Tumefactive multiple sclerosis lesions: Diagnostic challenge for the radiologist

Transcription

1 Tumefactive multiple sclerosis lesions: Diagnostic challenge for the radiologist Poster No.: C-0679 Congress: ECR 2015 Type: Educational Exhibit Authors: A. Gargallo Vaamonde, M. Iridoy Zulet, I. Rubio Baines, N. Baraibar Argota, T. Ayuso Blanco, T. Cabada; Pamplona/ES Keywords: CNS, Neuroradiology brain, MR-Diffusion/Perfusion, MR, Education, Outcomes analysis, Inflammation, Outcomes DOI: /ecr2015/C-0679 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. Page 1 of 32

2 Learning objectives To release radiological findings of tumefactive multiple sclerosis lesions. To understand clinical and radiological appearance of Bale concetric sclerosis. To diference these lesions from other space-occupying lesions in the central nervous system. To performed a pictorial review of all cases treated in our hospital in the last 13 years. Page 2 of 32

3 Background Multiple sclerosis (MS): Is an inflammatory demyelinating and neurodegenerative disease of central nervous system. Clinically it is chacaracterized by loss of motor and sensory function and it is one of the most common causes of neurological disability in young adults. Nowadays several variants of MS have been described. Tumefactive multiple sclerosis (TMS): Is an unusual subtype of MS. It is called tumefactive as the lesions are "tumor-like" and they can be radiographically indistinguisable from high grade primary tumours. Fig. 1: Discriminate features of demyelinating diseases. Abbreviations. ADEM: Acute disseminated encephalomyelitis. RRMS: Relapsing remitting multiple sclerosis. TMS: Tumefactive sclerosis multiple. NMO: Optic neuromielitis Page 3 of 32

4 References: Karussis D et al; The diagnosis of multiple sclerosis and the various related demuelinating syndromes: A critical review; Journal of Autoinmunity; 2014;48-49; Main characteristics of TMS include [1,2]: DEMOGRAPHIC DATA Incidence: 0,3/ hab/year. Female/Male ratio is 2,18. The mean age at onset: years CLINICAL FEATURES Polysymptomatic: Clinical presentations are dependent on the lesions location and size. Symptoms: Headache, cognitive abnormalities, confusion, aphasia, apraxia, loss of motor or sensory function. Acute-subacute presentation. RADIOLOGICAL FEATURES At least one large, >2cm, demyelinating lesion. Edema and mass effect. Hypointense rim in T2- weighted imaging. Incomplete ring enhancement post-gadolinium T1- weighted imaging. TREATMENT High-dose intravenous steroid treatment HISTOLOGICAL FINDINGS Rings of demyelinitation alternating with rings of intact myelin. Page 4 of 32

5 Central necrosis area surrounded by peripheral inflammatory zone and surrounding intact area. Prominent angiogenesis characterized by neovasculature formation. Perivascular cuffing with B cells, T cells and macrophages in the outer area. Perivascular demyelinitation. Page 5 of 32

6 Images for this section: Fig. 1: Discriminate features of demyelinating diseases. Abbreviations. ADEM: Acute disseminated encephalomyelitis. RRMS: Relapsing remitting multiple sclerosis. TMS: Tumefactive sclerosis multiple. NMO: Optic neuromielitis Karussis D et al; The diagnosis of multiple sclerosis and the various related demuelinating syndromes: A critical review; Journal of Autoinmunity; 2014;48-49; Page 6 of 32

7 Findings and procedure details This is a retrospective study to evaluate the clinical, laboratory and radiological information obtained from eight patients diagnosed of TMS in our medical center during the last 13 years. Patient inclusion criteria was having at least one cerebral demyelinating lesion, (larger than 20 mm in size) in cases with or without a definite diagnosis of MS. The information was obtained from the patients and their files. The radiological parameters registered were: the size, location, any patterns of Gadolinium enhancement, mass effect, degree of edema and restriction in diffusionweighted MRI. Demographic data: We found that 37.5% (n=3) of our patients were male and 62.5% (n=5) were female Fig. 2 on page 9. The mean age of the study group was 37.9 years (min: 17, max: 64) Fig. 3 on page % of our patients (n= 1) had a previous diagnosis of MS. Clinical course: Out of 8 patients, 4 had no additional attacks, thus remained as isolated tumefactive demyelinating lesion (TDL). The patient with previous diagnosis of relapsing-remitting MS progressed to secondary progressive MS. The final diagnosis of the remaining 3 cases was relapsing-remitting MS. One of them developed a second tumefactive demyelinating lesion 10 years after the disease onset. The mean follow up duration was 68.5 months. Pyramidal symptoms were the most common neurological findings, followed by sensitive symptoms Fig. 4 on page 10. Radiological features: Focal TDL was the most common presentation, with a predilection for parietal lobes of the brain. The most common contrast enhancement pattern was a closed ring Fig. 5 on page 11. We also found restriction to diffusion-weighted MRI as described by Hyland et al. Cerebrospinal fluid (CSF) findings: 50% (n=4) of our patients had positive oligoclonal bands (OCB) and 3 were negative. Lumbar puncture could not be performed in one patient. Fig. 6 on page 12 Patient treatment: All patients were treated with steroids, a currently accepted treatment for TDL. All of them showed a significant response, except the younger one, who precised plasma exchange with little clinical improvement. Page 7 of 32

