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Research ArticleAdult Brain

MRI Shrimp Sign in Cerebellar Progressive Multifocal Leukoencephalopathy: Description and Validation of a Novel Observation

N. Adra, A.E. Goodheart, O. Rapalino, P. Caruso, S.S. Mukerji, R.G. González, N. Venna and J.D. Schmahmann
American Journal of Neuroradiology June 2021, 42 (6) 1073-1079; DOI: https://doi.org/10.3174/ajnr.A7145
N. Adra
aFrom the Department of Neurology (N.A., A.E.G., S.S.M., N.V., J.D.S.)
bAtaxia Center, Cognitive Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology (N.A., A.E.G., J.D.S.)
eWellesley College (N.A.), Wellesley, Massachusetts.
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A.E. Goodheart
aFrom the Department of Neurology (N.A., A.E.G., S.S.M., N.V., J.D.S.)
bAtaxia Center, Cognitive Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology (N.A., A.E.G., J.D.S.)
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O. Rapalino
cNeuroradiology Division (O.R., P.C., R.G.G.)
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P. Caruso
cNeuroradiology Division (O.R., P.C., R.G.G.)
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S.S. Mukerji
aFrom the Department of Neurology (N.A., A.E.G., S.S.M., N.V., J.D.S.)
dDepartment of Neurology, (S.S.M., N.V.), Neuroinfectious Diseases Unit, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
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R.G. González
cNeuroradiology Division (O.R., P.C., R.G.G.)
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N. Venna
aFrom the Department of Neurology (N.A., A.E.G., S.S.M., N.V., J.D.S.)
dDepartment of Neurology, (S.S.M., N.V.), Neuroinfectious Diseases Unit, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
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J.D. Schmahmann
aFrom the Department of Neurology (N.A., A.E.G., S.S.M., N.V., J.D.S.)
bAtaxia Center, Cognitive Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology (N.A., A.E.G., J.D.S.)
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References

  1. 1.↵
    1. Major EO,
    2. Yousry TA,
    3. Clifford DB
    . Pathogenesis of progressive multifocal leukoencephalopathy and risks associated with treatments for multiple sclerosis: a decade of lessons learned. Lancet Neurol 2018;17:467–80 doi:10.1016/S1474-4422(18)30040-1 pmid:29656742
    CrossRefPubMed
  2. 2.↵
    1. Ferenczy MW,
    2. Marshall LJ,
    3. Nelson CD, et al
    . Molecular biology, epidemiology, and pathogenesis of progressive multifocal leukoencephalopathy, the JC virus-induced demyelinating disease of the human brain. Clin Microbiol Rev 2012;25:471–506 doi:10.1128/CMR.05031-11 pmid:22763635
    Abstract/FREE Full Text
  3. 3.↵
    1. Saribas AS,
    2. Ozdemir A,
    3. Lam C, et al
    . JC virus-induced progressive multifocal leukoencephalopathy. Future Virol 2010;5:313–23 doi:10.2217/fvl.10.12 pmid:21731577
    CrossRefPubMed
  4. 4.↵
    1. Major EO,
    2. Amemiya K,
    3. Tornatore CS, et al
    . Pathogenesis and molecular biology of progressive multifocal leukoencephalopathy, the JC virus-induced demyelinating disease of the human brain. Clin Microbiol Rev 1992;5:49–73 doi:10.1128/cmr.5.1.49 pmid:1310438
    Abstract/FREE Full Text
  5. 5.↵
    1. Brooks BR,
    2. Walker DL
    . Progressive multifocal leukoencephalopathy. Neurol Clin 1984;2:299–313 doi:10.1016/S0733-8619(18)31105-8
    CrossRefPubMedWeb of Science
  6. 6.↵
    1. Berger JR,
    2. Houff S
    . Progressive multifocal leukoencephalopathy: lessons from AIDS and natalizumab. Neurol Res 2006;28:299–305 doi:10.1179/016164106X98198 pmid:16687057
    CrossRefPubMedWeb of Science
  7. 7.↵
    1. Ho PR,
    2. Koendgen H,
    3. Campbell N, et al
    . Risk of natalizumab-associated progressive multifocal leukoencephalopathy in patients with multiple sclerosis: a retrospective analysis of data from four clinical studies. Lancet Neurol 2017;16:925–33 doi:10.1016/S1474-4422(17)30282-X pmid:28969984
    CrossRefPubMed
  8. 8.↵
    1. Zaheer F,
    2. Berger JR
    . Treatment-related progressive multifocal leukoencephalopathy: current understanding and future steps. Ther Adv Drug Saf 2012;3:227–39 doi:10.1177/2042098612453849 pmid:25083238
    CrossRefPubMed
