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Focal brain dysfunction in a 41-year old man with familial alternating hemiplegia

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Abstract

The acute pathophysiologic changes during hemiplegic spells and the long-term outcome of alternating hemiplegia remain obscure. In a 41-year-old male with familial alternating hemiplegia we found an increase in right frontal cerebral blood flow 3 h into a 5-h left hemiplegic episode. A repeat high-resolution brain SPECT study performed 26 h after the resolution of the left hemiplegia revealed normalization of the frontal blood flow accompanied by hyperperfusion in the right parietal lobe. An interictal SPECT scan several weeks later showed no asymmetries. Head CT and MRI scans were negative. Neuropsychologic assessment and neurologic examination revealed evidence of a diffuse disorder which predominantly involved the right hemisphere. To our knowledge, there are no previous correlative studies of serial highresolution brain SPECT with MRI, or of detailed neuropsychologic assessment, in adult patients with such an advanced course of alternating hemiplegia of childhood.

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Author notes

  1. Mohamed A. Mikati

    Present address: Department of Pediatrics, American University of Beirut, New York Office, 853rd Avenue, 18th floor, 10022, New York, NY, USA

Authors and Affiliations

  1. Department of Neurology, Brigham and Women’s Hospital, Harvard Medical School, 221 Longwood Avenue LMRC 113, 02115, Boston, MA, USA

    Fernando Dangond

  2. Department of Nuclear Medicine, Brigham and Women’s Hospital, Harvard Medical School, 75 Francis Street, 02115, Boston, MA, USA

    Basem Garada & B. Leonard Holman

  3. Department of Psychology, Brigham and Women’s Hospital, Harvard Medical School, 75 Francis Street, 02115, Boston, MA, USA

    Benjamin J. Murawski

  4. Department of Neuropsychology, Children’s Hospital, Harvard Medical School, 300 Longwood Avenue, 02115, Boston, MA, USA

    Celiane Rey-Casserly

  5. Department of Neurology, Children’s Hospital, Harvard Medical School, 300 Longwod Avenue, 02115, Boston, MA, USA

    Mohamed A. Mikati

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  1. Fernando Dangond
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  2. Basem Garada
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  4. Celiane Rey-Casserly
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  5. B. Leonard Holman
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  6. Mohamed A. Mikati
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Dangond, F., Garada, B., Murawski, B.J. et al. Focal brain dysfunction in a 41-year old man with familial alternating hemiplegia. Eur Arch Psychiatry Clin Nuerosci 247, 35–41 (1997). https://doi.org/10.1007/BF02916251

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  • DOI: https://doi.org/10.1007/BF02916251

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