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Research ArticleAdult Brain

Brain Abnormalities and Epilepsy in Patients with Parry-Romberg Syndrome

C. De la Garza-Ramos, A. Jain, S.A. Montazeri, L. Okromelidze, R. McGeary, A.A. Bhatt, S.J.S. Sandhu, S.S. Grewal, A. Feyissa, J.I. Sirven, A.L. Ritaccio, W.O. Tatum, V. Gupta and E.H. Middlebrooks
American Journal of Neuroradiology June 2022, 43 (6) 850-856; DOI: https://doi.org/10.3174/ajnr.A7517
C. De la Garza-Ramos
aFrom the Department of Neuroradiology (C.D.l.G.-R., A.J., S.A.M., L.O., R.M., A.A.B., S.J.S., V.G., E.H.M.), Mayo Clinic, Jacksonville, Florida
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A. Jain
aFrom the Department of Neuroradiology (C.D.l.G.-R., A.J., S.A.M., L.O., R.M., A.A.B., S.J.S., V.G., E.H.M.), Mayo Clinic, Jacksonville, Florida
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S.A. Montazeri
aFrom the Department of Neuroradiology (C.D.l.G.-R., A.J., S.A.M., L.O., R.M., A.A.B., S.J.S., V.G., E.H.M.), Mayo Clinic, Jacksonville, Florida
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L. Okromelidze
aFrom the Department of Neuroradiology (C.D.l.G.-R., A.J., S.A.M., L.O., R.M., A.A.B., S.J.S., V.G., E.H.M.), Mayo Clinic, Jacksonville, Florida
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R. McGeary
aFrom the Department of Neuroradiology (C.D.l.G.-R., A.J., S.A.M., L.O., R.M., A.A.B., S.J.S., V.G., E.H.M.), Mayo Clinic, Jacksonville, Florida
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A.A. Bhatt
aFrom the Department of Neuroradiology (C.D.l.G.-R., A.J., S.A.M., L.O., R.M., A.A.B., S.J.S., V.G., E.H.M.), Mayo Clinic, Jacksonville, Florida
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S.J.S. Sandhu
aFrom the Department of Neuroradiology (C.D.l.G.-R., A.J., S.A.M., L.O., R.M., A.A.B., S.J.S., V.G., E.H.M.), Mayo Clinic, Jacksonville, Florida
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S.S. Grewal
bDepartment of Neurologic Surgery (S.S.G.), Mayo Clinic, Jacksonville, Florida
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A. Feyissa
cDepartment of Neurology (A.F., J.I.S., A.L.R., W.O.T.), Mayo Clinic, Jacksonville, Florida
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J.I. Sirven
cDepartment of Neurology (A.F., J.I.S., A.L.R., W.O.T.), Mayo Clinic, Jacksonville, Florida
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A.L. Ritaccio
cDepartment of Neurology (A.F., J.I.S., A.L.R., W.O.T.), Mayo Clinic, Jacksonville, Florida
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W.O. Tatum
cDepartment of Neurology (A.F., J.I.S., A.L.R., W.O.T.), Mayo Clinic, Jacksonville, Florida
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V. Gupta
aFrom the Department of Neuroradiology (C.D.l.G.-R., A.J., S.A.M., L.O., R.M., A.A.B., S.J.S., V.G., E.H.M.), Mayo Clinic, Jacksonville, Florida
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E.H. Middlebrooks
aFrom the Department of Neuroradiology (C.D.l.G.-R., A.J., S.A.M., L.O., R.M., A.A.B., S.J.S., V.G., E.H.M.), Mayo Clinic, Jacksonville, Florida
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    FIG 1.

    Exemplary MR imaging of atrophy and white matter disease severity. Coronal FLAIR MR imaging shows mild left hemisphere atrophy (A), moderate right hemisphere atrophy (B), and severe left hemisphere atrophy (C). Axial FLAIR MR imaging shows mild right hemisphere WMD (D), moderate left hemisphere WMD (E), and severe right hemisphere WMD (F).

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    FIG 2.

    A, Axial FLAIR MR imaging in a patient with epilepsy shows severe, confluent left hemisphere white matter hyperintensity with extensive areas of punctate susceptibility artifacts throughout the left hemisphere on susceptibility-weighted imaging (B). Most of the hypointense foci on susceptibility-weighted imaging are hypointense on the matching phase image (C), consistent with prior microhemorrhages; however, few show hyperintense signal on the phase image (arrows in B and C), consistent with calcifications (confirmed by CT, not shown).

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    FIG 3.

    A, Axial FLAIR MR imaging in a patient with epilepsy shows severe, confluent left hemisphere white matter hyperintensity. B, Postcontrast T1-weighted MR imaging reveals leptomeningeal enhancement and sulcal effacement with decreased perfusion to the same area on arterial spin-labeling perfusion MR imaging (C).

