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Research ArticlePediatric Neuroimaging

“Ears of the Lynx” MRI Sign Is Associated with SPG11 and SPG15 Hereditary Spastic Paraplegia

B. Pascual, S.T. de Bot, M.R. Daniels, M.C. França, C. Toro, M. Riverol, P. Hedera, M.T. Bassi, N. Bresolin, B.P. van de Warrenburg, B. Kremer, J. Nicolai, P. Charles, J. Xu, S. Singh, N.J. Patronas, S.H. Fung, M.D. Gregory and J.C. Masdeu
American Journal of Neuroradiology January 2019, 40 (1) 199-203; DOI: https://doi.org/10.3174/ajnr.A5935
B. Pascual
aFrom the Departments of Neurology (B.P., M.R.D., J.C.M.)
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S.T. de Bot
dDepartment of Neurology (S.T.d.B.), Leiden University Medical Centre, Leiden, the Netherlands
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M.R. Daniels
aFrom the Departments of Neurology (B.P., M.R.D., J.C.M.)
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M.C. França Jr
eDepartment of Neurology (M.C.F.), University of Campinas, Campinas, Brazil
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C. Toro
fNational Institutes of Health Intramural Research Program (C.T., N.J.P., M.D.G.), Bethesda, Maryland
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M. Riverol
gDepartment of Neurology (M.R.), Clínica Universidad de Navarra, Pamplona, Spain
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P. Hedera
hDepartment of Neurology (P.H.), Vanderbilt University Medical Center, Nashville, Tennessee
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M.T. Bassi
iLaboratory of Molecular Biology (M.T.B.), Scientific Institute Istituto di Ricovero e Cura a Carattere Scientifico E. Medea, Bosisio Parini, Lecco, Italy
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N. Bresolin
jDepartment of Neuroscience and Mental Health (N.B.), University Hospital Policlinico Ca'Granda, University of Milan, Milan, Italy
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B.P. van de Warrenburg
kDepartment of Neurology (B.P.v.d.W.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands
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B. Kremer
lDepartment of Neurology (B.K.), University Medical Center Groningen, Groningen, the Netherlands
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J. Nicolai
mDepartment of Neurology (J.N.), Maastricht University Medical Centre, Maastricht, the Netherlands
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P. Charles
nDepartment of Genetics (P.C.), Hôpital Pitié-Salpêtrière, Paris, France.
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J. Xu
bBiostatistics (J.X.)
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S. Singh
cRadiology (S.S., S.H.F.), Houston Methodist Research Institute, Houston, Texas
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N.J. Patronas
fNational Institutes of Health Intramural Research Program (C.T., N.J.P., M.D.G.), Bethesda, Maryland
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S.H. Fung
cRadiology (S.S., S.H.F.), Houston Methodist Research Institute, Houston, Texas
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M.D. Gregory
fNational Institutes of Health Intramural Research Program (C.T., N.J.P., M.D.G.), Bethesda, Maryland
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J.C. Masdeu
aFrom the Departments of Neurology (B.P., M.R.D., J.C.M.)
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    Fig 1.

    Corpus callosum size similar to normal in a patient with HSP-TCC. The patient had mild cognitive impairment and a mild spastic paraparesis. She had SPG 11 pathogenetic mutations. The ear of the lynx sign in this patient is shown in Fig 2.

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    Fig 2.

    Ears of the lynx on MR imaging. Axial images across the anterior forceps of the corpus callosum. Note an abnormality in the region of the forceps minor of the corpus callosum, corresponding to the genu fibers, which appear dark on T1-weighted and bright on FLAIR images (arrows). Midline sagittal images from the same individuals are seen in Fig 1.

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    Fig 3.

    Ears of the lynx. Shown are the gray-scale and corresponding negative images of the head of a lynx. The hair tufts at the tip of the ears resemble the MR imaging finding described here. Modified with permission from an original photo taken by Aleksandar Vasic.

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    Fig 4.

    ROCs for T1-weighted and FLAIR images. The receiver operating characteristic curves show better discrimination for FLAIR than for T1-weighted images. For FLAIR images, the area under the ROC curve showed that the ears of the lynx sign performed in the very good-to-excellent range for a diagnostic test.

