Primary-progressive multiple sclerosis
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.
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Brain T2 lesion load tends to be lower in PPMS and the combination of MRI and CSF findings provides a higher sensitivity than MRI alone.
“Brain T2 lesion load tends to be lower in PPMS 6 and the combination of MRI and CSF findings provides a higher sensitivity than MRI alone.20”
From Diagnosis of multiple sclerosis: progress and challenges (2017) · cites this paper as [6]
The onset of PPMS is characterised by slowly progressive symptoms, most often an asymmetric paraparesis that evolves over months or years or, less commonly, a progressive hemiparesis or cerebellar ataxia or very rarely, visual failure or dementia.
“The onset of PPMS in contrast is characterised by slowly progressive symptoms, most often an asymmetric paraparesis that evolves over months or years 6 or, less commonly, a progressive hemiparesis or cerebellar ataxia or very rarely, visual failure or dementia.6”
From Diagnosis of multiple sclerosis: progress and challenges (2017) · cites this paper as [6]
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- Authors
- Miller, D. H., & Leary, S. M.
- Affiliations
- UCL Queen Square Institute of Neurology (GB)
- University College Hospital (GB)
- ORCID
- Leary, Siobhan M
- Venue
- The Lancet Neurology
- Publisher
- Elsevier BV
- Published
- 2007-10
- Volume
- 6
- Issue
- 10
- Pages
- 903-912
- ISSN
- 1474-4422 (ISSN-L)
- OpenAlex
- W1990040006
- Cited by
- 818
- References
- 98
- Citations by year
- 2026: 18 · 2025: 23 · 2024: 30 · 2023: 32 · 2022: 74 · 2021: 74 · 2020: 29 · 2019: 26 · 2018: 32 · 2017: 33 · 2016: 161 · 2015: 66 · 2014: 33 · 2013: 71 · 2012: 19
- FWCI
- 2.78
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Lancet Neurology (publishedVersion)
- PubMed (publishedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Funders
- Multiple Sclerosis Society
- Topics
- Health Sciences › Medicine › Pathology and Forensic Medicine › Multiple Sclerosis Research Studies
- Health Sciences › Medicine › Neurology › Peripheral Neuropathies and Disorders
- Health Sciences › Medicine › Oncology › Polyomavirus and related diseases
- Keywords
- primary progressive multiple sclerosis, axonal loss, neuroaxonal degeneration, microglial activation, cortical demyelination, spinal cord atrophy, quantitative MRI, cerebrospinal fluid biomarkers, interferon-beta, glatiramer acetate, neuroprotection, pathogenesis
- MeSH terms
- Brain, Diagnosis, Differential, Humans, Magnetic Resonance Imaging, Multiple Sclerosis, Chronic Progressive
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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