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

Research ArticlePediatric Neuroimaging

Neuroimaging Features of Ectopic Cerebellar Tissue: A Case Series Study of a Rare Entity

G. Orman, S.F. Kralik, R. Battini, B. Buchignani, N.K. Desai, R. Goetti, A. Meoded, C. Mitter, B. Wallacher-Scholz, E. Boltshauser and T.A.G.M. Huisman
American Journal of Neuroradiology June 2021, 42 (6) 1167-1173; DOI: https://doi.org/10.3174/ajnr.A7105
G. Orman
aFrom the Edward B. Singleton Department of Radiology (G.O., S.F.K., N.K.D., A.M., T.A.G.M.H.), Texas Children's Hospital, Houston, Texas
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S.F. Kralik
aFrom the Edward B. Singleton Department of Radiology (G.O., S.F.K., N.K.D., A.M., T.A.G.M.H.), Texas Children's Hospital, Houston, Texas
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R. Battini
bDepartment of Developmental Neuroscience (R.B.), Istituto di Ricovero e Cura a Carattere Scientifico Fondazione Stella Maris, Pisa, Italy
cDepartment of Clinical and Experimental Medicine (R.B., B.B.), University of Pisa, Pisa, Italy
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B. Buchignani
cDepartment of Clinical and Experimental Medicine (R.B., B.B.), University of Pisa, Pisa, Italy
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N.K. Desai
aFrom the Edward B. Singleton Department of Radiology (G.O., S.F.K., N.K.D., A.M., T.A.G.M.H.), Texas Children's Hospital, Houston, Texas
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R. Goetti
dDepartment of Medical Imaging (R.G.), The Children's Hospital at Westmead, The University of Sydney, Sydney, Australia
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A. Meoded
aFrom the Edward B. Singleton Department of Radiology (G.O., S.F.K., N.K.D., A.M., T.A.G.M.H.), Texas Children's Hospital, Houston, Texas
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C. Mitter
eDepartment of Biomedical Imaging and Image-Guided Therapy (C.M.), Medical University of Vienna, Vienna, Austria
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B. Wallacher-Scholz
fDepartment of Pediatric Neurology and Developmental Medicine and LMU Center for Children with Medical Complexity (B.W.-S.), Dr. von Hauner Children's Hospital, LMU University Hospital, Ludwig-Maximilians-Universität, Munich, Germany
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E. Boltshauser
gDepartment of Pediatric Neurology (E.B.), University Children's Hospital Zürich, Zurich, Switzerland
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T.A.G.M. Huisman
aFrom the Edward B. Singleton Department of Radiology (G.O., S.F.K., N.K.D., A.M., T.A.G.M.H.), Texas Children's Hospital, Houston, Texas
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  • FIG 1.
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    FIG 1.

    Axial T2- (A) and T1- (B) weighted, DWI (C), ADC (D), coronal T2-weighted (E), contrast-enhanced coronal T1-weighted (F), and sagittal pre- and postcontrast enhanced T1-weighted (G and H) MR images and map of patient 1 at 17 years of age show well-defined, ectopic, cerebellar tissue (A–H, arrows) in the suprasellar region. Note that the signal intensity is similar to that of the cerebellum in all sequences, and there is no contrast enhancement on postcontrast imaging.

  • FIG 2.
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    FIG 2.

    Coronal (A–C) and axial (D–F) T2-weighted and sagittal T1-weighted (G) MR images of patient 2 at 25 months of age show triangular-shaped ectopic cerebellar tissue on consecutive slices (A–F, arrows). Note the thin stalk connecting the ectopic cerebellar tissue with the brain stem (G, arrow).

  • FIG 3.
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    FIG 3.

