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Pseudotumoral hemicerebellitis as a mimicker of Lhermitte-Duclos disease in children: does neuroimaging help to differentiate them?

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Abstract

The clinical presentation and neuroimaging findings of children with pseudotumoral hemicerebellitis (PTHC) and Lhermitte-Duclos disease (LDD) may be very similar. The differentiation between these entities, however, is important because their management and prognosis are different. We report on three children with PTHC. For all three children, in the acute situation, the differentiation between PTHC and LDD was challenging. A review of the literature shows that a detailed evaluation of conventional and neuroimaging data may help to differentiate between these two entities. A striated folial pattern, brainstem involvement, and prominent veins surrounding the thickened cerebellar foliae on susceptibility weighted imaging favor LDD, while post-contrast enhancement and an increased choline peak on 1H-Magnetic resonance spectroscopy suggest PTHC.

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Authors’ contributions

TB and AP conceptualized and designed the report; all the co-authors participated in the acquisition and interpretation of the clinical and neuroimaging data; TB drafted the manuscript; and all the co-authors critically revised the manuscript for intellectual content and read and approved the final manuscript.

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Authors and Affiliations

  1. Section of Pediatric Neuroradiology, Division of Pediatric Radiology, Russell H. Morgan Department of Radiology and Radiological Science, The Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Thangamadhan Bosemani, Thierry A. G. M. Huisman & Andrea Poretti

  2. Department of Pediatric Neurology, University Children’s Hospital, Bern, Switzerland

    Maja Steinlin

  3. Department of Pediatric Neurology, University Children’s Hospital, Zurich, Switzerland

    Sandra P. Toelle & Eugen Boltshauser

  4. Department of Neurosurgery, University Hospital, Bern, Switzerland

    Jürgen Beck

Authors
  1. Thangamadhan Bosemani
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  2. Maja Steinlin
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  3. Sandra P. Toelle
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  4. Jürgen Beck
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  5. Eugen Boltshauser
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  6. Thierry A. G. M. Huisman
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  7. Andrea Poretti
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Correspondence to Andrea Poretti.

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Bosemani, T., Steinlin, M., Toelle, S.P. et al. Pseudotumoral hemicerebellitis as a mimicker of Lhermitte-Duclos disease in children: does neuroimaging help to differentiate them?. Childs Nerv Syst 32, 865–871 (2016). https://doi.org/10.1007/s00381-015-2977-y

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  • DOI: https://doi.org/10.1007/s00381-015-2977-y

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