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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).
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.
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).
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).
Competing interests None.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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