    Axial T1- (A and F) and T2- (B and G) weighted, axial DWI (C and H), ADC (D and I), and coronal T2- (E) and T1- (J) weighted MR images and maps of patient 3 at 13 years of age (upper row, arrows) and 18 years of age (lower row, arrows). The patient had a history of global developmental delay with intellectual disability, right sensorineural hearing loss, and simple febrile seizures. MR imaging shows well-defined ectopic cerebellar tissue in the right cerebellopontine angle (A–D, arrows), which was stable on follow-up MR imaging (E–H, arrows). Note the thin stalk connecting the ectopic cerebellar tissue with the middle cerebellar peduncle (E and J, arrows).

  • FIG 4.
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    FIG 4.

    Coronal (A–D) and sagittal T2-weighted (E–G) MR imaging of patient 4 at 32 months of age shows a distorted/dysmorphic superior portion of the right cerebellar hemisphere, which also shows folial and sulcal anomalies. The dysmorphic superior portion also displaces the adjacent vermis. Ectopic cerebellar tissue (A–G, arrows) is connected to the cerebellum. Its MR signal is isointense with the cerebellum, and its morphology is elongated with irregular borders. Additional brain malformations included temporal lobe dysgyria and malrotated hippocampi (not shown).

  • FIG 5.
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    FIG 5.

    Follow-up coronal and sagittal T1-weighted MR imaging of patient 4 at 6 years of age shows a stable imaging pattern of the ECT (arrows) with respect to the already-described findings in Fig 4.

  • FIG 6.
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    FIG 6.

    Sagittal T1- (upper row) and T2-weighted (lower row) MR images of patient 5 at 3 years of age show a thin stalk arising from the pontomesencephalic junction below the right inferior colliculus dorsolaterally, which extends to triangular-shaped ectopic cerebellar tissue (A–F, arrows) superior to the right anterior cerebellar lobe, with an arachnoid cyst (A–F, asterisks) posteriorly. The sagittal oblique reformat T1-weighted MR imaging shows the continuous stalk (G, arrows) between the triangular-shaped ECT and the brain stem. The findings were unchanged 1 year later (not shown).

  • FIG 7.
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    FIG 7.

    Sagittal T1-weighted (A), sagittal FLAIR (B), sagittal T2-weighted (C), sagittal contrast-enhanced T1-weighted (D), axial T2-weighted (E and F), and coronal T1-weighted (G and H) MR images of patient 6 at 17 months of age show ECT connected with the brain stem by a thin stalk (A–H, arrows). Most interesting, the MR imaging appearance of ectopic cerebellar tissue in this patient is almost identical to the MR imaging findings of patient 2.

  • FIG 8.
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    FIG 8.

    Postmortem sagittal (A) and axial (B) T2-weighted fetal MR images at the gestational age of 22 weeks 5 days and a matching macroscopic postmortem image (C) of the brain show an incidental finding (A and B, arrows) of a small appendage with a ribbed surface located superior to the left cerebellar hemisphere, originating from the left dorsal pontomesencephalic junction. Histologic analysis (D) reveals that this structure is of cerebellar origin with distinguishable external granular and molecular layers and developing Purkinje cells and an internal granular layer. Adapted with permission.23

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American Journal of Neuroradiology: 42 (6)
American Journal of Neuroradiology
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Cite this article
G. Orman, S.F. Kralik, R. Battini, B. Buchignani, N.K. Desai, R. Goetti, A. Meoded, C. Mitter, B. Wallacher-Scholz, E. Boltshauser, T.A.G.M. Huisman
Neuroimaging Features of Ectopic Cerebellar Tissue: A Case Series Study of a Rare Entity
American Journal of Neuroradiology Jun 2021, 42 (6) 1167-1173; DOI: 10.3174/ajnr.A7105

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Neuroimaging Features of Ectopic Cerebellar Tissue: A Case Series Study of a Rare Entity
G. Orman, S.F. Kralik, R. Battini, B. Buchignani, N.K. Desai, R. Goetti, A. Meoded, C. Mitter, B. Wallacher-Scholz, E. Boltshauser, T.A.G.M. Huisman
American Journal of Neuroradiology Jun 2021, 42 (6) 1167-1173; DOI: 10.3174/ajnr.A7105
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