A pictorial review of neurological complications of systemic lupus erythematosus and antiphospholipid syndrome

A pictorial review of neurological complications of systemic lupus erythematosus and antiphospholipid syndrome Poster No.: C-2780 Congress: ECR 2010 Type: Educational Exhibit Topic: Neuro Authors: E. Tavernaraki, V. Ouranos, S. Benakis, K. Stefanidis, D. Chondros; Athens/GR Keywords: SLE, ANTIPHOSPHOLIPID SYNDROM, CNS DOI: 10.1594/ecr2010/C-2780 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. www.myesr.org Page 1 of 35

Learning objectives To illustrate the imaging appearances of neurological complications of systemic lupus erythematosus and antiphospholipid syndrome. To demonstrate the imaging features which will point the radiologist towards particular diagnosis. Background SLE is an autoimmune disease that is frequently manifested by involvement of central nervous system. CT remains valuable in identifying hemorrhages and larger infarcts. MRI is more useful in subtle ischemia, white matter lesions and spinal involvement. Common findings are: 1. 2. 3. 4. 5. 6. Infarcts cortical or subcortical due to vasculitis or embolic disease(increased tendency towards thrombosis related to antiphospholipid antibodies, Libman-Sacks endocarditis) or due to atherosclerosis accelerated by hypertension or long term steroid use. They appear on T2WI, high signal in white matter sometimes in a vascular distribution but also involving cortical and subcortical areas, particular in the occipital region. Such lesions have been reported in a symmetric distribution in young female patient with diffuse CNS lupus. White matter lesions (microinfarction, demyelination) not confined to periventricular white matter, favor gray-white junction. Focal areas of increased intensity primarily in gray-matter (cortex, deep nuclei). Cerebral atrophy due to steroid therapy or encephalopathy. Intracerebral hemorrhage due to renal hypertension or underlying venous thrombosis. Subarachnoid hemorrage as a long term anticoagulation. Page 2 of 35

Differential diagnosis includes: MS which involves periventricular white matter and lesions are radially oriented along white matter tracts (Fig 3,4). Tumefactive MS appears as mass with irregular, thick, partial ring enhancement (Fig 1,2). LYME encephalopathy which appears as hyperintense periventricular white matter lesions on T2WI, may enhance, resemble MS, ADEM. Small vessel disease caused by diabetes, hypertension, arteriolosclerosis. Appears as T2WI hyperintense lesions within deep gray matter (basal ganglia,thalamus), centrum semiovale or diffuse confluent regions of periventricular involvement (Fig.5). Susac syndrome (triad:encephalopathy, retinal artery branch involvement, hearing loss) appears as deep white matter, corpus callosus central lesions. Other vasculitides:primary angitis of CNS, PAN, Wegener, Bechet, syphilis, Sjogren (Fig. 6,7). HIV encephalitis which appear as high signal abnormalities on T2WI and FLAIR in white matter, caudate nuclei, basal ganglia and associated atrophy. No enhancement is seen (Fig. 8,9). Spinal involvement is termed secondary acute transverse myelitis. It appears as focal or diffuse hyperintense lesion on T2WI with mild cord expansion and with variable enhancement. Size can be segmental or multisegmental or holocord. Sometimes on T1WI may be mild hyperintensity due to petechial hemorrhage. Differential diagnosis: MS lesions are peripheral in location, less than two vertebral segments in length and less than half cross section area. They do not respect gray-white matter boundary. They present homogeneous or ring enhancement. In 90% there is associated brain lesion (Fig 10,11,12). Idiopathic acute transverse myelitis ADEM as MS. Cord neoplasm which causes moderate cord expansion and is associated with edema, cystic or hemorrhagic components. More indolent clinical course (Fig. 13). Cord infarct which is ventraly located, has less mass effect initially and no enhancement in acute phase. Neuromyelitis optica which appears as diffuse intramedullary hyperintensity on T2WI, causes cord swelling and associated with bilateral retrobulbar optic neuritis. Vitamin B12 deficiency as hyperintense on T2WI lesions in dorsal/+ lateral columns, with no enhancement. Syringohydromyelia. Page 3 of 35

Images for this section: Page 4 of 35

Fig. 1: axial T2WI:mass like lesion in a patient with tumefactive MS, additional lesion in subcortical white matter. Page 5 of 35

Fig. 2: axial T1WI with contrast:irregular, thick, partial ring enhancement about a mass like lesion, in a patient with tumefactive MS. Page 6 of 35

Fig. 3: Axial FLAIR: hyperintensites perpendicular to ventricular system in a patient with MS. Page 7 of 35

