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

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.

Myelinoclastic diffuse sclerosis (Schilder’s disease) is immunologically distinct from multiple sclerosis: results from retrospective analysis of 92 lumbar punctures

“…Readers interested in the history of neurology may appreciate to learn that Poser and Bogaert, in their seminal 1956 article on MDS (in which they pointed out that the cases described by Schilder in 1913 [46] and 1924 [47] rather represented instances of leukodystrophy and subacute sclerosing encephalitis) classified Devic’s NMO as a variant of Schilder’s MDS [48], thereby further contributing to nosological confusion. The only patient with MDS so far tested for serum AQP4-IgG, a highly specific marker of NMO, was negative for that marker [23] (a second patient was negative for CSF AQP4-IgG [49]; however, CSF is not the specimen of choice when it comes to testing for AQP4-IgG [50]).…”