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

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-r...

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Bibliographic Details
Published in:European archives of psychiatry and clinical neuroscience 1997-02, Vol.247 (1), p.35-41
Main Authors: Dangond, F, Garada, B, Murawski, B J, Rey-Casserly, C, Holman, B L, Mikati, M A
Format: Article
Language:English
Subjects:
Adult
Brain
Dominance, Cerebral - genetics
Dominance, Cerebral - physiology
Frontal Lobe - blood supply
Frontal Lobe - diagnostic imaging
Hemiplegia - diagnostic imaging
Hemiplegia - genetics
Hemiplegia - physiopathology
Humans
Image Processing, Computer-Assisted
Magnetic Resonance Imaging
Male
Neuropsychological Tests
Orientation - physiology
Parietal Lobe - blood supply
Parietal Lobe - diagnostic imaging
Regional Blood Flow - physiology
Tomography, Emission-Computed, Single-Photon
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Summary: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 high-resolution brain SPECT with MRI, or of detailed neuropsychologic assessment, in adult patients with such an advanced course of alternating hemiplegia of childhood.
ISSN:0940-1334
1433-8491
DOI:10.1007/BF02916251