Hostname: page-component-586b7cd67f-2brh9 Total loading time: 0 Render date: 2024-11-27T23:33:16.275Z Has data issue: false hasContentIssue false
  • English
  • Français

Susac's Syndrome

Published online by Cambridge University Press:  02 December 2014

Kevin Lian ,
Rekha Siripurapu ,
Robert Yeung ,
Julia Hopyan ,
Kenneth T. Eng ,
Richard I. Aviv  and
Sean P. Symons
Kevin Lian
Affiliation:
Division of Neuroradiology, Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada
Rekha Siripurapu
Affiliation:
Division of Neuroradiology, Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada
Robert Yeung
Affiliation:
Division of Neuroradiology, Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada
Julia Hopyan
Affiliation:
Divison of Neurology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada
Kenneth T. Eng
Affiliation:
Department of Ophthalmology, Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada
Richard I. Aviv
Affiliation:
Division of Neuroradiology, Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada
Sean P. Symons*
Affiliation:
Division of Neuroradiology, Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada
*
Sunnybrook Health Sciences Centre, 2075 Bayview Avenue, AG31D, Toronto, Ontario, M4N 3M5, Canada
Rights & Permissions [Opens in a new window]

Extract

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

A 40-year-old woman with no significant previous medical history presented with a three month history of ataxia, confusion, memory difficulties, and headaches. Physical examination revealed numbness in the left hand, but was otherwise unremarkable. Magnetic resonance imaging fluid-attenuated inversion recovery (MRI FLAIR) images demonstrated multiple small white matter hyperintensities, including lesions involving the corpus callosum. There were also deep gray nuclei lesions (Figure 1). The corpus callosum lesions involved the central fibers (Figure 2). Post gadolinium T1 images demonstrated enhancement of some of the lesions as well as extensive perivascular and leptomeningeal enhancement (Figure 3). Extensive infectious serology, autoimmune panel, and paraneoplastic antibodies were negative. Lumbar puncture revealed elevated protein (1116 mg/L), but was otherwise normal. Brain biopsy indicated no apparent pathology. The patient was tentatively diagnosed with acute encephalopathy and treated with high dose steroids seven days after presentation. She was subsequently discharged and was sent for rehabilitation.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 38 , Issue 2 , March 2011 , pp. 335 - 337
Copyright
Copyright © The Canadian Journal of Neurological 2011

References

1Susac, JO, Egan, RA, Rennebohm, RM, Lubow, M.Susac’s syndrome: 1975-2005 microangiopathy / autoimmune endotheliopathy. J Neurol Sci. 2007; 257(1-2):270–2.Google Scholar
2Susac, JO.Susac’s syndrome. Am J Neuroradiol. 2004; 25(3):351–2.Google Scholar
3Susac, JO, Murtagh, FR, Egan, RA, et al.MRI findings in Susac’s syndrome. Neurology. 2003; 61(12):1783–7.CrossRefGoogle ScholarPubMed
4Do, TH, Fisch, C, Evoy, F.Susac syndrome: report of four cases and review of the literature. Am J Neuroradiol. 2004; 25(3):382–8.Google Scholar
5Ayache, D, Plouin-Gaudon, I, Bakouche, P, Elbaz, P, Gout, O.Microangiopathy of the inner ear, retina, and brain (Susac syndrome): report of a case. Arch Otolaryngol Head Neck Surg. 2000; 126(1):82–4.Google Scholar
6Petty, GW, Engel, AG, Younge, BR, et al.Retinocochleocerebral vasculopathy. Medicine (Baltimore). 1998; 77(1):12–40.Google Scholar