Sneddon syndrome – imaging findings

Síndrome de Sneddon – achados de imagem

The Sneddon syndrome is a rare clinical syndrome that associates strokes and livedo, etiologically related with the antiphospholipid antibody syndrome (Figure 1)^1,2,3,4,5.

Figure 1
reticular livedo on limbs.

This 42 year-old woman with headache and right hemiparesis had a MRI that showed acute/ subacute ischemia in part of the territory of the left middle cerebral artery (MCA) and bi-hemispheric gliosis/ encephalomalacia (Figure 2). MR angiography showed normal cervical carotid and vertebral circulation, filling defect in M2 segment of the left MCA. Catheter angiography disclosed difuse cortical occlusive arteriopathy associated with a rich collateral cortical network. The M2 occlusion was reperfused, with infarct sign in its territory (Figures 3 and 4).

Figure 2
AXIAL FLAIR (A e B) showing areas corresponding to bi-hemispheric gliosis / encephalomalacia and area of acute/ subacute ischemic stroke in part of the territory of the left MCA. AXIAL DIFFUSION (C and D) proves acute / subacute schemic stroke area.

Figure 3
Angiography early arterial phase: (A) right side apparently normal; (B) slow filling area (red circle) and luxury perfusion (arrow).

Figure 4
Angiography capillary phase (A, B and C): occlusions of distal vessels + prominent collateral circulation (arrows), best visualized in the enlarged images (B and C).

References

• ¹
  Maamar M, Rahmani M, Aidi S, Benabdeljlil M, El HassaniMy R, Jiddane M et al. [Sneddon’s syndrome: 15 cases with cerebral angiography]. Rev Neurol (Paris). 2007;163(8-9):809-16. French. doi:10.1016/S0035-3787(07)91463-0
• ²
  Marinho JL, Piovesan EJ, Leite Neto MP, Kotze LR, Noronha L, Twardowschy CA et al. Clinical, neurovascular and neuropathological features in Sneddon’s syndrome. ArqNeuropsiquiatr. 2007;65(2B):390-5. doi:10.1590/S0004-282X2007000300005
• ³
  Boesch SM, Plörer AL, Auer AJ, Poewe W, Aichner FT, Felber SR et al. The natural course of Sneddon syndrome: clinical and magnetic resonance imaging findings in a prospective six year observation study. J Neurol Neurosug Psychiatry. 2003;74(4):542-4. doi:10.1136/jnnp.74.4.542
• ⁴
  Stockhammer G, Felber SR, Zelger B, Sepp N, Birbamer GG, Fritsch PO et al. Sneddon’s syndrome: diagnosis by skin biopsy and MRI in 17 patients. Stroke.1993;24(5):685-90. doi:10.1161/01.STR.24.5.685
• ⁵
  Uthman, I.W., Khamashta, M.A. Livedo racemosa: a striking dermatological sign for the antiphospholipid syndrome [Editorial]. J Rheumatol. 2006;33(12):2379-82.

Publication Dates

• Publication in this collection
  20 Oct 2015
• Date of issue
  Jan 2016

History

• Received
  11 July 2015
• Accepted
  04 Aug 2015