Susac syndrome with frontal intermittent rhythmic delta activity (FIRDA).

Abstract

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.

CONCLUSIONS:

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

• Authors: Danielle Woolridge, Mark Stefanelli, Barbara Hoppe
• Journal: The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques (Can J Neurol Sci) Vol. 33 Issue 4 Pg. 403-6 (Nov 2006) ISSN: 0317-1671 [Print] England
• PMID: 17168166 (Publication Type: Case Reports, Journal Article)

Chemical References

• Calcium Channel Blockers
• Neuroprotective Agents
• lysine- salycilate
• Nimodipine
• Lysine
• Methylprednisolone

Topics

• Adult
• Brain Diseases (diagnosis, drug therapy, physiopathology)
• Calcium Channel Blockers (therapeutic use)
• Delta Rhythm
• Gait Disorders, Neurologic (etiology)
• Hearing Loss (etiology)
• Humans
• Lysine (analogs & derivatives, therapeutic use)
• Magnetic Resonance Imaging
• Male
• Methylprednisolone (therapeutic use)
• Neuroprotective Agents (therapeutic use)
• Nimodipine (therapeutic use)
• Retinal Artery Occlusion (etiology)
• Vision Disorders (etiology)