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Diagnostic imaging in 13 cases of Rasmussen's encephalitis: can early MRI suggest the diagnosis?

  • Paediatric Neuroradiology
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Abstract

Rasmussen's encephalitis (RE) is a rare, progressive, chronic encephalitis characterised by drug-resistant epilepsy, progressive hemiparesis and mental impairment. It typically involves only one cerebral hemisphere, which becomes atrophic. We present neuroradiological findings in 13 children with RE. MRI was performed in all patients, fluorodeoxyglucose positron-emission tomography (PET) in three, Tc-99m hexamethylpropylenamine oxime single-photon emission computed tomography (SPECT) in two and proton MR spectroscopy (1HMRS) in two. MRI showed progression of the hemisphere atrophy, always prevalent in the region primarily involved (13 patients), spread of the abnormal signal in white matter (11) and cortex (10) and progression of atrophy of the head of the caudate nucleus (nine). Associated secondary changes were: atrophy of the contralateral cerebellar hemisphere (in four patients), the ipsilateral hippocampus (in five) and the brain stem (in five). The earliest CT and MRI abnormalities, seen between 1 day and 4 months after the first seizure (in 12 patients examined, nine of whom had MRI) in one cerebral hemisphere included: high signal on T2-weighted images in the cortex (seven patients) and white matter (nine), cortical atrophy usually involving the frontoinsular region, with mild or severe enlargement of the lateral ventricle (eight) and moderate atrophy of the head of the caudate nucleus (seven). Cortical swelling in the early stage of the disease was recognisable only in two patients. PET revealed hypometabolism, SPECT decreased perfusion, and 1HMRS reduction of N-acetylaspartate in the affected hemisphere. PET and SPECT were usually performed in the late stages and did not provide specific findings. MRI thus demonstrates the progression of RE and may suggest the diagnosis in the early stages, often before the appearance of neurological deficits. Early diagnosis of RE may be crucial for selecting patients for aggressive medical therapy or major surgical interventions such as hemispherectomy.

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Acknowledgements

This research was supported by a grant from the Mariani Foundation. The study group on Rasmussen's Encephalitis was constituted under the auspices of the Italian League against Epilepsy (LICE).

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Authors and Affiliations

  1. Department of Neuroradiology, Istituto Nazionale Neurologico "C. Besta", Via Celoria 11, 20133, Milano, Italy

    L. Chiapparini, L. Farina, E. Ciceri, A. Erbetta & M. Savoiardo

  2. Department of Paediatric Neurology, Istituto Nazionale Neurologico "C. Besta", Milano, Italy

    T. Granata, F. Ragona & E. Freri

  3. Department of Paediatric Neurology, Ospedale Pediatrico Bambino Gesù, Roma, Italy

    L. Fusco

  4. Department of Paediatric Neurology, Ospedale Maggiore Pizzardi, Bologna, Italy

    G. Gobbi

  5. Department of Paediatric Neurology, Ospedale Poma, Mantova, Italy

    G. Capovilla

  6. Department of Epilepsy Surgery, Ospedale Niguarda Ca' Granda, Milano, Italy

    L. Tassi

  7. Epilepsy Centre, Ospedale Fatebenefratelli-Oftalmico, Milano, Italy

    M. Viri

  8. Department of Paediatric Neurology, Ospedale Civile, Brescia, Italy

    L. Giordano

  9. Department of Paediatric Neurology, Policlinico Borgo Roma, Verona, Italy

    B. Dalla Bernardina

  10. Department of Neurophysiology, Istituto Nazionale Neurologico "C. Besta", Milano, Italy

    R. Spreafico

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  1. L. Chiapparini
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  2. T. Granata
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  3. L. Farina
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  4. E. Ciceri
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  5. A. Erbetta
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  6. F. Ragona
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  7. E. Freri
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  8. L. Fusco
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  9. G. Gobbi
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  10. G. Capovilla
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  11. L. Tassi
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  13. M. Viri
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  14. B. Dalla Bernardina
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  15. R. Spreafico
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  16. M. Savoiardo
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Correspondence to L. Chiapparini.

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Chiapparini, L., Granata, T., Farina, L. et al. Diagnostic imaging in 13 cases of Rasmussen's encephalitis: can early MRI suggest the diagnosis?. Neuroradiology 45, 171–183 (2003). https://doi.org/10.1007/s00234-002-0923-7

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  • DOI: https://doi.org/10.1007/s00234-002-0923-7

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