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LETTER TO EDITOR
Year : 2014  |  Volume : 62  |  Issue : 3  |  Page : 304-307

Enhanced T 2 FNx01 weighted angiography imaging and 3-D time-resolved imaging of contrast kinetics findings in Balo′s concentric sclerosis


1 Department of Radiology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China
2 Department of Otorhinolaryngology, The Third People's Hospital of Chongqing City, Chongqing, China

Date of Submission30-Mar-2014
Date of Decision11-Apr-2014
Date of Acceptance27-May-2014
Date of Web Publication18-Jul-2014

Correspondence Address:
Chun Zeng
Department of Radiology, The First Affiliated Hospital of Chongqing Medical University, Chongqing
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.136978

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How to cite this article:
Zeng C, Xiong J, Li Y, Ouyang Y, Lv F, Rumzan R, Ouyang Z. Enhanced T 2 FNx01 weighted angiography imaging and 3-D time-resolved imaging of contrast kinetics findings in Balo′s concentric sclerosis . Neurol India 2014;62:304-7

How to cite this URL:
Zeng C, Xiong J, Li Y, Ouyang Y, Lv F, Rumzan R, Ouyang Z. Enhanced T 2 FNx01 weighted angiography imaging and 3-D time-resolved imaging of contrast kinetics findings in Balo′s concentric sclerosis . Neurol India [serial online] 2014 [cited 2021 Feb 28];62:304-7. Available from: https://www.neurologyindia.com/text.asp?2014/62/3/304/136978


Sir,

Balo's concentric sclerosis (BCS), is a variant of multiple sclerosis (MS). [1] The pathomechanisms of BCS are poorly understood. Magnetic resonance imaging (MRI) is a noninvasive and relatively simple method to study the pathomechanisms of BCS. Conventional MRI has been used to diagnose and monitor the disease spatially and temporally. Advanced MRI techniques including diffusion-weighted imaging (DWI), diffusion tensor imaging (DTI) and magnetic resonance spectroscopy (MRS) have been applied to detect different specificities of BCS pathology. Enhanced T 2 * weighted angiography imaging (ESWAN), a three-dimensional (3-D) high resolution multi-echo magnetic susceptibility imaging technique, has been shown to be very sensitive to iron depositions in lesions and changes in cerebral veins in MS. [2] We assume there are similar characteristics in BCS by using ESWAN. The 3-D time-resolved imaging of contrast kinetics (TRICKS) can assess extra-cranial veins including IJVs. [3] Here, we report the case of a 54-year-old BCS patient from mainland China and the findings by ESWAN and 3D-TRICKS. To the best of our knowledge, ESWAN and 3D-TRICKS have not been described in BCS.

A 54-year-old male patient was admitted with dizziness, numbness and weakness of his right limbs. Neurological examination revealed loss of orientation to time and place, poor calculation ability and memory disturbance. Cerebrospinal fluid (CSF) showed: 27/mm 3 cells, protein 54 mg/dL, normal CSF immunoglobulin G (IgG) index, and no oligoclonal bands. Brainstem and visual auditory evoked potential studies were normal. Patient was treated with 1000 mg/day methylprednisolone for five consecutive days with which he had symptomatic relief. During follow-up neurologic status is stable and there were no relapses.