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    FIG 4.

    A, Initial head CT in a patient with epilepsy reveals effacement of the sulci over the left parietal high convexity. B, Subsequent T1-weighted MR imaging performed 10 months later shows improvement in sulcal effacement, but with diffuse left hemisphere white matter hyperintensity on double inversion recovery MR imaging (C, arrow). D, Sagittal postcontrast T1-weighted MR imaging shows faint periventricular and perivascular enhancement (arrowheads). E, Concomitant FDG-PET shows left parietal hypometabolism in the affected area.

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    FIG 5.

    Spectrum of unilateral sulcal effacement. In 1 patient with epilepsy, axial T2-weighted MR imaging (A) shows right hemispheric sulcal effacement affecting the right frontal and parietal lobes, which remained unchanged on MR imaging performed 1 year later (B). C, Coronal FLAIR MR imaging in a second patient with epilepsy shows diffuse right hemisphere sulcal effacement with hyperintensity in the right hippocampal head, with resolution of sulcal effacement and development of right mesial temporal sclerosis on MR imaging approximately 6 months later (D).

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    FIG 6.

    Proton MRS in a 12-year-old boy with left-sided Parry-Romberg syndrome and epilepsy shows the spectrum from the normal-appearing right hemisphere white matter (A) compared with the area of white matter FLAIR hyperintensity in the left hemisphere (B). There is a relative reduction in Cho and NAA, with a slight elevation of Cr on the abnormal side. PPM indicates parts per million.

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    Table 1:

    Parry-Romberg syndrome patient characteristicsa

    Variables
    Total80
    Age at work-up and imaging (yr)37 (26–56)
    Age at hemifacial atrophy identification (yr)20 (13–43)
    Sex
        Male24 (30%)
        Female56 (70%)
    Laterality of hemifacial atrophy
        Right37 (46%)
        Left43 (54%)
        Bilateral0 (0%)
    Brain abnormalities on MR imaging
        Normal32 (40%)
        Abnormal48 (60%)
    Overall laterality of brain MR imaging findings
        Unilateral34 (42%)
        Bilateral14 (17%)
    Laterality of unilateral brain MR imaging findings in relation to hemifacial atrophy
        Ipsilateral26 (35%)
        Contralateral6 (7%)
    Epilepsy
        Present16 (20%)
        Absent64 (80%)
    • ↵a Data are No. (%) or median (IQR, 1–3).

    • View popup
    Table 2:

    Frequency and severity of unilateral brain abnormalities in patients with Parry-Romberg syndrome and epilepsy compared with cases without epilepsya

    Nonepilepsy (n = 64)Epilepsy (n = 16)P Value CategoriesP Value Overall
    Hemispheric atrophy
        None61 (95%)8 (50%)<.001<.001
        Mild1 (1.6%)1 (6%)
        Moderate1 (1.6%)3 (19%)
        Severe1 (1.6%)4 (25%)
    WMD
        None49 (77%)2 (12%)<.001<.001
        Mild10 (15%)7 (44%)
        Moderate2 (3%)4 (25%)
        Severe3 (5%)3 (19%)
    Hemispheric microhemorrhage
        None61 (95%)13 (81%).015.091
        Mild3 (5%)1 (6%)
        Moderate00
        Severe02 (12%)
    Leptomeningeal enhancement
        Absent63 (98%)15 (94%).362
        Present1 (1.6%)1 (6%)
    • ↵a Some patients had more than 1 finding.

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American Journal of Neuroradiology: 43 (6)
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Vol. 43, Issue 6
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C. De la Garza-Ramos, A. Jain, S.A. Montazeri, L. Okromelidze, R. McGeary, A.A. Bhatt, S.J.S. Sandhu, S.S. Grewal, A. Feyissa, J.I. Sirven, A.L. Ritaccio, W.O. Tatum, V. Gupta, E.H. Middlebrooks
Brain Abnormalities and Epilepsy in Patients with Parry-Romberg Syndrome
American Journal of Neuroradiology Jun 2022, 43 (6) 850-856; DOI: 10.3174/ajnr.A7517

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Brain Abnormalities in Parry-Romberg Syndrome
C. De la Garza-Ramos, A. Jain, S.A. Montazeri, L. Okromelidze, R. McGeary, A.A. Bhatt, S.J.S. Sandhu, S.S. Grewal, A. Feyissa, J.I. Sirven, A.L. Ritaccio, W.O. Tatum, V. Gupta, E.H. Middlebrooks
American Journal of Neuroradiology Jun 2022, 43 (6) 850-856; DOI: 10.3174/ajnr.A7517
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