Primary-progressive multiple sclerosis
- PMID: 17884680
- DOI: 10.1016/S1474-4422(07)70243-0
Primary-progressive multiple sclerosis
Erratum in
- Lancet Neurol. 2009 Aug;8(8):699
Abstract
About 10-15% of patients with multiple sclerosis (MS) present with gradually increasing neurological disability, a disorder known as primary-progressive multiple sclerosis (PPMS). Compared with relapse-onset multiple sclerosis, people with PPMS are older at onset and a higher proportion are men. Inflammatory white-matter lesions are less evident but diffuse axonal loss and microglial activation are seen in healthy-looking white matter, in addition to cortical demyelination, and quantitative MRI shows atrophy and intrinsic abnormalities in the grey matter and the white matter. Spinal cord atrophy corresponds to the usual clinical presentation of progressive spastic paraplegia. Although neuroaxonal degeneration seems to underlie PPMS, the pathogenesis and the extent to which immune-mediated mechanisms operate is unclear. MRI of the brain and spinal cord, and examination of the CSF, are important investigations for diagnosis; conventional immunomodulatory therapies, such as interferon beta and glatiramer acetate, are ineffective. Future research should focus on the clarification of the mechanisms of axonal loss, improvements to the design of clinical trials, and the development of effective neuroprotective treatments.
Similar articles
-
Brain atrophy and physical disability in primary progressive multiple sclerosis: A volumetric study.Neuroradiol J. 2015 Jun;28(3):354-8. doi: 10.1177/1971400915594984. Neuroradiol J. 2015. PMID: 26246109 Free PMC article.
-
Cord atrophy separates early primary progressive and relapsing remitting multiple sclerosis.J Neurol Neurosurg Psychiatry. 2006 Sep;77(9):1036-9. doi: 10.1136/jnnp.2006.094748. Epub 2006 Jun 22. J Neurol Neurosurg Psychiatry. 2006. PMID: 16793860 Free PMC article.
-
[MRI comparison between hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) and primary progressive multiple sclerosis (PPMS)].Rinsho Shinkeigaku. 2014;54(12):1162-4. doi: 10.5692/clinicalneurol.54.1162. Rinsho Shinkeigaku. 2014. PMID: 25519970 Japanese.
-
Magnetic Resonance Imaging in Primary Progressive Multiple Sclerosis Patients : Review.Clin Neuroradiol. 2022 Sep;32(3):625-641. doi: 10.1007/s00062-022-01144-3. Epub 2022 Mar 8. Clin Neuroradiol. 2022. PMID: 35258820 Free PMC article. Review.
-
The role of advanced magnetic resonance imaging techniques in primary progressive MS.J Neurol. 2012 Apr;259(4):611-21. doi: 10.1007/s00415-011-6195-6. Epub 2011 Aug 4. J Neurol. 2012. PMID: 21814822 Review.
Cited by
-
A Comprehensive Examination of the Role of Epigenetic Factors in Multiple Sclerosis.Int J Mol Sci. 2024 Aug 16;25(16):8921. doi: 10.3390/ijms25168921. Int J Mol Sci. 2024. PMID: 39201606 Free PMC article. Review.
-
A genetic-epigenetic interplay at 1q21.1 locus underlies CHD1L-mediated vulnerability to primary progressive multiple sclerosis.Nat Commun. 2024 Jul 30;15(1):6419. doi: 10.1038/s41467-024-50794-z. Nat Commun. 2024. PMID: 39079955 Free PMC article.
-
A novel PSMB8 isoform associated with multiple sclerosis lesions induces P-body formation.Front Cell Neurosci. 2024 May 15;18:1379261. doi: 10.3389/fncel.2024.1379261. eCollection 2024. Front Cell Neurosci. 2024. PMID: 38812791 Free PMC article.
-
Mapping brain volume change across time in primary-progressive multiple sclerosis.Neuroradiology. 2024 Jul;66(7):1189-1197. doi: 10.1007/s00234-024-03354-7. Epub 2024 Apr 13. Neuroradiology. 2024. PMID: 38609687
-
Visual outcome measures in clinical trials of remyelinating drugs.BMJ Neurol Open. 2024 Feb 19;6(1):e000560. doi: 10.1136/bmjno-2023-000560. eCollection 2024. BMJ Neurol Open. 2024. PMID: 38389586 Free PMC article. Review.
Publication types
MeSH terms
Grants and funding
LinkOut - more resources
Full Text Sources
Other Literature Sources