Imaging was performed on a 3.0 T system with an eight-channel array head coil. Conventional MRI showed two lesions in the right centrum semi-oval with altering isointense and hypointense concentric rings on T 1 -weighted images (T 1 WI) and isointense [Figure 1]a and hyperintense lamellae on T 2 -weighted images (T 2 WI) [Figure 1]b. There were two small ovoid lesions-like MS in the left centrum semi-oval and in the supratentorial right white matter area. Administration of gadolinium-diethylene triamine pentaacetate acid (gd-DTPA) demonstrated the lamellae with concentric ring contrast enhancement [Figure 1]c. ESWAN, a new susceptibility-weighted imaging modality was acquired with 3-D high resolution and eight echoes. All echoes were then automatically reconstructed and combined as a weighted average by the postprocessing algorithm including magnitude images and phase images. Two ring hypointensities on phase images showed evidence of iron deposition, with a phase value of (0.04 ± 0.03) for the isointense regions, (−0.05 ± 0.08) for the hypointense regions, and (0.06 ± 0.05) for the contralateral normal appearing white matter (NAWM) regions [` 1d]. In addition, diminished and shortened right deep medullary veins were observed on minimum intensity projection (minIP) compared with the contralateral NAWM [Figure 1]e. Morphologic features of the IJVs were classified into the following five categories based on the literature. [3] absent, pinpoint, flattened, crescentic and ellipsoidal. Only absent and pinpoint flows were defined as abnormal. On 3-D TRICKS, local absence of the left IJV and more venous collateral circulations in the left extracranial veins were shown on reconstructed coronal images, which was considered abnormal [Figure 1]f.
Figure 1: (a) Axial T1–WI shows two lesions with typical concentric pattern in the right centrum semi-oval, coexisting with plaque-like lesions in the left centrum semi-oval (arrows) and in the supratentorial right white matter area (b) Axial T2-WI shows two lesions with typical concentric pattern in the right centrum semi-oval, coexisting with plaque-like lesions in the left centrum semi-oval (arrows) and in the supratentorial right white matter area (c) The bigger larger lesion displays ring enhancement with Gd-DTPA (d) The bigger larger lesion shows altering isointensity and hypointensity concentric rings (e) The deep medullary veins in right side decrease in the minIP on ESWAN (f) Volumetrically reconstructed coronal 3D-TRICKS image shows absence of left internal jugular veins (arrow)

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Here, we investigated the iron deposition in the concentric lesion and the changes of the deep medullary veins using ESWAN and assessed the morphologic features of the IJVs by using 3-D TRICKS in a patient with BCS. To our knowledge, this is the first report of BCS based on ESWAN and 3-D TRICKS imaging.

BCS is a rare demyelinating disorder and usually considered as a variant of MS. With regard to its pathology, the most conspicuous feature is the presence of large concentric lesions, consisting of alternating layers of preserved and destroyed myelin. The diagnosis of BCS was supported by the typical radiological appearance consisting of alternating rings and at least two rings of simultaneous Gd-enhancement. Various advanced MRI modalities can detect different pathology of BCS. ESWAN is a novel MRI technique for noninvasive evaluation of the iron deposition and the changes of deep medullary veins in MS. [4] Recent literature reported that the 3-D TRICKS technique was used to show IJV abnormalities in MS patients. [3] Coincidentally, we successfully observed similar results using ESWAN and 3D-TRIKS in this case.

ESWAN is a new 3-D T 2 *-based multi-echo acquisition with a special reconstruction algorithm, which can demonstrate the iron deposition and image small vascular structures in MS patients. In this case, the negative phase value of the ring hypointensity regions on the phase image represents iron deposition, similar to presentations of MS. [2] More interestingly, the ring hypointensity regions showed the ring enhancement after administration of gd-DTPA, thereby indicating a disruption of the blood-brain barrier. As previously shown, iron is enriched within oligodendrocytes myelin in both normal and diseased tissues. In this case, iron depositions in the ring hypointensity regions may be derived from phagocytosis and destruction myelin by macrophages, red cells break down or the hemorrhagic production from damaged brain vessels. The iron deposition may reflect the extent of tissue damage, which may be used as another critical factor to understand pathogenesis of BCS. However, the exact relationship between iron deposition and inflammation in MS and BCS is uncertain.

The diminished and shortened right deep medullary veins in this case were observed, which is consistent with reports of MS, suggesting decreased oxygen utilization in the chronic and widespread diseased tissue state of BCS and leading to decreased levels of venous deoxyhemoglobin. In fact, prior positron emission tomography (PET) investigation had shown that regional cerebral oxygen utilization and oxygen extraction was reduced in both white matter and peripheral cortical gray matter in MS. [5] In contrast, vascular endothelial cell hyperplasia and fiber deposition are the main factors of vascular occlusion. Histopathologic evidence of vascular occlusion was described in the 1930s by Putnam, who reported that vascular inflammation was a precursor of demyelination and may be the primary event in the evolution of the disease [6] Wakefield et al. [7] later demonstrated fibrin deposition and thrombosis of vessels in the absence of cellular infiltration and suggested that thrombosis of small veins and capillaries could represent an ischemic basis for the disease.

