MRI IN THE DIAGNOSIS OF NEUROBEHCET S DISEASE ABOUT 25 CASES

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1 MRI IN THE DIAGNOSIS OF NEUROBEHCET S DISEASE ABOUT 25 CASES N. Cherif Idrissi El Ganouni1, L. Akka1, H. Jaafari2, M. Zahlane3, L. Essadouni3, N. Kissani2, O. Essadki1, A.Ousehal1. (1) Department of radiology, (2) department of neurology, (3) department of internal medicine University Hospital MOHAMMED VI MARRAKECH, MOROCCO

2 PURPOSE Neurobehcet s disease is a multisystemic vasculitis of unknown etiology, the neurological attack is generally stern, recovered in 5 to 50% and makes the gravity of this disease. The objective of this work is to analyze the type and the seat of the lesions observed in IRM during the neuro-behcet

3 PATIENTS AND METHODS Retrospective study of 25 cases March October 2007 MRI 100% Multiplanar study 1,5 Tesla system Weighted sequences on T2 Weighted sequences on T1 Sequences FLAIR Angio-MRI Injection of gadolinium The diagnosis of Behcet s disease has been kept according to the criterias established by the international group of Behcet.

4 RESULTS Middle age was 32 years old (17 to 52 years) Masculine predominance : men 76% vs. women 24%

5 Parenchymal lesions : 17 cases : 68% Type: In 16 cases: Variable sizes and shapes (nodular, linear) Low or iso signal on T1 High signal on T2 No perilesionnal edema No enhancemant after contrast No effect of mass on the structures of neighborhood

6 In 1 case : pseudo- tumoral shape Low signal on T1 High signal on T2 Heterogenous enhancement Perilesionnel edema Without effect of mass on the structures of neighborhood BIOPSY: Vascularitis

7 Seat of lesions White substance : 11 cases (44%) Internal capsule : 6 cases Subcortical and semi-oval centers : 5 cases Brain stem : 8 cases (24%) Protuberance : 6 cases Peduncles : 3 cases

8 Thalamus : 4 (16%) Central gray cores : 3 (12%) Cerebellum : 2 (8%) Bulb : 1 (4%) Spinal cord : 1 (4%)

9 MRI axial flair : mutiple periventricular lesions in high signal on flair sequence

10 MRI axial flair : multiples round and ovular lesions of the white substance and the brain stem in high signal on flair sequence

11 Axial T2 Axial T2 Sagittal T2 Axial T1 +Gd Sagittal T1 -Gd MRI : lesions of the white substance and of the bulb are in low signal on T1, high signal on T2, with peripheric enhancement

12 Axial T1 Axial T2 MRI : Frontal pseudotumoral shape in low signal on T1, high signal on T2, with perilesionnal edema and effect of mass on the lateral ventricle associated with a lesion of the left thalamus

13 Medullary MRI on sagittal T2 : lesions in high signal T2 of T11-T12 and T8-T9 levels

14 Vascular lesions : 4 cases Thrombophlebitis of dural venous sinus in 3 cases : Superior longitudinal sinus (SLS) : 2 cases SLS + sigmoid sinus + lateral sinus : 1 case Ischemia stroke of brain stem in 1 case

15 Angio MRI : Thrombophlebitis of the posterior part of superior longitudinal sinus

16 Sagittal T1 -Gd Axial flair Sagittal T1 +Gd Coronal T1 +Gd Axial T1 +Gd Angio MRI MRI : Thrombophlebitis of superior longitudinal sinus, right sigmoid sinus and lateral sinus complicated of bilateral hemorragic encephalomalacia

17 Atrophy : 4 cases Cerebellum : 2 cases Brain stem : 1 case Cortico-subcortical : 1 case Normal MRI : 6 cases

18 DISCUSSION Historic In 1931, a case of iritis of hypopyon associated to bucco-genital ulcerations, to a phlebitis and a hydarthrose of the knees has been described by Adamantiades for the first time. In 1936 Hulusi Behcet, a Turkish Dermatologist, described the clinical triad of recurrent aphthous ulcers, genital ulcerations and uveitis.

19 Epidemiology The prevalence of the Behcet s disease varies geographically. It is endemic in the Mediterranean periphery, in Central Asia, in Japan and in Iran. It is an illness of the young adult (3rd decade), but the cases occurring of the first month until 72 years have been reported. A juvenile beginning has been observed in 7,1% of the cases in Morocco. The masculine predominance described a long time, notably in the Arabian countries, stretches toward the equality of the sex man-woman ratio in the recent sets.

20 Etiopathogeny Vascularitis of venous tropism reaching the small venules of the brain stem and the diencephalon Histology: Neuronal loss, demyelination around the vascular structures, lesion of the vasa vasorum and of the sinus veins = veinous thrombosis +++ Evolution: Gliosis, meningeal fibrosis, atrophy.

