquerading as Hashimoto
Chien-Ya Hsu1, Chun-Wei Chang2
1Department of Neurology, Chang Gung Memorial Hospital Linkou Medical Center, Taoyuan, Taiwan.
Acta Neurologica Taiwanica
|April 25, 2026
Summary
A rare case of Neuronal Intranuclear Inclusion Disease (NIID) mimicked Hashimoto encephalopathy in an elderly woman. Diagnosis required advanced imaging and genetic testing, highlighting challenges in differentiating neurodegenerative from autoimmune brain disorders.
Area of Science:
- Neurology
- Neuroimmunology
- Genetics
Background:
- Neuronal Intranuclear Inclusion Disease (NIID) is a rare neurodegenerative disorder.
- Hashimoto encephalopathy is an autoimmune condition affecting the brain.
- Differentiating these conditions can be clinically challenging.
Purpose of the Study:
- To report a unique case of NIID presenting as Hashimoto encephalopathy.
- To emphasize the diagnostic complexities in neurodegenerative and autoimmune neurological disorders.
- To highlight the need for comprehensive diagnostic strategies.
Main Methods:
- Clinical case presentation of a 72-year-old female.
- Comprehensive neurological and immunological evaluations.
- Magnetic resonance imaging (MRI) and genetic testing for NIID confirmation.
Main Results:
- The patient presented with acute consciousness disturbance.
- Diagnosis confirmed as NIID, initially suspected as Hashimoto encephalopathy.
- Elevated antithyroid peroxidase antibodies were noted.
- Characteristic MRI findings and genetic confirmation of NIID were obtained.
- Partial improvement was observed with corticosteroid treatment.
Conclusions:
- NIID can present atypically, mimicking autoimmune encephalopathies like Hashimoto encephalopathy.
- Thorough diagnostic evaluation, including advanced imaging and genetic analysis, is crucial.
- Distinguishing between neurodegenerative and autoimmune neurological conditions remains a significant clinical challenge.
Keywords:
Case reporthashimoto encephalopathyneurodegenerative disorderneuronal intranuclear inclusion diseaseMore Related Videos
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