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Balo ’ s concentric lesions with concurrent features of Schilder ’ s disease in relapsing multiple sclerosis: neuropathological findings

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ABSTRACT

Atypical inflammatory demyelinating syndromes are rare neurological diseases that differ from multiple sclerosis (MS), owing to unusual clinicoradiological and pathological findings, and poor responses to treatment. The distinction between them and the criteria for their diagnoses are poorly defined due to the lack of advanced research studies. Balo’s concentric sclerosis (BCS) and Schilder’s disease (SD) are two of these syndromes and can present as monophasic or in association with chronic MS. Both variants are difficult to distinguish when they present in acute stages. We describe an autopsy case of middle-aged female with a chronic history of MS newly relapsed with atypical demyelinating lesions, which showed concurrent features of BCS and SD. We also describe the neuropathological findings, and discuss the overlapping features between these two variants.

No MeSH data available.


Histological section of the right frontal lesion treated with H&E and LFB (10X) shows the gradual loss of myelin in deep white matter, which is associated with spared subcortical U-fibers (arrow).
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g04: Histological section of the right frontal lesion treated with H&E and LFB (10X) shows the gradual loss of myelin in deep white matter, which is associated with spared subcortical U-fibers (arrow).

Mentions: Sections from the left and right frontal lesions were examined using light and electron microscopy. We used different staining protocols on the sections taken from the right and left frontal lobes. Hematoxylin and eosin (H&E) with Luxol fast blue (LFB) (Figure 3A and 4) was the main staining method. We also created combined stains (Bielschowsky silver stain with LFB (Figure 3B) and neurofilament with LFB (Figure 3C), performed on the left frontal lesion to highlight the SCUF, to examine the axonal process, and to assess the myelin in relation to the axons.


Balo ’ s concentric lesions with concurrent features of Schilder ’ s disease in relapsing multiple sclerosis: neuropathological findings
Histological section of the right frontal lesion treated with H&E and LFB (10X) shows the gradual loss of myelin in deep white matter, which is associated with spared subcortical U-fibers (arrow).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5304558&req=5

g04: Histological section of the right frontal lesion treated with H&E and LFB (10X) shows the gradual loss of myelin in deep white matter, which is associated with spared subcortical U-fibers (arrow).
Mentions: Sections from the left and right frontal lesions were examined using light and electron microscopy. We used different staining protocols on the sections taken from the right and left frontal lobes. Hematoxylin and eosin (H&E) with Luxol fast blue (LFB) (Figure 3A and 4) was the main staining method. We also created combined stains (Bielschowsky silver stain with LFB (Figure 3B) and neurofilament with LFB (Figure 3C), performed on the left frontal lesion to highlight the SCUF, to examine the axonal process, and to assess the myelin in relation to the axons.

View Article: PubMed Central - PubMed

ABSTRACT

Atypical inflammatory demyelinating syndromes are rare neurological diseases that differ from multiple sclerosis (MS), owing to unusual clinicoradiological and pathological findings, and poor responses to treatment. The distinction between them and the criteria for their diagnoses are poorly defined due to the lack of advanced research studies. Balo’s concentric sclerosis (BCS) and Schilder’s disease (SD) are two of these syndromes and can present as monophasic or in association with chronic MS. Both variants are difficult to distinguish when they present in acute stages. We describe an autopsy case of middle-aged female with a chronic history of MS newly relapsed with atypical demyelinating lesions, which showed concurrent features of BCS and SD. We also describe the neuropathological findings, and discuss the overlapping features between these two variants.

No MeSH data available.