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Basic and advanced imaging of a case of Balò's concentric sclerosis
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  1. Paolo Ripellino1,2,
  2. Alessandro Stecco3,
  3. Maurizio A Leone2,
  4. Roberto Cantello2
  1. 1Department of Neurology, University of Turin, Torino, Italy
  2. 2Department of Neurology, University of Eastern Piedmont, AOU Maggiore della Carità, Novara, Italy
  3. 3Department of Radiology, University of Eastern Piedmont, AOU Maggiore della Carità, Novara, Italy

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Description

In January 2012 a 51-year-old woman was admitted to our department for a slow increasing right hemiparesis with right brisk deep tendon reflexes. She suffered from Sjogren syndrome, fibromyalgia and hepatitis B virus (healthy carrier).

Conventional MRI scans (figure 1A,B) were typical for Balò's sclerosis.1

Figure 1

At diagnosis. (A) Axial fluid attenuated inversion recovery sequence and (B) sagittal T2-sequence evidence the typical concentric ring lesion in the white matter.

The patient rejected a brain biopsy. In order to better define the lesion, we therefore performed advanced radiological techniques such as diffusion, spectroscopy and perfusion.

In the diffusion sequences (figure 2A) a concentric ring of unrestricted diffusion appeared clearly distinguishable.2

Figure 2

At diagnosis. (A) Diffusion shows a restricted peak depicting the classical ring of demyelination and remyelination. (B) Spectroscopy shows a high ratio Cho/N-Acetylaspartate, double pick of lactate-lipids. (C) Perfusion MRI analysis, based on four regions of interest depicted in D (A, pink, B, white, C, blue, D, red) suggests that the central layers of the ring are more perfused compared to the peripheral ones.

The spectroscopic image (figure 2B) of the lesion documented increased choline peak and decreased N-acetyl aspartate peak, but normal peaks in the normal appearing white matter near the lesion.1

The perfusion analysis (figure 2C), based on four different regions of interest (figure 2D), suggested a decreasing gradient of perfusion from the centre to the periphery of the lesion, supporting the hypothesis that the centre of the ring corresponds anatomically to a deep venous vessel.3 Authors are not aware of previous report of perfusion studies in Balò's sclerosis.

Cerebrospinal fluid analysis detected oligoclonal bands, without anti-aquaporin-4 antibodies.

High-dose intravenous methylprednisolone was started (1 g/day for 10 days) and followed by oral prednisone (1 mg/kg/day for 2 months, then slowly tapered in 2 months) with concomitant lamivudine as antiviral prophylaxis. The right paresis fully recovered after 2 weeks and has not relapsed after 11 months.

The 8 months follow-up MRI showed a reduction in the lesion volume and absence of new demyelinating lesions (figure 3A,B).

Figure 3

At 8 months follow-up (after treatment). (A) Axial fluid attenuated inversion recovery sequence and (B) sagittal T2-sequence evidence the reduction in the lesion volume compared to figure 1.

Learning points

  • Balò's concentric sclerosis is a rare demyelinating disease presenting with a concentric ring in the white matter. The clinical and radiological features can mimic other diseases such as primary central nervous system lymphoma, low-grade glioma or stroke.

  • A brain biopsy should be obtained whenever possible. However, nowadays advanced neuroimaging studies (spectroscopy, MRI diffusion and perfusion) seem to be a reliable tool for the diagnosis.

  • Balò's sclerosis could respond very well to high-dose steroids alone or in combination with other immunosuppressive treatments (eg, plasma exchange).

References

View Abstract

Footnotes

  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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