A young woman presents with migraine-like headaches, black spots in her vision and emotional lability. Think you know the diagnosis? You may be surprised – this medical mystery is not what it seems. In this episode, learn more about this rare diagnosis, and gain clinical insight from neurologist Devon Conway, MD, and ophthalmologist Arthi Venkat, MD.

• Intro :11
• A 24-year-old woman presents with gradual worsening of a migraine-like headache accompanied by nausea :22
  • Description of symptoms :37
  • ER visit 1:23
  • Onset of new symptoms 1:44
  • Second ER visit 2:11
  • Outpatient MRI findings 2:28
  • Lumbar puncture findings 2:50
  • Patient is admitted to hospital 3:52
  • Second MRI findings 4:16
  • Ophthalmology consult and results of fluorescein angiography 4:46
  • Audiogram is ordered 5:17
  • Case summary 5:40
  • What is the most likely diagnosis? 6:05
• Triad of Susac syndrome 6:27
• Remember: This is not a vasculitis 7:11
• History of Susac syndrome 7:40
• CNS manifestations of the Susac triad 8:27
• Consult with Devon Conway, MD 10:13
  • Flare signals for non-radiologists/neurologists 11:05
  • Anything specific that says "demyelinating" on MRI? 13:35
  • Distinguishing the lesions in this case from those of MS 15:36
  • Neurology work-up approach to this type of case 16:28
  • The utility of lumbar puncture in this situation 18:05
  • About ADEM (acute disseminated encephalomyelitis) 21:06
• Ocular manifestations of Susac syndrome 22:26
• Consult with Arthi Venkat, MD 23:17
  • Ophthalmology differential in a patient with intermittent vision loss in various fields of vision 23:55
  • Overview of branch retinal artery occlusions 25:22
  • Difference between branch retinal artery occlusions and other ophthalmologic presentations common to rheumatologists 27:51
  • How the ophthalmologic exam helps differentiate MS from Susac syndrome 30:07
• Overview of sensorineural hearing loss in Susac syndrome 34:09
• Do we know for sure this is an autoimmune disease? 34:59
• Aggressive immunosuppression seems to halt the progression of disease 35:34
• What are treatment options? 35:42
• What do we know about long-term outcomes? 36:31
• Who takes care of these patients? 37:29
• Treatment, outcome of this patient 38:07
• Episode/case summary 38:20

Devon Conway, MD, is a staff neurologist at Cleveland Clinic's Mellen Center for Multiple Sclerosis.

Arthi Venkat, MD, is a retinal and uveitis specialist at Cole Eye Institute at Cleveland Clinic.

We'd love to hear from you! Send your comments/questions to [email protected].

References:

Aubart-Cohen F, et al. Medicine (Baltimore). 2007;86:93-102.

Greco A, et al. Autoimmun Rev. 2014;13:814-821.

Rennebohm RM, et al. Int J Stroke. 2018;doi:10.1177/1747493017751737.