How is Susac's syndrome diagnosed?

A neurological exam may find that the patient has:

  • Poor memory, confusion, and poor thinking ability
  • Loss of balance
  • Difficulty walking
  • Slight paralysis or weakness
  • Overactive tendon reflexes
  • Poor muscle control

Magnetic resonance imaging (MRI) may find:

  • Abnormalities (lesions) in the central portion of the corpus callosum (the bundle of nerves that connects the right and left halves of the brain)
  • Other white matter lesions
  • Lesions in the deep gray matter
  • Enhancement (increased visibility) of the leptomeninges, the tissue that covers the brain

Fluorescein angiography looks at blood flow in the eye. Findings in Susac's syndrome include:

  • Visible white spots in the retina. These "cotton wool spots" are the result of injury due to disturbed blood flow.
  • Blockage of blood vessels in the retina
  • Blood vessel "leakage"
  • Increased "staining" of the walls of the vessels

A hearing test may find:

  • Various degrees of hearing loss, particularly of low-pitched sounds
  • Difficulty understanding speech

Because its symptoms are similar to those of a number of other diseases—such as multiple sclerosis, ADEM, encephalitis, meningitis, lupus, and CNS vasculitis—Susac's syndrome may be difficult to diagnose.

Last reviewed by a Cleveland Clinic medical professional on 05/26/2017.

References

  • Susac, JO. Editorial: Susac's Syndrome. Am J Neuroradiology 2004 25:351-352.
  • Genetic and Rare Diseases Information Center (GARD). Susac syndrome Accessed 5/26/2017.
  • Rennebohm R, Susac JO, Egan RA, Daroff RB. Susac's Syndrome--update. J Neurol Sci 2010 Dec 15;299(1-2):86-91. Epub 2010 Sep 19. PMID: 20855088 [PubMed - indexed for MEDLINE]
  • Rennebohm RM, Egan RA, Susac JO. Treatment of Susac's Syndrome. Curr Treat Options Neurol. 2008 Jan;10(1):67-74. PMID: 18325301
  • Susac JO, Egan RA, Rennebohm RM, Lubow M. Susac's syndrome: 1975-2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci 2007 Jun 15;257(1-2):270-2. Epub 2007 Feb 28. Review. PMID: 17331544 [PubMed - indexed for MEDLINE] [PubMed]
  • Rennebohm RM, Susac JO. Treatment of Susac's syndrome. J Neurol Sci 2007 Jun 15;257(1-2):215-20. Epub 2007 Feb 26. Review. PMID: 17324441 [PubMed - indexed for MEDLINE]
  • Rennebohm RM, Lubow M, Rusin J, Martin L, Grzybowski DM, Susac JO. Aggressive immunosuppressive treatment of Susac's syndrome in an adolescent: using treatment of dermatomyositis as a model. Pediatr Rheumatol Online J 2008 Jan 29;6:3. PMID: 18230188 [PubMed]

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