Mapping macrostructural and microstructural brain alterations in patients with neuronal intranuclear inclusion disease.

Background and purpose: Neuronal intranuclear inclusion disease (NIID) is a rare complex neurodegenerative disorder presents with various radiological features. The study aimed to investigate the structural abnormalities in NIID using multi-shell diffusion MR. Materials and methods: Twenty-eight pat...

Descripción completa

Detalles Bibliográficos
Publicado en:Neuroradiology Vol. 66; no. 8; pp. 1373 - 1383
Autores principales: Lv, Shan, Tai, Hongfei, Sun, Jun, Zhuo, Zhizheng, Duan, Yunyun, Liu, Shaocheng, Wang, An, Zhang, Zaiqiang, Liu, Yaou
Formato: research tables/charts Journal Article
Publicado: Springer Nature Aug2024
Acceso en línea:Ver este registro en EBSCOhost
fields @attributes:
  recordID: 1
pdfLink:
plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=178416302&site=ehost-live
header:
  @attributes:
    shortDbName: ccm
    uiTerm: 178416302
    longDbName: CINAHL Complete
    uiTag: AN
  controlInfo:
    bkinfo:
    dissinfo:
    jinfo:
      jid:
        00283940
        NYZ
      jtl: Neuroradiology
      issn: 00283940
      maglogo: N
    pubinfo:
      dt: Aug2024
      vid: 66
      iid: 8
      pid: 237
      pub: Springer Nature
      place: New York, New York
    artinfo:
      ui:
        178416302
        177813235
        178416302
        178416302
        10.1007/s00234-024-03406-y
        178416302
      ppf: 1373
      ppct: 10
      formats:
        fmt:
          – @attributes:
              type: T
          – @attributes:
              type: P
      tig:
        atl: Mapping macrostructural and microstructural brain alterations in patients with neuronal intranuclear inclusion disease.
      aug:
        au:
          Lv, Shan
          Tai, Hongfei
          Sun, Jun
          Zhuo, Zhizheng
          Duan, Yunyun
          Liu, Shaocheng
          Wang, An
          Zhang, Zaiqiang
          Liu, Yaou
        affil: https://ror.org/013xs5b60 Department of Radiology, Beijing Tiantan Hospital, Capital Medical University, 100070, Beijing, China
      sug:
        subj:
          Neurodegenerative Diseases Pathology
          Magnetic Resonance Imaging Methods
          Neurodegenerative Diseases Radiography
          Brain Pathology
          Diagnostic Imaging
          Human
          Male
          Female
          Adult
          Middle Age
          Aged
          Questionnaires
          Case Control Studies
          Cognition Disorders
          Adult: 19-44 years
          Middle Aged: 45-64 years
          Aged: 65+ years
          Male
          Female
      ab: Background and purpose: Neuronal intranuclear inclusion disease (NIID) is a rare complex neurodegenerative disorder presents with various radiological features. The study aimed to investigate the structural abnormalities in NIID using multi-shell diffusion MR. Materials and methods: Twenty-eight patients with adult-onset NIID and 32 healthy controls were included. Volumetric and diffusion MRI measures, including volume, fractional anisotropy (FA), mean diffusivity (MD), intracellular volume fraction (ICVF), orientation dispersion index (ODI), and isotropic volume fraction (ISOVF) of six brain structures, including cortex, subcortical GM, cerebral WM, cerebellar GM and WM, and brainstem, were obtained and compared between NIID and healthy controls. Associations between MRI measures and clinical variables were investigated. Results: Brain lesions of NIID included corticomedullary junction lesions on DWI, confluent leukoencephalopathy, lesions on callosum, cerebellar middle peduncle, cerebellar paravermal area and brainstem, and brain atrophy. Compared to healthy controls, NIID showed extensive volume loss of all the six brain regions (all p < 0.001); lower FA in cerebral WM (p < 0.001); higher MD in all WM regions; lower ODI in cortex (p < 0.001); higher ODI in subcortical GM (p < 0.001) and brainstem (p = 0.016); lower ICVF in brainstem (p = 0.001), and cerebral WM (p < 0.001); higher ISOVF in all the brain regions (p < 0.001). Higher MD of cerebellar WM was associated with worse cognitive level as evaluated by MoCA scores (p = 0.011). Conclusions: NIID patients demonstrated widespread brain atrophy but heterogeneous diffusion alterations. Cerebellar WM integrity impairment was correlated with the cognitive decline. The findings of the current study offer a sophisticated picture of brain structural alterations in NIID.
      pubtype: Academic Journal
      doctype:
        research
        tables/charts
        Journal Article
      ougenre: Article
    language: English
    refInfo:
    holdings:
      @attributes:
        islocal: N