Susac syndrome with frontal intermittent rhythmic delta activity (FIRDA).
BACKGROUND
Susac syndrome is a rare condition involving the brain, retina, and cochlea. Electroencephalogram (EEG) findings from published case reports show mainly generalized slowing.


METHODS
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.
Woolridge
Danielle
D
Memorial University of Newfoundland, St. John's, NL, Canada.
Stefanelli
Mark
M
Hoppe
Barbara
B
D000328:Adult; D001927:Brain Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology*; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D003700:Delta Rhythm; D020233:Gait Disorders, Neurologic / Q000209:etiology; D034381:Hearing Loss / Q000209:etiology; D006801:Humans; D008239:Lysine / Q000031:analogs & derivatives / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D018696:Neuroprotective Agents / Q000627:therapeutic use; D009553:Nimodipine / Q000627:therapeutic use; D015356:Retinal Artery Occlusion / Q000209:etiology; D014786:Vision Disorders / Q000209:etiology
D002121:Calcium Channel Blockers; D018696:Neuroprotective Agents; C404803:lysine- salycilate; D009553:Nimodipine; D008239:Lysine; D008775:Methylprednisolone