At the heart of primary progressive multiple sclerosis: three cases with diffuse MRI abnormalities only

@article{Zwemmer2008AtTH,
  title={At the heart of primary progressive multiple sclerosis: three cases with diffuse MRI abnormalities only},
  author={J N P Zwemmer and Joseph C. J. Bot and Brechtje Jelles and Frederik Barkhof and Chris H. Polman},
  journal={Multiple Sclerosis},
  year={2008},
  volume={14},
  pages={428 - 430}
}
We present three patients with a clinical course and cerebrospinal fluid findings consistent with a diagnosis of primary progressive multiple sclerosis (PPMS). Extensive and repeated magnetic resonance imaging (MRI) examinations showed only diffuse abnormality in brain and spinal cord, but no focal lesions. We propose that these cases represent the most pure form of PPMS, even though according to currently applied criteria this diagnosis can not be made in the absence of focal lesions on MRI… 
View on SAGE
ncbi.nlm.nih.gov
5 Citations
Background Citations
1

Figures from this paper

5 Citations

Diffusion magnetic resonance imaging in multiple sclerosis.

Will the real multiple sclerosis please stand up?

TLDR
It is argued that MS may be an 'immunological convolution' between an underlying primary degenerative disorder and the host's aberrant immune response, with non-inflammatory primary progressive MS as the 'real' MS, with inflammatory forms reflecting secondary, albeit very important, reactions.

The Therapeutic Potential of the Ketogenic Diet in Treating Progressive Multiple Sclerosis

TLDR
Evidence that theketogenic diet can improve mitochondrial function and the potential of the ketogenic diet in treating progressive multiple sclerosis for which no treatment currently exists are reviewed.

Understanding progression in primary progressive multiple sclerosis: a longitudinal clinical and magneticresonance imaging study

TLDR
The work presented in this thesis suggests that grey matter damage has a predilection for certain brain regions and is an important determinant of progression in early PPMS.

Translation towards personalized medicine in Multiple Sclerosis

References

SHOWING 1-10 OF 16 REFERENCES

Diagnostic criteria for primary progressive multiple sclerosis: A position paper

TLDR
New diagnostic criteria developed by a group of investigators on the basis of a review of their considerable experience with primary progressive multiple sclerosis require prospective validation in a cohort of newly diagnosed patients and by postmortem examination.

Imaging primary progressive multiple sclerosis: the contribution of structural, metabolic, and functional MRI techniques

TLDR
The results of these studies underpin that the main factors possibly explaining the clinical/MRI discrepancy observed in patients with PPMS include the presence of a diffuse tissue damage that is beyond the resolution of conventional imaging, the extent of cervical cord damage, and the impairment of the adaptive capacity of the cortex to limit the functional consequences of subcortical pathology.

Diagnostic criteria for multiple sclerosis: 2005 revisions to the “McDonald Criteria”

TLDR
New evidence and consensus now strengthen the role of these criteria in the multiple sclerosis diagnostic workup to demonstrate dissemination of lesions in time, to clarify the use of spinal cord lesions, and to simplify diagnosis of primary progressive disease.

MR of the spinal cord in multiple sclerosis: relation to clinical subtype and disability.

TLDR
The MR appearance of the spinal cord differs among clinical subgroups of MS, with diffuse abnormality associated with a progressive clinical course and greater disability than did patients without diffuse abnormalities.

Evidence for widespread axonal damage at the earliest clinical stage of multiple sclerosis.

TLDR
Widespread axonal pathology, largely independent of MRI-visible inflammation and too extensive to be completely reversible, occurs in patients even at the earliest clinical stage of multiple sclerosis, and argues strongly in favour of early neuroprotective intervention.

Occult tissue damage in patients with primary progressive multiple sclerosis is independent of T2-visible lesions

TLDR
It is shown that in PPMS patients there is a significant microscopic damage of the NABT which is independent of the extent of T2-visible abnormalities, which suggests that Wallerian degeneration of fibers passing through macroscopic abnormalities is not a major factor contributing to diffuse NABt pathology of these patients.

Recommended diagnostic criteria for multiple sclerosis: Guidelines from the international panel on the diagnosis of multiple sclerosis

TLDR
The revised criteria facilitate the diagnosis of MS in patients with a variety of presentations, including “monosymptomatic” disease suggestive of MS, disease with a typical relapsing‐remitting course, and disease with insidious progression, without clear attacks and remissions.

Spinal cord abnormalities in recently diagnosed MS patients

TLDR
Spinal cord abnormalities are prevalent in patients with early-stage MS, have distinct morphologic characteristics, and help to determine dissemination in space at time of diagnosis, according to the most recent diagnostic criteria for multiple sclerosis.

Imaging axonal damage of normal-appearing white matter in multiple sclerosis.

TLDR
Results add to data suggesting that axonal damage or loss may be responsible for functional impairments in multiple sclerosis, and may be of particular significance for understanding chronic disability in this disease.

Post-mortem high-resolution MRI of the spinal cord in multiple sclerosis: a correlative study with conventional MRI, histopathology and clinical phenotype.

TLDR
High-resolution MRI revealed a great range of abnormalities in spinal cord multiple sclerosis, which related to disease course during life and very good correlation between the extent of abnormalities shown by histopathology and the SI changes on proton-density MRIs was found.