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1: Brain. 2008 Jul;131(Pt 7):1722-35. Epub 2008 May 30. Links
Mitochondrial defects in acute multiple sclerosis lesions.Mahad D, Ziabreva I, Lassmann H, Turnbull D.
The Mitochondrial Research Group, University of Newcastle upon Tyne, UK.

Multiple sclerosis is a chronic inflammatory disease, which leads to focal plaques of demyelination and tissue injury in the CNS. The structural and immunopathological patterns of demyelination suggest that different immune mechanisms may be involved in tissue damage. In a subtype of lesions, which are mainly found in patients with acute fulminant multiple sclerosis with Balo's type concentric sclerosis and in a subset of early relapsing remitting multiple sclerosis, the initial myelin changes closely resemble those seen in white matter stroke (WMS), suggesting a hypoxia-like tissue injury. Since mitochondrial injury may be involved in the pathogenesis of such lesions, we analysed a number of mitochondrial respiratory chain proteins in active lesions from acute multiple sclerosis and from WMS using immunohistochemistry. Functionally important defects of mitochondrial respiratory chain complex IV [cytochrome c oxidase (COX)] including its catalytic component (COX-I) are present in Pattern III but not in Pattern II multiple sclerosis lesions. The lack of immunohistochemically detected COX-I is apparent in oligodendrocytes, hypertrophied astrocytes and axons, but not in microglia. The profile of immunohistochemically detected mitochondrial respiratory chain complex subunits differs between multiple sclerosis and WMS. The findings suggest that hypoxia-like tissue injury in Pattern III multiple sclerosis lesions may be due to mitochondrial impairment.

PMID: 18515320 [PubMed - indexed for MEDLINE]

We herein present five cases diagnosed as BCS based on their MR imaging findings, which were similar to those of other previously reported cases with antemortem diagnosis (9, 12, 13, 16).

All our patients had a benign course without further relapses within a mean follow-up period of 30 months (range, 6–47 months) (Table). Such an outcome has also been reported in recent cases, with some showing spontaneous remission, as seen in our first case (16, 22).

Most of the earlier cases with a progressive and fatal course were based on autopsy findings. Therefore, it is very likely that many cases with a benign course were either missed or misdiagnosed before the introduction of MR imaging into clinical practice.

This report presents seven Hans Chinese subjects diagnosed as BCS on the basis of the pathognomonic MR (magnetic resonance) findings.

Upon diagnosis, all the cases displayed good responses to corticosteroids and showed an overall benign prognosis during a follow-up period of 4-13.5 years, although three relapsed later.

MR findings suggest that the characteristic concentric lesions of BCS frequently (5/7) coexist with multiple sclerosis-like lesions.

During follow-up, the Baló-like lesions may either dissolve over time or transform into an MS-like lesion. Moreover, the Baló and MS-like lesions occurred one after another at the onset and relapse phases of the same patient in two cases.

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