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Research ArticlePediatric Neuroimaging

Acute Necrotizing Encephalopathy of Childhood: Correlation of MR Findings and Clinical Outcome

A.M. Wong, E.M. Simon, R.A. Zimmerman, H.-S. Wang, C.-H. Toh and S.-H. Ng
American Journal of Neuroradiology October 2006, 27 (9) 1919-1923;
A.M. Wong
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E.M. Simon
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R.A. Zimmerman
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H.-S. Wang
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C.-H. Toh
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S.-H. Ng
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    Fig 1.

    A 1.3-year-old boy (patient 1) left with spasticity and decerebrate posture. A, coronal T2-weighted imaging (4000 ms/90 ms, repetition time [TR]/echo time [TE]) shows symmetrical hyperintensity in the thalami (arrows), the centrum semiovale (arrowheads), and the brain stem, including the midbrain (double arrowheads) and the pons (double arrows). Note swelling of the thalami.

    B, Postcontrast coronal T1-weighted imaging (630 ms/20 ms, TR/TE) shows irregular ringlike enhancement in the thalami (arrows).

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    Fig 2.

    A 2.3-year-old boy (patient 12) with severe sequelae.

    A, Axial T1-weighted imaging (449 ms/12 ms, TR/TE) shows sharply marginated hypointensity in the cerebellum (arrowheads).

    B, Axial T1-weighted imaging shows hyperintensity in the thalami (arrows) and the right occipital lobe (double arrows). Sharply marginated hypointense areas are found in the cerebral white matter (arrowheads) and the internal capsules (small arrowheads). The lentiform nuclei also show mixed intensity (double arrowheads).

    C and D, Coronal T2-weighted imaging show mixed intensity in the thalami (arrows), and hyperintensity in the cerebral white matter, internal capsules (small arrowheads), and the cerebellum (double arrowheads). There are 2 types of white matter lesions: those in the central white matter and internal capsules showing hyperintensity comparable with the CSF (arrowheads) and those in the subcortical regions being less hyperintense (double arrows).

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    Fig 3.

    A 10-year-old boy (patient 5) recovered with hand tremor. Follow-up coronal T2-weighted imaging shows shrunken bilateral thalami with cavitation (arrows).

Tables

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    Table 1:

    Clinical features of 12 patients with acute necrotizing encephalopathy of childhood

    Patient No./Age/SexClinical FeaturesPositive Viral StudyOutcomeHUI:1 Outcome Category
    1/1 y 4 mo/MFever, conscious disturbance, seizureInfluenza BSpasticity with decerebrate posture3
    2/3 y 3 mo/MFever, conscious disturbance, seizureMycoplasmaSpasticity2
    3/12 y/FFever, conscious disturbance, decerebrate posture, spasticity, urine and stool incontinenceInfluenza B, mycoplasma (possible)Spasticity, urine and stool incontinence2
    4/7 mo/MConscious disturbanceN/AFully recovered1
    5/10 y/MFever, conscious disturbance, seizureInfluenza ABilateral hand tremor; left-sided dystonia2
    6/1 y/MFever, lower extremity rigidityN/AFully recovered1
    7/3 y 5 mo/MFever, conscious disturbance, decerebrate postureInfluenza BSpasticity, speech disorder2
    8/2 mo/MFever, conscious disturbance, seizure, motor weakness with cogwheel rigidityHHV-6Speech disorder; full motor recovery2
    9/6 y/FFever, skin rash, conscious disturbance, seizure, lower extremity paralysis, speech disabilityAdenovirusNeurogenic bladder, lower extremity weakness2
    10/2 y/MFever, coma, seizureN/ALower extremity weakness with rigidity2
    11/2 y 6 mo/FFever, conscious disturbance, seizure, lower extremity paralysisNegativeLower extremity weakness2
    12/2 y 4 mo/FFever, conscious disturbance 1 mo, vomiting, seizureEnterovirusConscious disturbance, diplegia, spasticity3
    • Note:—NUI:1 indicates Health Utilities Index Mark 1; HHV-6, human herpes virus 6; N/A, not available.

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    Table 2:

    Neuroimaging findings in the initial MR and the follow-up MR study in 12 patients with acute necrotizing encepalopathy of childhood

    Patient No.Duration from Onset to Initial and to Follow-up MR Studies (d)Location of InvolvementHemorrhage (1)Cavitation (1)MR Score
    Brain Stem (1)White Matter (1) (Cerebral, cerebellar)AB
    10; 43++, +++43
    20; 103−+, +−−10
    310; 47+−, ++−32
    43; 83++, −−−21
    52; 182+−, −−+22
    69; 76−+, −−−10
    70; 19++, −+−32
    87; 15++, −−+32
    918; 134+−, −−−11
    100; 16++, −−−21
    113; 16+−, −+−22
    1210; 29++, +++43
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American Journal of Neuroradiology: 27 (9)
American Journal of Neuroradiology
Vol. 27, Issue 9
October 2006
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Cite this article
A.M. Wong, E.M. Simon, R.A. Zimmerman, H.-S. Wang, C.-H. Toh, S.-H. Ng
Acute Necrotizing Encephalopathy of Childhood: Correlation of MR Findings and Clinical Outcome
American Journal of Neuroradiology Oct 2006, 27 (9) 1919-1923;

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Acute Necrotizing Encephalopathy of Childhood: Correlation of MR Findings and Clinical Outcome
A.M. Wong, E.M. Simon, R.A. Zimmerman, H.-S. Wang, C.-H. Toh, S.-H. Ng
American Journal of Neuroradiology Oct 2006, 27 (9) 1919-1923;
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