The DOI matches its Crossref record; 96 of the 98 references it cites were found in a registry or an index.
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 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]
In the citing paper
“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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Natural history of multiple sclerosis: a unifying concept
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Acute axonal injury in multiple sclerosis: Correlation with demyelination and inflammation
Bitsch, A. (2000) · Brain · doi:10.1093/brain/123.6.1174 · Cited by 898
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Metadata
- 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 › Clinical Neurology › Multiple Sclerosis Research Studies
- Health Sciences › Medicine › Clinical 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
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