Lesion morphology at 7 Tesla MRI differentiates Susac syndrome from multiple sclerosis

Abstract

Background:

Although an orphan disease with still obscure aetiopathogenesis, Susac syndrome has to be considered as differential diagnosis in multiple sclerosis (MS), since its clinical presentation and paraclinical features including routine magnetic resonance imaging (MRI) findings partially overlap.

Objective:

We aimed to study a potential benefit of 7T MRI for (i) the differentiation between Susac syndrome and MS and (ii) the clarification of pathogenesis of Susac syndrome.

Methods:

Five patients suffering from Susac syndrome, 10 sex- and age-matched patients with relapsing–remitting MS (median Expanded Disability Status Scale (EDSS) score 1.5) and 15 matching healthy controls were investigated at 7 Tesla MRI. The protocol included T1-weighted MPRAGE, T2*-weighted FLASH, and TIRM sequences.

Results:

Almost all T2* FLASH lesions in patients with MS were centred by a small central vein (325 lesions; 92%) and often showed a small hypointense rim (145 lesions; 41%). In contrast, white matter lesions in Susac syndrome exhibited a perivascular setting significantly less frequently (148 lesions; 54%, p=0.002), and very rarely exhibited a hypointense rim (12 lesions; 4%, p=0.004). Furthermore, in addition to callosal atrophy, Susac patients showed cerebrospinal fluid-isointense lesions within the central part of corpus callosum that are not commonly seen in MS.

Conclusion:

At 7T MRI, plaques in MS patients and patients with Susac syndrome differed substantially with respect to morphology and pattern. Thus, lesion morphology at 7T (i) may serve as a marker to distinguish Susac syndrome from MS and (ii) reflects a different pathophysiological mechanism underlying Susac syndrome, for example microinfarction rather than demyelination.

Keywords

Susac syndrome multiple sclerosis ultrahigh field magnetic resonance imaging differential diagnosis

Get full access to this article

View all access options for this article.

References

Susac

Hardman

Selhorst

. Microangiopathy of the brain and retina. Neurology 1979; 29: 313–316.

Susac

. Susac’s syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology 1994; 44: 591–593.

Dorr

Radbruch

Bock

. Encephalopathy, visual disturbance and hearing loss-recognizing the symptoms of Susac syndrome. Nat Rev Neurol 2009; 5: 683–688.

Saux

Niango

Charif

. Susac’s syndrome, a rare, potentially severe or lethal neurological disease. J Neurol Sci 2010; 297: 71–73.

Susac

Egan

Rennebohm

. Susac’s syndrome: 1975-2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci 2007; 257: 270–272.

Susac

Murtagh

Egan

. MRI findings in Susac’s syndrome. Neurology 2003; 61: 1783–1787.

Rennebohm

Susac

Egan

. Susac’s Syndrome – update. J Neurol Sci 2010; 299: 86–91.

Kleffner

Deppe

Mohammadi

. Diffusion tensor imaging demonstrates fiber impairment in Susac syndrome. Neurology 2008; 70: 1867–1869.

Kleffner

Deppe

Mohammadi

. Neuroimaging in Susac’s syndrome: focus on DTI. J Neurol Sci 2010; 299: 92–96.

10.

Dorr

Jarius

Wildemann

. [Susac syndrome: An interdisciplinary challenge.]. Nervenarzt 2011; 82: 1250–1263.

11.

Rennebohm

Egan

Susac

. Treatment of Susac’s Syndrome. Curr Treat Options Neurol 2008; 10: 67–74.

12.

Sinnecker

Mittelstaedt

Dörr

. Multiple Sclerosis Lesions and Irreversible Brain Tissue Damage: A Comparative Ultrahigh-Field Strength Magnetic Resonance Imaging Study. Arch Neurol. Epub ahead of print 20 February 2011. DOI: 10.1001/archneurol.2011.245010.1001/archneurol.2011.2450.

13.

Zohrabian

Grossman

. Seven-Tesla magnetic resonance imaging: new vision of microvascular abnormalities in multiple sclerosis. Arch Neurol 2008; 65: 812–816.

14.

Kollia

Maderwald

Putzki

. First clinical study on ultra-high-field MR imaging in patients with multiple sclerosis: comparison of 1.5T and 7T. AJNR Am J Neuroradiol 2009; 30: 699–702.

15.

Tallantyre

Morgan

Dixon

. A comparison of 3T and 7T in the detection of small parenchymal veins within MS lesions. Invest Radiol 2009; 44: 491–494.

16.

Tallantyre

Brookes

Dixon

. Demonstrating the perivascular distribution of MS lesions in vivo with 7-Tesla MRI. Neurology 2008; 70: 2076–2078.

17.

Pitt

Boster

Pei

. Imaging cortical lesions in multiple sclerosis with ultra-high-field magnetic resonance imaging. Arch Neurol 2010; 67: 812–818.

18.

Hermann

Hansen

Seidenberg

. Neurodevelopmental vulnerability of the corpus callosum to childhood onset localization-related epilepsy. Neuroimage 2003; 18: 284–292.

19.

Dixon

Simpson

Mistry

. Optimisation of T(2)(*)-weighted MRI for the detection of small veins in multiple sclerosis at 3T and 7T. Eur J Radiol. Epub ahead of print 02 December 2011. DOI: 10.1016/j.ejrad.2011.09.02310.1016/j.ejrad.2011.09.023.

20.

Suzuki

Kudo

Sasaki

. Detection of active plaques in multiple sclerosis using susceptibility-weighted imaging: comparison with gadolinium-enhanced MR imaging. Magn Reson Med Sci 2011; 10: 185–192.

21.

Tallantyre

Dixon

Donaldson

. Ultra-high-field imaging distinguishes MS lesions from asymptomatic white matter lesions. Neurology 2011; 76: 534–539.

22.

Rennebohm

Susac

. Treatment of Susac’s syndrome. J Neurol Sci 2007; 257: 215–220.

23.

Yaldizli

Glassl

Sturm

. Fatigue and progression of corpus callosum atrophy in multiple sclerosis. J Neurol 2011; 258: 2199–2205.

24.

Ozturk

Smith

Gordon-Lipkin

. MRI of the corpus callosum in multiple sclerosis: association with disability. Mult Scler 2010; 16: 166–177.

25.

Larson

Burnison

Brown

. Callosal function in multiple sclerosis: bimanual motor coordination. Cortex 2002; 38: 201–214.

26.

Evangelou

Konz

Esiri

. Regional axonal loss in the corpus callosum correlates with cerebral white matter lesion volume and distribution in multiple sclerosis. Brain 2000; 123: 1845–1849.

27.

Peterson

Mork

. Transected neurites, apoptotic neurons, and reduced inflammation in cortical multiple sclerosis lesions. In: Ann Neurol 2001; 50: 389–400.

28.

Sati

George

Shea

. FLAIR*: a new MRI contrast for visualizing perivascular distribution of multiple sclerosis lesions. In: 5th joint triennial congress of the European and Americas committees for treatment and research in multiple sclerosis, Amsterdam, the Netherlands, 19–22 October 2011, paper no. 91.