21 Criteria of diagnosis of the Behcet s disease (International Study Group goes Behçet's Disease) Recurrent oral ulceration, with at least 3 episodes in one year And two of the following criteria: Genital ulceration : lesion active or scar Skin lesions: erythema nodosum, folliculitis, other ulcers Ocular involvement: uveitis, vasculitis Positive pathergy test : formation of sterile pustule in 24-48h

22 Neurological manifestations The neurological attack of the behcet s disease or neuro- Behcet varies from 2,2 to 50% of the cases It can be inaugural in 5% of the cases. It is one of the most devastating manifestations of the disease because of the functional sequels that it generates. the main clinical aspects of the neuro-behcet are the meningoencephalomyelitis, the brain stem syndrome and the psycho-organic syndrome. Other shapes are described like an intracranial hypertension usually in relation with the thrombosis of a venous sinus and/or of a cerebral vein, ischemic strokes, myelitis, polyneuritis and myositis.

23 MRI signs The parenchymal lesions are the most frequent The vascular lesions are also present and are essentially represented by the thrombophlebitis of the dural veinous sinus Isolated cephalalgia with normal MRI constitutes the third shape of the neuro- Behçet. It s due to a benign intracranial hypertension.

24 Parenchymal lesions Seat of the lesions: The seat of predilection of this lesions is by decreasing order : the cerebral trunk (protuberance), the corona radiata, the internal capsule, the central gray cores and the thalami.

25 Signal anomalies : These lesions are in highsignal on T2, in variable signal on T1, enhancing after gadolinium injection in 60 % of the cases. This enhancement is variable (in clods or annular). The lesions of the neuro-behcet predominate in brain stem. The large and confluent lesions in highsignal on T2 of the brain stem and the central gray cores seems to be specific of the acute phase of neuro-behcet and doesn't pose a differential diagnosis problem with the multiple sclerosis and the systemic lupus erythematosus.

26 Characteristic involvement along the corticospinal tract is well correlated with neurological signs (pyramidal syndrome) The atrophy of the brain stem is one of the signs of the chronic neuro-behcet. An atrophy of the brain stem without cerebral volume loss is a specific sign (specificity of 96,5 % and a low sensitivity).

27 Diffusion weighted image and proton MR spectroscopy are useful in differentiating parenchymal neuro-behcet's disease from acute infarction.

28 Meningeal lesion A meningeal thickening enhanced after injection of gadolinium is also a sign described in the neuro-behcet

29 Medullary lesion The medullary attack is rare. The sites of predilection are the cervical and the dorsal medulla. The attack is typically in highsignal T2, multifocale and non adjoining. This character permits to differentiate the neuro- Behcet of multiple sclerosis, main differential diagnosis.

30 Vascular lesions The venous cerebral thrombosis are relatively common in the neuro-behcet. The differential diagnosis of these thrombosis lands with all other reasons of thrombosis; the clinic and the analysis of the CSF permit to orient the diagnosis. The arterial lesion in the neuro-behcet is rare. Some cases of intracranian arterial aneurysms associated to the neuro- Behcet has been reported in the literature.

31 The MRI with angio-mri sequences have an important contribution in the diagnosis of the thrombophlebitis because of their sensitivity to the phenomena of flow and of thrombosis with possibility of multiplanar analysis. They permit to study: The endoluminal clot The absence of circulating flow within the obstructed vessels The reverberation on the subjacent cerebral parenchyma The evolution of the thrombus.

32 CONCLUSION Magnetic Resonance (MR) findings in Neuro- Behcet are non-specific. Nevertheless, MR has a role in characterizing brain lesions topography, helping in the positive and differential diagnosis and in the follow-up of these patients.

33 REFERENCES 1) N. Mnif, H. Rajhi, N. Mlika, S. Kechaou, B. Abdallah Nejmeddine, R. Hamza, MRI findings in neuro-behcet s disease, Journal of Neuroradiology 2006, 33 (4) : ) ET. Tali, S. Atilla, T. Keskin, T. Simonson, S. Isik, WT. Yuh. MRI in neuro-behçet's disease. Neuroradiology. 1997; 39(1):2-6 3) R. Nuri Sener. Neuro-Behcet s Disease: Diffusion MR Imaging and Proton MR Spectroscopy. Am J Neuroradiol 2003, 24: ) SH. Lee, PH. Yoon, SJ. Park, DI. Kim. MRI findings in neurobehçet's disease. Clin Radiol. 2001; 56 (6): ) C. Ramos, G. Sa, V Cruz. Tedim, A. Lopes, J. Xavier, R. CruzNeuro-Behçet: MR study of a group of patients. Acta Med Port. 2006; 19(6):

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