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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.


Ultrastructural examination of the lesion by electron microscopy. A - Myeloid bodies; B - Zebra bodies.
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g05: Ultrastructural examination of the lesion by electron microscopy. A - Myeloid bodies; B - Zebra bodies.

Mentions: There were no features of acute ischemia in the affected areas. Periodic acid-Schiff (PAS) stain showed no evidence of specific variants of leukodystrophies. The optic nerve, optic chiasm, brainstem, spinal cord, and cerebellum were unremarkable. Ultrastructural examination of poorly preserved fixed tissue only showed scattered myeloid and zebra bodies (Figure 5). The myelin was degenerated and the axons were somewhat preserved.


Balo ’ s concentric lesions with concurrent features of Schilder ’ s disease in relapsing multiple sclerosis: neuropathological findings
Ultrastructural examination of the lesion by electron microscopy. A - Myeloid bodies; B - Zebra bodies.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

g05: Ultrastructural examination of the lesion by electron microscopy. A - Myeloid bodies; B - Zebra bodies.
Mentions: There were no features of acute ischemia in the affected areas. Periodic acid-Schiff (PAS) stain showed no evidence of specific variants of leukodystrophies. The optic nerve, optic chiasm, brainstem, spinal cord, and cerebellum were unremarkable. Ultrastructural examination of poorly preserved fixed tissue only showed scattered myeloid and zebra bodies (Figure 5). The myelin was degenerated and the axons were somewhat preserved.

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.