Tables

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    Table 1:

    Demographics

    Diagnosis
    SPG11 (n = 31) or SPG15 (n = 3) MutationsMSHealthy Control
    Number343434
    Sex, female/male16:1816:1816:18
    Mean age (SD) at MRI (yr)24.6 (7.8)25.0 (6.8)24.6 (7.5)
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    Table 2:

    Interrater reliability (Cohen κ)

    Cohen κ for FLAIR (95% CI)Cohen κ for T1-Weighted (95% CI)
    Rater 1 vs 20.74 (0.60–0.88)0.51 (0.32–0.71)
    Rater 2 vs 30.74 (0.60–0.88)0.42 (0.24–0.63)
    Rater 3 vs 40.77 (0.63–0.91)0.75 (0.54–0.96)
    Rater 1 vs 30.66 (0.51–0.82)0.59 (0.38–0.80)
    Rater 2 vs 40.71 (0.56–0.86)0.40 (0.20–0.60)
    Rater 1 vs 40.73 (0.59–0.87)0.62 (0.42–0.83)
    All raters0.72 (0.61–0.82)0.53 (0.35–0.69)
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    Table 3:

    ROC results with sensitivity and specificity for FLAIR and T1-weighted images

    ROC Results
    AUC (95% CI)Sensitivity (95% CI)Specificity (95% CI)
    Sensitivity and specificity for FLAIR images
        Rater 193.9 (89.4–98.5)97.0 (84.2–99.9)90.9 (81.3–96.6)
        Rater 287.1 (79.7–94.5)81.8 (64.5–93.0)92.4 (83.2–97.5)
        Rater 387.1 (79.6–94.6)78.8 (61.1–91.0)95.5 (87.3–99.1)
        Rater 489.4 (82.3–96.5)78.8 (61.1–91.0)100 (94.6–100)
    Sensitivity and specificity for T1-weighted images
        Rater 184.8 (76.1–93.5)71.4 (51.3–86.8)98.2 (90.4–100)
        Rater 281.3 (72.0–90.5)75.0 (55.1–89.3)87.5 (75.9–94.8)
        Rater 368.8 (59.4–78.1)39.3 (21.5–59.4)98.2 (90.4–100)
        Rater 469.6 (60.4–78.9)39.3 (21.5–59.4)100 (93.6–100)
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American Journal of Neuroradiology: 40 (1)
American Journal of Neuroradiology
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B. Pascual, S.T. de Bot, M.R. Daniels, M.C. França, C. Toro, M. Riverol, P. Hedera, M.T. Bassi, N. Bresolin, B.P. van de Warrenburg, B. Kremer, J. Nicolai, P. Charles, J. Xu, S. Singh, N.J. Patronas, S.H. Fung, M.D. Gregory, J.C. Masdeu
“Ears of the Lynx” MRI Sign Is Associated with SPG11 and SPG15 Hereditary Spastic Paraplegia
American Journal of Neuroradiology Jan 2019, 40 (1) 199-203; DOI: 10.3174/ajnr.A5935

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“Ears of the Lynx” MRI Sign Is Associated with SPG11 and SPG15 Hereditary Spastic Paraplegia
B. Pascual, S.T. de Bot, M.R. Daniels, M.C. França, C. Toro, M. Riverol, P. Hedera, M.T. Bassi, N. Bresolin, B.P. van de Warrenburg, B. Kremer, J. Nicolai, P. Charles, J. Xu, S. Singh, N.J. Patronas, S.H. Fung, M.D. Gregory, J.C. Masdeu
American Journal of Neuroradiology Jan 2019, 40 (1) 199-203; DOI: 10.3174/ajnr.A5935
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Cited By...

  • 'Ear of the lynx sign: hereditary spastic paraplegia (HSP) type 11
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  • Systematic Analysis of Brain MRI Findings in Adaptor Protein Complex 4-Associated Hereditary Spastic Paraplegia
  • 'Ears of the Lynx sign: an important and useful MRI clue for diagnosis of hereditary spastic paraplegia (HSP) caused by mutation in SPG 15 gene
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