Bal�'s concentric sclerosis: MRI

Chen, C. J.; Ro, Long Sun; Wang, L. J.; Wong, Yon-Cheong

doi:10.1007/s002340050252

Cited by 3 publications

(7 citation statements)

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“…BCS had beenthoughttohavea slightlyhigherprevalence among young males and to run an acute, rapidlyprogressivelyfatalcourse [1,2].Recently,several patients with a non-fatal form of BCS and the characteristic MR features have also been reported [3^6, 8,10]. These patients, like the one presented here, remained alive for many months or years after the diagnosis.Thismayimplythatmanycasesof BCS with a benign course were not recognized before the advent of MRI.…”

Section: Discussionmentioning

confidence: 50%

“…There is still controversy about whether BCS is a separate demyelinating disease from multiple sclerosis [2,5,7,8]. Interestingly, both concentric lesions and multiple sclerosis-like plaques were found in the MR images of our patient.…”

Section: Discussionmentioning

confidence: 67%

“…Over time, the MR abnormalities of BCS decrease in size and do not enhance with contrast medium. The duration of the characteristic concentric pattern is variable, ranging from 6.5 weeks to 1 year [3,6,8,9]. Bolay et al [9] suggested that the concentric pattern might be a transient feature in the development of BCS.…”

Section: Discussionmentioning

confidence: 99%

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MRI features of Balo's concentric sclerosis.

Ko²,

Cheung³

et al. 1999

The British Journal of Radiology

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We report MRI findings in a 56-year-old woman with Balo's concentric sclerosis (BCS) who initially presented with a progressive hemiparesis. MRI showed two lesions with a concentric pattern in the left frontoparietal region and a laminated, arcuate pattern in the right frontal region. These patterns were best seen in post-contrast images and were consistent with BCS. In addition, there were several small cerebral multiple sclerosis-like plaques. The clinical symptoms improved and the MR findings regressed after corticosteroid therapy. The patient had completely recovered 12 months later, except for mild right hand numbness. MRI showed further regression of the lesions, but the concentric pattern was still present. This case demonstrated that BCS can run a benign prolonged course and may persist for a long time. Concentric or laminated contrast enhancement in the acute phase may suggest that bands of demyelination in BCS occur synchronously rather than successively.

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Section: Discussionmentioning

confidence: 50%

Section: Discussionmentioning

confidence: 67%

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MRI features of Balo's concentric sclerosis.

Ko²,

Cheung³

et al. 1999

The British Journal of Radiology

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“…Balo's concentric sclerosis is a very rare type of demyelinating disease characterized pathologically by large alternating lamellae of demyelinated and myelinated white matter arranged in a concentric pattern. [1][2][3][4][5] This progressive disease is more often found in young male adults and is more common in the Philippines. 3,6 Balo's concentric sclerosis is considered an unusual variant of multiple sclerosis (MS); however, some authors believe it to be a different entity.…”

Section: Introductionmentioning

confidence: 99%

“…3,6 Balo's concentric sclerosis is considered an unusual variant of multiple sclerosis (MS); however, some authors believe it to be a different entity. 1 Although the pathogenesis of the concentric sclerosis is debated, the cause of demyelination is generally presumed to be the same as that of multiple sclerosis. 1 There is striking resemblance between the magnetic resonance (MR) appearance and the histopathological features of MS. 5 Not all cases may show a typical MR appearance.…”

Section: Introductionmentioning

confidence: 99%

Balo’s concentric sclerosis: Value of magnetic resonance imaging in diagnosis

Singh¹,

Kuruvilla²,

Alexander

et al. 1999

Australasian Radiology

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We report two cases of Balo’s concentric sclerosis that demonstrate the typical magnetic resonance imaging (MRI) findings of concentric rings of demyelination involving the superficial and deep white matter and sparing the cortex. In both cases biopsy was not performed as MRI findings and multi‐mode evoked potential studies were consistent with demyelinating illness. The theories regarding the pathogenesis of this peculiar appearance are briefly reviewed.

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Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis

Lucchinetti

Gavrilova

Metz

et al. 2008

Brain

413

648

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Atypical imaging features of multiple sclerosis lesions include size >2 cm, mass effect, oedema and/or ring enhancement. This constellation is often referred to as ‘tumefactive multiple sclerosis’. Previous series emphasize their unifocal and clinically isolated nature, however, evolution of these lesions is not well defined. Biopsy may be required for diagnosis. We describe clinical and radiographic features in 168 patients with biopsy confirmed CNS inflammatory demyelinating disease (IDD). Lesions were analysed on pre- and post-biopsy magnetic resonance imaging (MRI) for location, size, mass effect/oedema, enhancement, multifocality and fulfilment of Barkhof criteria. Clinical data were correlated to MRI. Female to male ratio was 1.2 : 1, median age at onset, 37 years, duration between symptom onset and biopsy, 7.1 weeks and total disease duration, 3.9 years. Clinical course prior to biopsy was a first neurological event in 61%, relapsing–remitting in 29% and progressive in 4%. Presentations were typically polysymptomatic, with motor, cognitive and sensory symptoms predominating. Aphasia, agnosia, seizures and visual field defects were observed. At follow-up, 70% developed definite multiple sclerosis, and 14% had an isolated demyelinating syndrome. Median time to second attack was 4.8 years, and median EDSS at follow-up was 3.0. Multiple lesions were present in 70% on pre-biopsy MRI, and in 83% by last MRI, with Barkhof criteria fulfilled in 46% prior to biopsy and 55% by follow-up. Only 17% of cases remained unifocal. Median largest lesion size on T2-weighted images was 4 cm (range 0.5–12), with a discernible size of 2.1 cm (range 0.5–7.5). Biopsied lesions demonstrated mass effect in 45% and oedema in 77%. A strong association was found between lesion size, and presence of mass effect and/or oedema (P < 0.001). Ring enhancement was frequent. Most tumefactive features did not correlate with gender, course or diagnosis. Although lesion size >5 cm was associated with a slightly higher EDSS at last follow-up, long-term prognosis in patients with disease duration >10 years was better (EDSS 1.5) compared with a population-based multiple sclerosis cohort matched for disease duration (EDSS 3.5; P < 0.001). Given the retrospective nature of the study, the precise reason for biopsy could not always be determined. This study underscores the diagnostically challenging nature of CNS IDDs that present with atypical clinical or radiographic features. Most have multifocal disease at onset, and develop RRMS by follow-up. Although increased awareness of this broad spectrum may obviate need for biopsy in many circumstances, an important role for diagnostic brain biopsy may be required in some cases.

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Bal�'s concentric sclerosis: MRI

Cited by 3 publications

References 0 publications

MRI features of Balo's concentric sclerosis.

MRI features of Balo's concentric sclerosis.

Balo’s concentric sclerosis: Value of magnetic resonance imaging in diagnosis

Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis

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