8 3 out of 4 patients with a monophasic course do not take chronic treatment. The patient left follows a second line treatment due to the severity of the attack. 50% of our patients are currently diagnosed of MS and thus take first or second line MS treatments Fig. 7 on page 13 In Fig. 8 on page 14 we present main clinical feaures of each case. The main radiological profiles we found were the following: Hyperintensity in T2 weighted sequences: Fig. 9 on page 15, Fig. 10 on page 16, Fig. 11 on page 17, Fig. 12 on page 18, Fig. 13 on page 19, Fig. 14 on page 20, Fig. 15 on page 21 and Fig. 16 on page 22 Gadolinium enhancement patterns: Fig. 17 on page 23, Fig. 18 on page 24 Fig. 19 on page 25, and Fig. 20 on page 26. Restricted diffusion: Fig. 21 on page 27 and Fig. 22 on page 28 Page 8 of 32

9 Images for this section: Fig. 2 Page 9 of 32

10 Fig. 3 Page 10 of 32

11 Fig. 4 Page 11 of 32

12 Fig. 5 Page 12 of 32

13 Fig. 6 Page 13 of 32

14 Fig. 7 Page 14 of 32

15 Fig. 8 Page 15 of 32

16 Fig. 9: Case 1. Inhomogeneous T2 hyperintense lesion in the right periventricular parietal white matter Page 16 of 32

17 Fig. 10: Case 2. Right middle cerebellar peduncle lesion, hyperintense in T2 weigthed images. Page 17 of 32

18 Fig. 11: Case 3. Inhomogeneous T2 hyperintense lesion in the right periventricular parietal white matter. Page 18 of 32

19 Fig. 12: Case 4. Parietal hyperintensity in T2 weighted sequences. Demyelinating lesions according to previously diagnosed Multiple Sclerosis. Page 19 of 32

20 Fig. 13: Case 5. Parietal lesion, hyperintense in T2 weighted images. Page 20 of 32

21 Fig. 14: Case 7. Inhomogeneous T2 hyperintense lesion in the left periventricular parietal white matter. Page 21 of 32

22 Fig. 15: Case 7. T2-weighted sagital image shows alternating concentric zones of demyelinated and myelinated white matter in the left parietal white matter. Demyelinating lesions according to previously diagnosed Multiple Sclerosis. Page 22 of 32

23 Fig. 16: Case 7. T2-weighted axial image shows alternating concentric zones of demyelinated and myelinated white matter in the left parietal white matter. Demyelinating lesions according to previously diagnosed tumefactive lesion and Multiple Sclerosis. Page 23 of 32

24 Fig. 17: Case 2. Contrast-enhanced T1-weighted axial image showing a right middle cerebellar peduncle lesion with typical open-ring enhancement. Page 24 of 32

25 Fig. 18: Case 3. Gadolinium-enhanced T1-weighted magnetic resonance images showing a inhomogeneous pattern of enhancement. Page 25 of 32

26 Fig. 19: Case 6. Example of contrast-enhanced T1-weighted coronal MRI showing typical concentric ring pattern of enhancement. Page 26 of 32

27 Fig. 20: Case 6. Contrast-enhanced T1-weighted coronal MRI showing typical concentric ring pattern of enhancement. Page 27 of 32

28 Fig. 21: Case 1. Diffusion-weighted MRI showing restricted diffusion with homogeneous reduced ADC signal. Page 28 of 32

29 Fig. 22: Case 7. Diffusion-weighted MRI showing restricted diffusion in lesion borders with reduced ADC signal. Page 29 of 32

30 Conclusion Tumefactive inflammatory demyelinating lesions are a diagnostic challenge for the radiologist. They can mimic brain tumors or other occupational lesions in MRI, however, radiological and clinical data may narrow the diferential diagnosis. Advanced MR techniques such as proton MR spectroscopy (H-MRS) can provide useful additional information to differentiate these lesions, although sometimes brain biopsy may be needed for a definitive diagnosis. Page 30 of 32

31 Personal information a b b a Gargallo Vaamonde A, Iridoy Zulet M, Rubio Baines I, Baraibar Agorta N, Ayuso b Blanco T, Bacaicoa MC a a. Servicio de Radiología Complejo Hospitalario de Navarra (Spain). b. Servicio de Neurología Complejo Hospitalario de Navarra (Spain). Page 31 of 32

32 References 1. Megan R et al; Fulminant demyelinating diseases; The Neurohospitalist; 2013; 3: Karussis D et al; The diagnosis of multiple sclerosis and the various related demuelinating syndromes: A critical review; Journal of Autoinmunity; 2014;48-49; Masaky K et al; Gadolinium enhancement patterns of tumefactive demyelinating lesions: correlations with brain biopsy findings and pathophysiology; J Neurol; 2014; 261: Altintas A et al; Clinical and radiological characteristics of tumefactive demyelinating lesions: follow-up study. Multiple Sclerosis Journal; 2012; 18(10); Barun B et al; Balo s Concentric Sclerosis un Multiple Sclerosis. Intern Med 2012; 51; Berghoff M et al. 7 Tesla MRI demonstrates vascular pathology in Balo s concentric sclerosis. Mult sclera 2013; 19: Lanciano NJ et al. High-Dose steroid Treatment in a patient with baló disease diagnosed by means of magnetic resonance imaging. 8. Gavra M et al. Pitfalls in the diagnosis of a tumefactive demyelinating lesion: a case report. Jorunal of Medical Case Reports 2011; 5: Stadekmann C et al. Tissue preconditioning may explain concentric lesions in Baló s type of multiple sclerosis. 10. Filippi M et al. Association between pathological and MRI findings in multiple sclerosis. Lancet Neurol. 2012; 11: Oliver MD et al. Balo s concntric sclerosis. The New England Journal of Medicine; 2011, 8, Hyland M et al. Restricted diffusion preceding gadolinium enhancement in large or tumefactive demyelinating lesions. Neurology, 2013 Page 32 of 32