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"ymax": 3116 }, { "text": "CVNS", "xmin": 151, "ymin": 3132, "xmax": 258, "ymax": 3161 }, { "text": "2022", "xmin": 272, "ymin": 3132, "xmax": 350, "ymax": 3161 }, { "text": "V4N2", "xmin": 366, "ymin": 3132, "xmax": 462, "ymax": 3161 }, { "text": "14", "xmin": 1269, "ymin": 3120, "xmax": 1304, "ymax": 3148 } ], "raw_text": "Journal of Cardiovascular Neurovascular & Stroke https://mycvns.com THE MRI FEATURES IN DIAGNOSING TUMEFACTIVE DEMYELINATING LESION See Yun Foo' Nurul Hafidzah Rahim? : Aida Widure Mustapha Mohd Mustapha *1 Noraini Rose Mohd Zaini? Department of Radiology | Hospital Canselor Tuanku Muhriz 7 Universiti Kebangsaan Malaysia Cheras. Malaysia 2 Department of Radiology Hospital Kuala Lumpur Kementerian Kesihatan Malaysia Kuala Lumpur Malaysia * Corresponding author | Aida Widure Mustapha Mohd Mustapha, Department of Radiology Hospital Canselor Tuanku Muhriz 1 Universiti Kebangsaan Malaysia . Cheras, Malaysia Email aida.widure.mustapha@ppukm ukm.edu.my DOI : https://doi.org/ 10.32896/cvns.v4n3.14-20 Received : 25.11.2021 Revised : 31.07.2022 Accepted | 03.08.2022 Published | 30. .09.2022 ABSTRACT Background : A demyelinating lesion can present as a space occupying lesion in Magnetic Resonance Imaging (MRI) brain Lesions that have diameter greater than 2 cm is referred as tumefactive demyelinating lesion These lesions can mimic a brain tumor namely glioma or primary central nervous system lymphoma. Case presentation | We present two female patients over 30 years old who presented with limb weakness Their MRI demonstrated typical appearance of a solitary . incomplete rim enhancing lesion that involved the subcortical white matter with minimal mass effect. Also D the rim of enhancement opened to the grey matter Due to their typical appearance C biopsy was not performed The lesions in both patients reduced in size upon follow up after treatment commencement and currently recovering Conclusion | In this case report we focus on discussing the MRI features of tumefactive demyelinating lesion and its main differentials, which are central nervous system lymphoma ( CNSL I and glioma Keywords Magnetic Resonance Imaging (MRI); Tumefactive demyelinating lesion (TDL ) | Open ring enhancement INTRODUCTION diabetes mellitus, and previously activities of daily A demyelinating lesion living ( can present as a space ADL independent. She presented with occupying lesion mimicking brain tumor (1). With a aphasia, difficulty reading and expressing word for 5 diameter greater than 2 cm it is referred to as a months and progressive right sided body weakness tumefactive demyelinating lesion ( (TDL), which for 2 may months. The body weakness worsened and she have variable presentation In this case report we became wheelchair bound No history of fever loss present two cases of TDL and discuss about the of appetite or weight loss On examination there was findings as well as differential diagnosis. right sided homonymous hemianopia, reduced tone power and sensation of the right upper and lower CASE PRESENTATION limbs After thorough neurological examination the Case 1 provisional diagnosis was left parietal lobe syndrome Madam B 44 years old female with underlying with pyramidal sign TRO inflammation / demyelinating syndrome. 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| The lesion parietal lobe. Her blood investigation and cerebral involved the subcortical white matter and cortex with spinal fluid (CSF) study negative for infection. White minimal mass effect. The lesion had low signal on cell count and inflammatory marker were not T1W images and high signal intensity on T2W elevated and her CSF study is normal. Anti- images with peripheral incomplete rim enhancement. Aquaporin 4 antibody is (MRI) negative Magnetic The overall findings were suggestive of TDL She resonance imaging of her brain showed ill- was treated with IV methylprednisolone 1 g for five defined left parietal lobe white matter lesion days subsequently 1 session of measuring 4.5 cm widest which has completed 4th plasma exchange and in dimension . cycle of IV rituximab. I Her demonstrated hypointensity on T1W high signal symptoms improved after the treatment Two follow intensity on T2W images with heterogenicity within up MRI brain showed reduction in size of the lesion and incomplete rim enhancement No significant and resolution of the incomplete rim enhancement perilesional edema or mass effect detected I The (Figure 2 ) overall features were suggestive of TDL with differential diagnosis of primary brain tumor. A DISCUSSION repeated MRI brain with added advance MRI TDL is a lesion with usual size greater than 2 cm sequences which are magnetic resonance diameter spectroscopy (MRS ) was then performed The (2, 3). Solitary TDL is the most common initial MRI