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Research ArticleAdult Brain

MR Imaging Features of the Cerebellum in Adult-Onset Neuronal Intranuclear Inclusion Disease: 8 Cases

A. Sugiyama, N. Sato, Y. Kimura, T. Maekawa, M. Enokizono, Y. Saito, Y. Takahashi, H. Matsuda and S. Kuwabara
American Journal of Neuroradiology November 2017, 38 (11) 2100-2104; DOI: https://doi.org/10.3174/ajnr.A5336
A. Sugiyama
aFrom the Departments of Radiology (A.S., N.S., Y.K., T.M., M.E.)
eDepartment of Neurology (A.S., S.K.), Graduate School of Medicine, Chiba University, Chiba, Japan.
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N. Sato
aFrom the Departments of Radiology (A.S., N.S., Y.K., T.M., M.E.)
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Y. Kimura
aFrom the Departments of Radiology (A.S., N.S., Y.K., T.M., M.E.)
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T. Maekawa
aFrom the Departments of Radiology (A.S., N.S., Y.K., T.M., M.E.)
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M. Enokizono
aFrom the Departments of Radiology (A.S., N.S., Y.K., T.M., M.E.)
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Y. Saito
bPathology and Laboratory Medicine (Y.S.)
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Y. Takahashi
cNeurology (Y.T.)
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H. Matsuda
dIntegrative Brain Imaging Center (H.M.), National Center of Neurology and Psychiatry, Tokyo, Japan
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S. Kuwabara
eDepartment of Neurology (A.S., S.K.), Graduate School of Medicine, Chiba University, Chiba, Japan.
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    Fig 1.

    A representative case (patient 1) showing the abnormal signals at the paravermal area and the middle cerebellar peduncle. FLAIR axial images (A and B), a coronal image (C), and a sagittal image (D) show atrophy of the cerebellum and bilateral high signal intensity in the medial part of the cerebellar hemisphere immediately beside the vermis (the paravermal area) (black arrows) and in the middle cerebellar peduncle (black arrowheads).

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    Fig 2.

    Patient 4. DWI (A) shows high-intensity signal along the corticomedullary junction. A FLAIR axial image (B) shows diffuse high intensity in the bilateral cerebral hemispheres. FLAIR axial (C) and coronal (D) images show atrophy of the cerebellum and high-intensity signal in the medial part of the cerebellar hemisphere right beside the vermis (the paravermal area).

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    Fig 3.

    Patient 2. A FLAIR axial image shows bilateral high signal intensities in the middle cerebellar peduncle.

Tables

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    Table 1:

    Summary of the clinical features of patients with NIID

    Patient No.
    12345678
    Age at onset (yr)6452626267676764
    SexMFFMFMFM
    Disease duration at MRI (yr)29835615
    Clinical manifestations
        Dementia–––++–+–
        Delusion––+–––––
        Muscle weakness––––––––
        Tremor––Postural–PosturalPostural–Resting, postural
        Rigidity––––––––
        Ataxia–––++––+
        Hyporeflexia or areflexia in DTR++++++++
        Sensory disturbance––––––––
        Urinary incontinence–––++–––
        Transient visual field abnormality++–––––+
        Transient hemiparesis–+––––––
        Transient abnormal behavior–+–+–+––
        Generalized convulsion––––––––
        Disturbance of consciousness–––––+++
    Cognitive screening test
        MMSE30293014a30282429
    Frontal lobe function test
        FAB13b1614b11b12b168bNE
    CSF
        Cell (mm3)324116NENE
        Protein (mg/dL)6655100475248NENE
    • Note:—DTR indicates deep tendon reflex; –, absence of the manifestation; +, presence of the manifestation; NE, not examined.

    • ↵a Decreased MMSE score below a cutoff score of 24.

    • ↵b Decreased FAB score below the published age-matched average.

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    Table 2:

    Summary of MRI findings of the 8 patients with NIID

    Patient No.
    12345678
    Cerebrum
        High-intensity signal along the corticomedullary junction on DWI++++++++
        Diffuse high-intensity signal of cerebral white matter on FLAIR images++++++++
    Cerebellum
        Atrophy++++++++
        High-intensity signal in the medial part of cerebellar hemisphere right beside the vermis on FLAIR images+–++++–+
        High-intensity signal in the middle cerebellar peduncle on FLAIR images+++––+––
    • Note:— – indicates absence of the finding; +, presence of the finding.

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American Journal of Neuroradiology: 38 (11)
American Journal of Neuroradiology
Vol. 38, Issue 11
1 Nov 2017
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MR Imaging Features of the Cerebellum in Adult-Onset Neuronal Intranuclear Inclusion Disease: 8 Cases
A. Sugiyama, N. Sato, Y. Kimura, T. Maekawa, M. Enokizono, Y. Saito, Y. Takahashi, H. Matsuda, S. Kuwabara
American Journal of Neuroradiology Nov 2017, 38 (11) 2100-2104; DOI: 10.3174/ajnr.A5336

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MR Imaging Features of the Cerebellum in Adult-Onset Neuronal Intranuclear Inclusion Disease: 8 Cases
A. Sugiyama, N. Sato, Y. Kimura, T. Maekawa, M. Enokizono, Y. Saito, Y. Takahashi, H. Matsuda, S. Kuwabara
American Journal of Neuroradiology Nov 2017, 38 (11) 2100-2104; DOI: 10.3174/ajnr.A5336
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