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AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Research ArticleNeurodegenerative Disorder Imaging

Brainstem Chipmunk Sign: A Diagnostic Imaging Clue across All Subtypes of Alexander Disease

Thaís Armangue, Matthew T. Whitehead, Davide Tonduti, Laura Farina, Ali Reza Tavasoli, Arastoo Vossough, Mariko L. Bennett, Ylenia Vaia, Geneviève Bernard, Ettore Salsano, Saadet Mercimek-Andrews, Amy Waldman and Adeline Vanderver
American Journal of Neuroradiology June 2024, 45 (6) 769-772; DOI: https://doi.org/10.3174/ajnr.A8220
Thaís Armangue
aFrom the Neuroimmunology Program (T.A.), Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS)-Hospital Clinic, University of Barcelona, Barcelona, Spain
bNeurology Department (T.A.), Neuroimmunology Unit, Sant Joan de Deu Children’s Hospital, University of Barcelona, Barcelona, Spain
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Matthew T. Whitehead
cDepartment of Radiology (M.T.W., A.Vossough), Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
dPerelman School of Medicine (M.T.W., A.Vossough, A.W., A.Vanderver), University of Pennsylvania, Philadelphia, Pennsylvania
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  • ORCID record for Matthew T. Whitehead
Davide Tonduti
eUnit of Pediatric Neurology (D.T., Y.V.), Center for Diagnosis and Treatment of Leukodystrophies, V. Buzzi Children’s Hospital, University of Milan, Milan, Italy
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Laura Farina
fNeuroimaging Laboratory (L.F.), IRCCS Fondazione Santa Lucia, Rome, Italy
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Ali Reza Tavasoli
gDepartment of Neurology (A.R.T.), Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
hDepartment of Neurology (A.R.T.), Barrow Neurological Institute, Phoenix Children’s Hospital, Phoenix, Arizona
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Arastoo Vossough
cDepartment of Radiology (M.T.W., A.Vossough), Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
dPerelman School of Medicine (M.T.W., A.Vossough, A.W., A.Vanderver), University of Pennsylvania, Philadelphia, Pennsylvania
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Mariko L. Bennett
oDivision of Neurology (M.L.B., Y.V., A.W., A.Vanderver), Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania.
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Ylenia Vaia
eUnit of Pediatric Neurology (D.T., Y.V.), Center for Diagnosis and Treatment of Leukodystrophies, V. Buzzi Children’s Hospital, University of Milan, Milan, Italy
oDivision of Neurology (M.L.B., Y.V., A.W., A.Vanderver), Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania.
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Geneviève Bernard
iDepartments of Neurology and Neurosurgery, Pediatrics, and Human Genetics (G.B.), McGill University, Montreal, Quebec, Canada
jDepartment of Specialized Medicine (G.B.), Division of Medical Genetics, McGill University Health Centre, Montreal, Quebec, Canada
kChild Health and Human Development Program (G.B.), Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada
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Ettore Salsano
lUnit of Rare Neurological Diseases (E.S.), Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
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Saadet Mercimek-Andrews
mDepartment of Medical Genetics (S.M.-A.), Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada
nThe Hospital for Sick Children (S.M.-A.), Toronto, Ontario, Canada
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Amy Waldman
dPerelman School of Medicine (M.T.W., A.Vossough, A.W., A.Vanderver), University of Pennsylvania, Philadelphia, Pennsylvania
oDivision of Neurology (M.L.B., Y.V., A.W., A.Vanderver), Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania.
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Adeline Vanderver
dPerelman School of Medicine (M.T.W., A.Vossough, A.W., A.Vanderver), University of Pennsylvania, Philadelphia, Pennsylvania
oDivision of Neurology (M.L.B., Y.V., A.W., A.Vanderver), Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania.
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    FIGURE

    Chipmunk MR imaging sign. T2-weighted axial MRI through the medulla oblongata in subjects with AxD type I (A) and AxD type II (B), an image of a chipmunk face (A, white circle), and a T2WI axial scan of the medulla oblongata in a healthy subject (B, white circle). A typical chipmunk sign in AxD type I (white box in A) and AxD type II (white box in B). The black arrow indicates the inferior olivary nucleus; black arrowhead, medial lemniscus; white arrow, inferior peduncle; white arrowhead, medial longitudinal fasciculus; double white arrows, pyramidal tracts.

Tables

  • Figures
  • Involvement of different anatomic structures of the medulla oblongata and additional MR imaging features in subjects with AxD and non-AxD leukodystrophy on T2-weighted imagesa

    AxDNon-AxDAxD Type IAxD Type IIVWMAPBDOther
    Total MRIs reviewed557432216467
    Positive chipmunk sign48102818343
    T2-hyperintense dorsal medulla52172921548
    T2-hyperintense pyramids
     No859712057
     Yes, asymmetric5123001
     Yes, symmetric42132317445
    T2-hyperintense central ION
     No858621057
     Yes, asymmetric5114001
     Yes, symmetric41132415445
    T2-hypointense peripheral ION
     No662422060
     Yes, asymmetric1001000
     Yes, symmetric47112718443
    T2-hypointense IP
     No758431057
     Yes, asymmetric3112001
     Yes, symmetric45142716536
    T2-hyperintense ML38121918444
    Atrophy207416142
    Mass effect lesions5232002
    T2-hypointense MLF358258332
    • Note:—MLF indicates medial longitudinal fasciculus; ML, medial lemniscus; IP, inferior peduncle.

    • ↵a See the Online Supplemental Data for details on patients and the comparison group.

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American Journal of Neuroradiology: 45 (6)
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Cite this article
Thaís Armangue, Matthew T. Whitehead, Davide Tonduti, Laura Farina, Ali Reza Tavasoli, Arastoo Vossough, Mariko L. Bennett, Ylenia Vaia, Geneviève Bernard, Ettore Salsano, Saadet Mercimek-Andrews, Amy Waldman, Adeline Vanderver
Brainstem Chipmunk Sign: A Diagnostic Imaging Clue across All Subtypes of Alexander Disease
American Journal of Neuroradiology Jun 2024, 45 (6) 769-772; DOI: 10.3174/ajnr.A8220

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Brainstem Chipmunk Sign in Alexander Disease
Thaís Armangue, Matthew T. Whitehead, Davide Tonduti, Laura Farina, Ali Reza Tavasoli, Arastoo Vossough, Mariko L. Bennett, Ylenia Vaia, Geneviève Bernard, Ettore Salsano, Saadet Mercimek-Andrews, Amy Waldman, Adeline Vanderver
American Journal of Neuroradiology Jun 2024, 45 (6) 769-772; DOI: 10.3174/ajnr.A8220
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