findings show significant raised Choline reduced findings (3` 3). It and may exhibit mass effect, with or without NAA perilesional edema and with or particularly at the center of the lesion which without contrast enhancement (1, 3 ). Subcortical suggests neuronal loss. This helped in confirming white matter is lesion is demyelinating commonly involved followed the lesion by the that | She was treated periventricular white matter and brainstem (1). The with methylprednisolone 1 g OD for 5 days | 5 of exchange common location of TDL is followed by sessions plasma and is supratentorial region currently on two monthly (IV especially frontal and intravenous parietal region (4). Smaller ) cyclophosphamide demyelinating lesion in between 05 to 2 cm 500 mg while awaiting for social may also demonstrate similar welfare for IV Rituximab Her imaging findings ( 5 ) support | symptoms following It is a female and follow predominant condition with improved treatment two up female to male ratio of 2.3:1 (1, 6 | ). The mean of MRI brain showed smaller size of the lesion and age onset is in the third decade (3,6, 7). In our case | both resolution of the incomplete rim enhancement of the (Figure 1 1). patients are female aged over thirty years old The clinical presentation of TDL can be variable. The majority is pyramidal involvement such Case 2 as limb weakness 3, hemiparesis or hemiplegia (3, . 8 ) Madam H. | 38 years old female C no known medical Other involvement includes sensory , brainstem 1 illness | previously ADL independent. She cerebellar optic neuritis | visual field defects , complained of progressive right sided upper then dysfunction of the bowel Subsequently, or bladder sexual lower limb weakness she required dysfunction 1 acute cognitive changes, global aphasia, wheelchair for ambulation. On examination 1 there diplopia, and epileptic seizures (3, 8 ). Both of our were increased tone and reflex and reduced power of patients had limb weakness One of them initially the right upper and lower limbs. Sensations of her presented with aphasia. limbs were intact Her blood investigation and CSF 21% of patients with TDL developed classical study does not favor infection White cell count and inflammatory multiple sclerosis (MS ) during follow-up (3). In our marker were not elevated and her CSF cases, both the patients had brain and whole spine study is normal. Anti-Aquaporin 4 antibody is MRI during their follow up but did not show new negative. CT brain and MRI brain showed left brain or spinal cord lesions. parietal intra-axial cystic lesion | The provisional As TDL is one of the mimicker of a CNS differential diagnosis was neuroglial cyst and neoplasm such as glioma and CNS lymphoma atypical supratentorial cystic astrocytoma. 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Both TDL distinguishing TDL from primary CNS neoplasm and CNSL also demonstrate hyperintensity or mixed ( 14 ). Study by Lu et al stated significant difference signal intensity compared to grey matter on T2W seen between PCNSL and TDL. The minimum value images (9 ). In our cases, the lesions demonstrated of ADC histogram ( ADCmin ) of TDL is 684 4+ 101 X T1W hypointensity as compared to white matter and 10-6 mm?/s while the ADCmin of PCNSL is lower T2W hyperintensity as compared to grey matter On which is 563 + - 90 X 10-6 mm?/s (14 ) post contrast study | majority of TDL demonstrate TDL will demonstrate increased choline (CHO | incomplete rim enhancement as compared to CNSL peak and decreased N-acetyl aspartate (NAA ) peak and glioma (7, I 9 ). Some authors commented that the with increased Cho/NAA ratio. A Cho/NAA ratio of enhancement is seen in active demyelination (10 ) | greater than 1.72 or 1.73 favors high grade CNS The incomplete portion of the rim opens to the grey neoplasm. Therefore C in adiunct to the findings in matter (2, 11). The part of the rim enhancement in conventional MRI this helps increase the confident the white matter represents the leading edge of in diagnosing TDL Lactate peak and glutamate- demyelination while the non-enhancing center glutamine peak may also see in TDL (4, 15 ). represents chronic phase (2). In both of our cases, the Correlating the CT features of the TDL with the incomplete rim enhancement opened to the grey MR feature also plays a role The enhancing matter which matched the characteristic MRI component of TDL in MRI appears hypodense on findings of demyelination. CT scan. This finding is highly specific Meanwhile | There are a few MRI features which are more the enhancing component on MRI in CNSL and commonly seen in TDL as compared to CNS glioma. glioma mostly demonstrate either isodense or One ofit is area of T2W hyperintensity that does not hyperdense on CT scan (9 / 12 ). Unfortunately | the show enhancement on post gadolinium image (12 ) I CT images