  9. 9.↵
    1. Gagne F,
    2. Bouchard JP,
    3. Bernier JP
    . Progressive multifocal leukoencephalopathy: observation with predominant pontocerebellar lesions and association with congenital immune deficiency. Acta Neuropathol 1977;38:167–69 doi:10.1007/BF00688566 pmid:878853
    CrossRefPubMed
  10. 10.↵
    1. Jones HR Jr.,
    2. Hedley-Whyte ET,
    3. Freidberg SR, et al
    . Primary cerebellopontine progressive multifocal leukoencephalopathy diagnosed premortem by cerebellar biopsy. Ann Neurol 1982;11:199–202 doi:10.1002/ana.410110218 pmid:7073254
    CrossRefPubMedWeb of Science
  11. 11.↵
    1. Hodel J,
    2. Darchis C,
    3. Outteryck O, et al
    . Punctate pattern: a promising imaging marker for the diagnosis of natalizumab-associated PML. Neurology 2016;86:1516–23 doi:10.1212/WNL.0000000000002586 pmid:27009257
    Abstract/FREE Full Text
  12. 12.↵
    1. Wattjes MP,
    2. Verhoeff L,
    3. Zentjens W, et al
    . Punctate lesion pattern suggestive of perivascular inflammation in acute natalizumab-associated progressive multifocal leukoencephalopathy: productive JC virus infection or preclinical PML-IRIS manifestation? J Neurol Neurosurg Psychiatry 2013;84:1176–77 doi:10.1136/jnnp-2013-304986 pmid:23695498
    FREE Full Text
  13. 13.↵
    1. Yadav R,
    2. Ramdas M,
    3. Karthik N, et al
    . “Hot cross bun” sign in HIV-related progressive multifocal leukoencephalopathy. Neurol India 2011;59:293–94 doi:10.4103/0028-3886.79149 pmid:21483137
    CrossRefPubMedWeb of Science
  14. 14.↵
    1. Padmanabhan S,
    2. Cherian A,
    3. Iype T, et al
    . Hot cross bun sign in HIV-related progressive multifocal leukoencephalopathy. Ann Indian Acad Neurol 2013;16:672–73 doi:10.4103/0972-2327.120479 pmid:24339604
    CrossRefPubMed
  15. 15.↵
    1. Lee YC,
    2. Liu CS,
    3. Wu HM, et al
    . The “hot cross bun” sign in the patients with spinocerebellar ataxia. Eur J Neurol 2009;16:513–16 doi:10.1111/j.1468-1331.2008.02524.x pmid:19187260
    CrossRefPubMed
  16. 16.↵
    1. Soares-Fernandes JP,
    2. Ribeiro M,
    3. Machado A
    . “Hot cross bun” sign in variant Creutzfeldt-Jakob disease. AJNR Am J Neuroradiol 2009;30:E37 doi:10.3174/ajnr.A1335 pmid:19279286
    CrossRefPubMed
  17. 17.↵
    1. Muqit MM,
    2. Mort D,
    3. Miskiel KA, et al
    . “Hot cross bun” sign in a patient with parkinsonism secondary to presumed vasculitis. J Neurol Neurosurg Psychiatry 2001;71:565–66 doi:10.1136/jnnp.71.4.565 pmid:11601421
    FREE Full Text
  18. 18.↵
    1. Berger JR,
    2. Aksamit AJ,
    3. Clifford DB, et al
    . PML diagnostic criteria: consensus statement from the AAN Neuroinfectious Disease Section. Neurology 2013;80:1430–38 doi:10.1212/WNL.0b013e31828c2fa1 pmid:23568998
    Abstract/FREE Full Text
  19. 19.↵
    1. Manto M,
    2. Gruol DL,
    3. Schmahmann J, et al
    . Handbook of the Cerebellum and Cerebellar Disorders. Vol 1. Springer-Verlag; 2013
  20. 20.↵
    1. Whiteman ML,
    2. Post MJ,
    3. Berger JR, et al
    . Progressive multifocal leukoencephalopathy in 47 HIV-seropositive patients: neuroimaging with clinical and pathologic correlation. Radiology 1993;187:233–40 doi:10.1148/radiology.187.1.8451420 pmid:8451420
    CrossRefPubMedWeb of Science
  21. 21.↵
    1. Shah R,
    2. Bag AK,
    3. Chapman PR, et al
    . Imaging manifestations of progressive multifocal leukoencephalopathy. Clin Radiol 2010;65:431–39 doi:10.1016/j.crad.2010.03.001 pmid:20451009
    CrossRefPubMed
  22. 22.↵
    1. Sahraian MA,
    2. Radue EW,
    3. Eshaghi A, et al
    . Progressive multifocal leukoencephalopathy: a review of the neuroimaging features and differential diagnosis. Eur J Neurol 2012;19:1060–69 doi:10.1111/j.1468-1331.2011.03597.x pmid:22136455
    CrossRefPubMed
  23. 23.↵
    1. Kuker W,
    2. Mader I,
    3. Nagele T, et al
    . Progressive multifocal leukoencephalopathy: value of diffusion-weighted and contrast-enhanced magnetic resonance imaging for diagnosis and treatment control. Eur J Neurol 2006;13:819–26 doi:10.1111/j.1468-1331.2006.01362.x pmid:16879291
    CrossRefPubMedWeb of Science
  24. 24.↵
    1. Bergui M,
    2. Bradac GB,
    3. Oguz KK, et al