Fig. 4: sagittal T2WI:lesions perpendicular to callososeptal interface and subcortical white matter. Page 8 of 35

Fig. 5: Axial FLAIR: periventricular hyperintensity and diffuse hyperintense lesions in centrum semiovale due to small vessel disease. Page 9 of 35

Fig. 6: Axial T2WI: hyperintense lesion in pons in a patient with Bechet disease. Page 10 of 35

Fig. 7: Axial T2WI:hyperintense lesions in cerebral penducles in a patient with Behcet. Page 11 of 35

Fig. 8: axial FLAIR: subcortical white matter hyperintensites and atrophy in a patient with HIV encephalitis. Page 12 of 35

Fig. 9: axial FLAIR:HIV encephalitis. Page 13 of 35

Fig. 10: axial T2WI:well circumscribed demyelinating plaque in anterolateral aspect of cord. Page 14 of 35

Fig. 11: sagittal T1 with contrast:homogeneous enhancing demyelinating plaque. Page 15 of 35

Fig. 12: axial T2WI:hyperintense lesion that does not respect gray-white matter boundary, in a patient with MS. Page 16 of 35

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Fig. 13: axial T1WI with contrast: solid enhancing and cystic part of a spinal cord tumor. Page 18 of 35

Imaging findings OR Procedure details We present 5 cases of female patients (age 21-27, 42yrs) with history of SLE. Three of them (case 2,4,5) had additionally antiphospholid syndrome. Imaging findings were the following: Case 1 : abnormal signal intensity lesions in body of corpus callosum (Fig.1sagittal T2 WI) and extensive bilateral infarcts parietoccipital (Fig2,3-axial Flair WI). Case 2: subacute ischemic lesions at caudate nucleus, basal ganglia, internal and external capsule on left side (Fig4-Diffusion WI, Fig5-FLAIR WI, Fig6- T2WI, Fig7-T1WI with cm). Case 3:acute ischemic lesions in left parietal-occipital lobe(fig 8,9-FLAIR WI, Fig 10,11- Diffusion-WI) and periventricular hyperintensity of white matter. Case 4: Old hemmorrhagic lesion-gliosis in right cerebellar lobe (Fig 12axial CT scan), old ishemic lesion in left parietal lobe (Fig 13- axial CT scan). She has also a drainage tube in ventricular system because she had developed hydrocephalus (Fig 13). Case 5: Central cord lesion involving almost all cross sectional area of spinal cord, there is smooth cord expansion (Fig.14- axial T2WI). On sagittal sections lesion is more than two vertebral segments in length. Images for this section: Page 19 of 35

Fig. 1: Sagittal T2WI:Abnormal signal intensity lesions in body of corpus calosum. Page 20 of 35

Fig. 2: Axial FLAIR :Bilateral extensive infracts parietoccipital. Page 21 of 35

Fig. 3: As Fig 1,2 Page 22 of 35

Fig. 4: Restricted diffusion due to subacute ischemic lesion involving left basal ganglia. Page 23 of 35

Fig. 5: Axial FLAIR :Increased signal intensity of left basal ganglia due to subacute ischemic lesion. Page 24 of 35

Fig. 6: Axial T2WI:As previous image. Page 25 of 35

Fig. 7: Axial T1WI with contrast :Luxury perfussion of above ischemic lesions. Page 26 of 35

Fig. 8: Axial FLAIR :Periventricular white matter hyperintensity and acute ischemic lesion left parietal. Page 27 of 35

Fig. 9: Axial FLAIR:As previous. Page 28 of 35

Fig. 10: Restricted diffusion due to acute ischemic lesion. Page 29 of 35

Fig. 11: Restricted diffusion due to acute ischemic lesion. Page 30 of 35

Fig. 12: Gliosis of left cerebellar lobe due to previous hemorrhage. Page 31 of 35

Fig. 13: Old ischemic lesion left parietal-occipital. Drainage tube due to hydrocephalus. Page 32 of 35

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Fig. 14: Axial T2WI:Central hyperintense lesion involving almost all cross sectional area of cord. Page 34 of 35

Conclusion MRI is very supportive of SLE diagnosis in association with history, clinical and laboratory findings. Personal Information Tavernaraki Ekaterini Consultant Radiologist <<Evagelismos>> Hospital, CT-MRI Dept. e-mail:katiatav@yahoo.com References R. Grossman, D. Yousem <<The requisites- Neuroradiolgy>> 2nd edition, 2003, Mosby editor. A. Osborn <<Diagnostic Imaging-Brain>> 1st edition, 2004, Amirsys editor. J. Ross<<Diagnostic Imaging-Spine>> 1st edition, 2007, Amirsys editor. Page 35 of 35