of the patients were not available for In this case report the T2W high signal intensity correlation The common sites of metastases are areas of both patients did not demonstrate lungs, liver and bones (3 ). In the head and neck enhancement on post gadolinium study region 1 the nose and the paranasal sinuses is the Other than conventional MRI MR perfusion is commonest site for metastasis of RCC (3). However an advanced MRI technique that helps to primary tumor of the nasal and paranasal regions is differentiate TDL from other CNS neoplasm with still the commonest compared to metastatic disease similar conventional MRI features. In a study conducted by Cha et al, all TDLs had relative CONCLUSION cerebral blood volume (rCBV) of less than 2 ml/100 In conclusion 1 although differentiating TDL from g. Comparing with CNS neoplasm 1 there is CNS tumor can be challenging for the reporting statistically significant in the difference in mean radiologist. However the typical imaging features in rCBV between both TDL and CNS neoplasm in TDL can increase confidence in diagnosing it. In which higher rCBV is expected in neoplasm due to these cases, the characteristic open rim enhancement neovascularity (4 13 ). In our case reports, MR where the enhancing rim signify the leading edge of perfusion was performed on the first case and her demyelination and open to grey matter with rCBV value is less than 2 ml/100 g which is not supporting finding of T2W hyperintensity which suggestive of CNS neoplasm does not enhance in post contrast study point toward With the emerging used of advanced MRI TDL rather than other diagnosis such as CNSL and techniques, apparent diffusion coefficient (ADC ) glioma. Other than conventional MRI findings b value and Magnetic Resonance Spectroscopy (MRS | advanced MRI techniques such as MR perfusion and were also used to differentiate TDL and CNS MRS serve as great adjuncts to the diagnosis of TDL neoplasm Together with the experience of the reporting The center of TDL which has myelin radiologist, both patients are diagnosed with TDL in destruction and edema changes will have high ADC their initial MRI in our center Biopsies were avoided value while the periphery enhancing region of TDL and both patients responded to treatment and has low ADC value A threshold of 0.556 X 10-3 currently on regular follow up. 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MR features and clinical STATEMENT OF ETHICS correlation in multiple sclerosis. Neuroradiol Written informed consent was obtained from the J.2016 Oct;29(5):384-9 patients for publication of this case and any 6. Altintas A 1 Petek B, Isik N | Terzi M accompanying images. Bolukbasi F Tavsanli M I Saip S. 1 Boz C | Aydin T Arici-Duz O . Ozer F | Siva A ACKNOWLEDGEMENTS Clinical and radiological characteristics of We would like to thank the Director General of tumefactive demyelinating lesions follow-up Health Malaysia for his permission to publish this study. Mult Scler 2012 Oct: 18(10):1448-53 article 7. Mabray MC 2 Cohen BA 1 Villanueva-Meyer JE Valles FF - Barajas RF Rubenstein JL CONFLICT OF INTEREST Cha S. Performance of Apparent Diffusion The authors declare that they have no conflict of Coefficient Values and Conventional MRI interest Features in Differentiating Tumefactive Demyelinating Lesions From Primary Brain FUNDING Neoplasms. AJR Am T Roentgenol. 2015 The authors received no funding from any party for Nov;205(5):1075-85 | this scientific work 8 Balloy G T Pelletier J | Suchet L, Lebrun C Cohen M | Vermersch P, . Zephir H Duhin E DATA A AVAILABILITY STATEMENT Gout O Deschamps R, Le Page E | Edan G , No additional data than the one presented in this Labauge P | Carra-Dallieres C, . Rumbach L article was used Berger E Lejeune P | Devos P, N'Kendjuo JB | Coustans M | Auffray-Calvier E REFERENCES Daumas 1 Jeong Duport B , Michel L - F IH Kim SH Hyun JW | Joung A, Cho Lefrere Laplaud DA . Brosset C HJ Kim HJ | Derkinderen P | Tumefactive demyelinating de Seze J. Wiertlewski S | lesions as a first clinical Clinical. Societe Francophone de la event: imaging, and follow-up observations. J Sclerose en Plaques. Inaugural tumor-like Neurol Sci. I 15;358(1-2):118-24 multiple sclerosis clinical 2015 Nov presentation and medium -term outcome in patients J 2. Given CA 2nd 1 Stevens BS, Lee C. 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( a ) Initial MRI shows left frontoparietal lobe lesion 1 involving the subcortical white matter and cortex demonstrating high signal intensity on T2W ( white arrow ) low signal intensity on T1W and incomplete rim enhancement on post-gadolinium study (black arrow ) The high signal intensity area on T2W does not enhance in post-gadolinium study (b ) Latest MRI in 4 months apart shows significantly smaller lesion with resolution of enhancement CVNS 2022 V4N2 20 " } ] }