    . Progressive multifocal leukoencephalopathy: diffusion-weighted imaging and pathological correlations. Neuroradiology 2004;46:22–25 doi:10.1007/s00234-003-1115-9 pmid:14593446
    CrossRefPubMedWeb of Science
  25. 25.↵
    1. Arai Y,
    2. Tsutsui Y,
    3. Nagashima K, et al
    . Autopsy case of the cerebellar form of progressive multifocal leukoencephalopathy without immunodeficiency. Neuropathology 2002;22:48–56 doi:10.1046/j.0919-6544.2001.00424.x pmid:12030415
    CrossRefPubMed
  26. 26.↵
    1. Boster A,
    2. Hreha S,
    3. Berger JR, et al
    . Progressive multifocal leukoencephalopathy and relapsing-remitting multiple sclerosis: a comparative study. Arch Neurol 2009;66:593–99 doi:10.1001/archneurol.2009.31 pmid:19433659
    CrossRefPubMedWeb of Science
  27. 27.↵
    1. Ryschkewitsch CF,
    2. Jensen PN,
    3. Monaco MC, et al
    . JC virus persistence following progressive multifocal leukoencephalopathy in multiple sclerosis patients treated with natalizumab. Ann Neurol 2010;68:384–91 doi:10.1002/ana.22137 pmid:20818792
    CrossRefPubMedWeb of Science
  28. 28.↵
    1. Ikeda J,
    2. Matsushima A,
    3. Ishii W, et al
    . Brain biopsy is more reliable than the DNA test for JC virus in cerebrospinal fluid for the diagnosis of progressive multifocal leukoencephalopathy. Intern Med 2017;56:1231–34 doi:10.2169/internalmedicine.56.7689 pmid:28502942
    CrossRefPubMed
  29. 29.↵
    1. Marzocchetti A,
    2. Di Giambenedetto S,
    3. Cingolani A, et al
    . Reduced rate of diagnostic positive detection of JC virus DNA in cerebrospinal fluid in cases of suspected progressive multifocal leukoencephalopathy in the era of potent antiretroviral therapy. J Clin Microbiol 2005;43:4175–77 doi:10.1128/JCM.43.8.4175-4177.2005 pmid:16081969
    Abstract/FREE Full Text
  30. 30.↵
    1. De Luca A,
    2. Giancola ML,
    3. Ammassari A, et al
    . The effect of potent antiretroviral therapy and JC virus load in cerebrospinal fluid on clinical outcome of patients with AIDS-associated progressive multifocal leukoencephalopathy. J Infect Dis 2000;182:1077–83 doi:10.1086/315817 pmid:10979902
    CrossRefPubMedWeb of Science
  31. 31.↵
    1. Giudici B,
    2. Vaz B,
    3. Bossolasco S, et al
    . Highly active antiretroviral therapy and progressive multifocal leukoencephalopathy: effects on cerebrospinal fluid markers of JC virus replication and immune response. Clin Infect Dis 2000;30:95–99 doi:10.1086/313598 pmid:10619739
    CrossRefPubMedWeb of Science
  32. 32.↵
    1. Marshall LJ,
    2. Major EO
    . Molecular regulation of JC virus tropism: insights into potential therapeutic targets for progressive multifocal leukoencephalopathy. J Neuroimmune Pharmacol 2010;5:404–17 doi:10.1007/s11481-010-9203-1 pmid:20401541
    CrossRefPubMed
  33. 33.↵
    1. Hagerman PJ,
    2. Hagerman RJ
    . Fragile X-associated tremor/ataxia syndrome. Ann N Y Acad Sci 2015;1338:58–70 doi:10.1111/nyas.12693 pmid:25622649
    CrossRefPubMed
  34. 34.↵
    1. Koralnik IJ,
    2. Wuthrich C,
    3. Dang X, et al
    . JC virus granule cell neuronopathy: a novel clinical syndrome distinct from progressive multifocal leukoencephalopathy. Ann Neurol 2005;57:576–80 doi:10.1002/ana.20431 pmid:15786466
    CrossRefPubMedWeb of Science
  35. 35.↵
    1. Willott RH,
    2. Sunman W,
    3. Munshi SK
    . Progressive multifocal leukoencephalopathy masquerading as cerebellar infarction. Age Ageing 2016;45:564–65 doi:10.1093/ageing/afw052 pmid:27056083
    CrossRefPubMed
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Cite this article
N. Adra, A.E. Goodheart, O. Rapalino, P. Caruso, S.S. Mukerji, R.G. González, N. Venna, J.D. Schmahmann
MRI Shrimp Sign in Cerebellar Progressive Multifocal Leukoencephalopathy: Description and Validation of a Novel Observation
American Journal of Neuroradiology Jun 2021, 42 (6) 1073-1079; DOI: 10.3174/ajnr.A7145

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MRI Shrimp Sign in Cerebellar Progressive Multifocal Leukoencephalopathy: Description and Validation of a Novel Observation
N. Adra, A.E. Goodheart, O. Rapalino, P. Caruso, S.S. Mukerji, R.G. González, N. Venna, J.D. Schmahmann
American Journal of Neuroradiology Jun 2021, 42 (6) 1073-1079; DOI: 10.3174/ajnr.A7145
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