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<p>Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disorder characterized primarily by progressive cognitive decline and tremor. While these clinical features are well-recognized, the specific structural neural substrates and morphometric alterations underlying these symptoms remain poorly understood. This prospective study included 10 genetically confirmed NIID patients and 10 age- and sex-matched controls. Following high-resolution T1-weighted MRI and MoCA-based cognitive assessment, atlas-based morphometry was utilized to characterize gray-matter alterations. The analytical workflow prioritized identifying global atrophy patterns between groups, followed by correlation analyses between identified atrophic regions and clinical markers (MoCA scores and disease onset). Additionally, subgroup comparisons were conducted within the patient cohort to identify the neural correlates of tremor. Compared with healthy controls, NIID patients exhibited widespread gray-matter atrophy primarily involving the extensive temporal and occipital cortices, limbic and paralimbic regions (including the cingulate gyri, hippocampus, and amygdala), and the thalamus (Bonferroni-corrected, <i>p</i> &lt; 0.05). Notably, lower MoCA scores were significantly associated with reduced volumes in the left hippocampus and the left temporal pole (extending to the superior and middle temporal gyri) (all <i>p</i> &lt; 0.05). Earlier disease onset was correlated with the relative preservation of the left thalamus (<i>r</i> = -0.755, <i>p</i> = 0.019). Finally, patients manifesting tremor displayed significantly diminished gray-matter volume in the right hippocampus compared to tremor-free patients (<i>t</i> = 2.472, <i>p</i> = 0.039). Distinct morphometric substrates underpin cognitive dysfunction and tremor in NIID. Cognitive decline is primarily associated with atrophy in the left hippocampus and temporal regions, whereas tremor manifestation is linked to right hippocampal volume loss. These findings elucidate the neuroanatomical basis of NIID’s core symptoms and highlight regional brain atrophy as a potential imaging biomarker for disease monitoring and future therapeutic trials.</p>

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Neural substrates of cognitive decline and tremor in neuronal intranuclear inclusion disease: a morphometric MRI study

  • 期刊论文

  • 发表日期：

    2026年7月17日

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  • Yahui Ouyang,
  • Jiehuan Wang,
  • ChongChong Li,
  • Guorong Zhang,
  • Shujun Zhang

摘要

Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disorder characterized primarily by progressive cognitive decline and tremor. While these clinical features are well-recognized, the specific structural neural substrates and morphometric alterations underlying these symptoms remain poorly understood. This prospective study included 10 genetically confirmed NIID patients and 10 age- and sex-matched controls. Following high-resolution T1-weighted MRI and MoCA-based cognitive assessment, atlas-based morphometry was utilized to characterize gray-matter alterations. The analytical workflow prioritized identifying global atrophy patterns between groups, followed by correlation analyses between identified atrophic regions and clinical markers (MoCA scores and disease onset). Additionally, subgroup comparisons were conducted within the patient cohort to identify the neural correlates of tremor. Compared with healthy controls, NIID patients exhibited widespread gray-matter atrophy primarily involving the extensive temporal and occipital cortices, limbic and paralimbic regions (including the cingulate gyri, hippocampus, and amygdala), and the thalamus (Bonferroni-corrected, p < 0.05). Notably, lower MoCA scores were significantly associated with reduced volumes in the left hippocampus and the left temporal pole (extending to the superior and middle temporal gyri) (all p < 0.05). Earlier disease onset was correlated with the relative preservation of the left thalamus (r = -0.755, p = 0.019). Finally, patients manifesting tremor displayed significantly diminished gray-matter volume in the right hippocampus compared to tremor-free patients (t = 2.472, p = 0.039). Distinct morphometric substrates underpin cognitive dysfunction and tremor in NIID. Cognitive decline is primarily associated with atrophy in the left hippocampus and temporal regions, whereas tremor manifestation is linked to right hippocampal volume loss. These findings elucidate the neuroanatomical basis of NIID’s core symptoms and highlight regional brain atrophy as a potential imaging biomarker for disease monitoring and future therapeutic trials.