Recently, a condition called chronic cerebrospinal venous insufficiency (CCSVI) has been postulated in MS, which is characterized by anomalies of the main extra-cranial cerebrospinal venous routes that cause abnormal cerebral and spinal venous drainage and opening of collateral veins. [2] Interestingly, stenosis of the left IJV and opening of the venous collateral circulation was observed by 3-D TRICKS in this case. However, we are uncertain whether our findings really are similar to MS or coincidental.

Advancements in other neuroimaging modalities can also provide important in vivo markers of BCS. Proton magnetic resonance spectroscopy ( 1 H-MRS) provides insight into the chemical composition change of lesions, and it was reported that 1 H-MRS in BCS shows high choline peak and a decrease in the N-acetylaspartate peak, similar to acute MS plaques. [8] In addition, dynamic enhanced susceptibility-weighted perfusion MR imaging (DSC-MRI) [9] demonstrated increased cerebral blood volume (CBV) within the central part and near the periphery of the lesion with relatively normal CBV in the corresponding region. Although our case has not been detected by 1 H-MRS and DSC-MRI, the iron deposition, diminished and shortened deep medullary veins, and stenosis of IJVs in BCS has been shown, which had not been reported before.

In conclusion, our case report shows that iron deposition in the ring hypointensity and enhancement regions, diminished and shortened deep medullary veins and resulted in stenosis of IJVs in this BCS patient as observed on ESWAN and 3D-TRICKS, thereby illustrating that BCS is a variant of MS. Nevertheless, future studies are necessary to understand the mechanism of the iron depositions with advanced MRI technologies in MS and BCS.

[email protected]

This work were supported by the National Natural Science Foundation of China {grant number 81171309}, the Chongqing Municipal Natural Science Foundation of China [grant number CSTC2011JJA1073], the Medicine Scientific key Research Project of Chongqing Health Bureau Grant {grant number 2011-1-031 and 2012-1-017}, and the National Key Clinical Specialties Construction Program of China.

 
  References Top

1.Kira J. Astrocytopathy in Balo′s disease. Mult Scler 2011;17:771-9.  Back to cited text no. 1
    
2.Haacke EM, Makki M, Ge Y, Maheshwari M, Sehgal V, Hu J, et al. Characterizing iron deposition in multiple sclerosis lesions using susceptibility weighted imaging. J Magn Reson Imaging 2009;29:537-44.  Back to cited text no. 2
    
3.Zivadinov R, Lopez-Soriano A, Weinstock-Guttman B, Schirda CV, Magnano CR, Dolic K, et al. Use of MR venography for characterization of the extracranial venous system in patients with multiple sclerosis and healthy control subjects. Radiology 2011;258:562-70.  Back to cited text no. 3
    
4.Zivadinov R, Ramanathan M, Dolic K, Marr K, Karmon Y, Siddiqui AH, et al. Chronic cerebrospinal venous insufficiency in multiple sclerosis: Diagnostic, pathogenetic, clinical and treatment perspectives. Expert Rev Neurother 2011;11:1277-94.  Back to cited text no. 4
    
5.Brooks DJ, Leenders KL, Head G, Marshall J, Legg NJ, Jones T. Studies on regional cerebral oxygen utilisation and cognitive function in multiple sclerosis. J Neurol Neurosurg Psychiatry 1984;47:1182-91.  Back to cited text no. 5
    
6.Putnam TJ. Evidence of vascular occlusion in multiple sclerosis and encephalomyelitis. Arch Neurol Neuropsychol 1935;32:1298-321.  Back to cited text no. 6
    
7.Wakefield AJ, More LJ, Difford J, McLaughlin JE. Immunohistochemical study of vascular injury in acute multiple sclerosis. J Clin Pathol 1994;47:129-33.  Back to cited text no. 7
    
8.Khiat A, Lesage J, Boulanger Y. Quantitative MRS study of Baló′s concentric sclerosis lesions. Magn Reson Imaging 2007;25:1112-5.  Back to cited text no. 8
    
9.Mowry EM, Woo JH, Ances BM. Baló′s concentric sclerosis presenting as a stroke-like syndrome. Nat Clin Pract Neurol 2007;3:349-54.  Back to cited text no. 9
    


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