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Susac Syndrome with Frontal Intermittent Rhythmic Delta Activity (FIRDA)

Published online by Cambridge University Press:  02 December 2014

Danielle Woolridge ,
Mark Stefanelli  and
Barbara Hoppe
Danielle Woolridge*
Affiliation:
Memorial University of Newfoundland, St. John's, NL, Canada
Mark Stefanelli
Affiliation:
Memorial University of Newfoundland, St. John's, NL, Canada
Barbara Hoppe
Affiliation:
Memorial University of Newfoundland, St. John's, NL, Canada
*
Memorial University of Newfoundland, 10 Larner St., St. John’s, Newfoundland, A1A 5S7, Canada
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Abstract

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Background:

Susac syndrome is a rare condition involving the brain, retina, and cochlea. Electroencephalogram (EEG) findings from published case reports show mainly generalized slowing.

Case report:

A 30-year-old man presented with acute onset of superior vision loss, unsteady gait, and hearing loss. This was accompanied by short-term memory loss and behavioral and mood changes. MRI showed multiple white matter hyperintensities. The EEG showed frontal intermittent rhythmic delta activity. A diagnosis of Susac syndrome was made and treatment with methylprednisolone, ASA, and Nimodipine was instituted. At one-year follow-up, bilateral hearing loss, mild visual impairment, and mood changes were still apparent.

Conclusion:

Frontal intermittent rhythmic delta activity can be seen in Susac syndrome.

Résumé:

RÉSUMÉ: Contexte:

Le syndrome de Susac est une maladie rare du cerveau, de la rétine et du limaçon. L’électroencéphalogramme (ÉEG) démontre surtout un ralentissement généralisé chez les cas qui ont été publiés.

Observation:

Un homme de 30 ans a consulté pour une perte de vision aiguë dans le champ de vision supérieur, un déséquilibre à la marche et une perte auditive. De plus, il accusait une diminution de la mémoire à court terme et des changements de comportement et d’humeur. L’IRM a montré de multiples zones d’hyperintensité dans la substance blanche. L’ÉEG a montré une activité delta rythmique intermittente au niveau frontal. Le diagnostic de syndrome de Susac a été posé et le patient a été traité par la méthylprednisolone, l’ASA et la nimodipine. Au suivi, une perte auditive bilatérale, une légère perte de vision et des changements d’humeur étaient encore présents un an plus tard.

Conclusions:

Une activité rythmique intermittente frontale peut être observée dans le syndrome de Susac.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 33 , Issue 4 , November 2006 , pp. 403 - 406
Copyright
Copyright © The Canadian Journal of Neurological 2006

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