Mills’ syndrome revisited Article Swipe
Stephan R. Jaiser
,
Dipayan Mitra
,
Timothy L. Williams
,
Mark R. Baker
·
YOU?
·
· 2019
· Open Access
·
· DOI: https://doi.org/10.1007/s00415-019-09186-3
YOU?
·
· 2019
· Open Access
·
· DOI: https://doi.org/10.1007/s00415-019-09186-3
Mills' syndrome is an idiopathic, slowly progressive, spastic hemiparesis. We describe three cases that have been under review for a minimum of 11 years (range 11-19). In all patients, symptoms started in a leg, with a mean age of onset of 59 years (range 53-63). The only abnormality on laboratory investigations was a mildly elevated CSF protein in one case. MRI demonstrated focal T2 hyper-intensity located eccentrically in the cervical cord ipsilateral to the symptomatic side. No cerebral abnormality was demonstrated. Whilst visual and somatosensory evoked potentials were unremarkable, motor evoked potentials were abnormal in all patients: central motor conduction times were significantly prolonged unilaterally in two patients and bilaterally but asymmetrically in the third. Beta-band (15-30 Hz) intermuscular coherence, a potentially more sensitive method of assessing upper motor neuron integrity, was absent unilaterally in one patient and bilaterally in the other two. One patient developed amyotrophy and thus a picture of amyotrophic lateral sclerosis after 16 years, suggesting that Mills' syndrome is part of the motor neuron disease spectrum. Both amyotrophy and subclinical contralateral upper motor neuron disease can therefore be features of Mills' syndrome. However, even with the most sensitive electrodiagnostic techniques, unilateral upper motor neuron disease can remain the only abnormality for as long as 10 years. We conclude that whilst Mills' syndrome should be classified as a motor neuron disorder, it is a distinct nosological entity which can be distinguished from amyotrophic lateral sclerosis, upper motor neuron-dominant amyotrophic lateral sclerosis and primary lateral sclerosis. We propose diagnostic criteria for Mills' syndrome, and estimate a point prevalence of at least 1.2:1,000,000 based on our well-defined referral population in the North of England.
Related Topics
Concepts
Amyotrophic lateral sclerosis
Upper motor neuron
Motor neuron
Lower motor neuron
Medicine
Abnormality
Neurology
Amyotrophy
Neuroradiology
Progressive muscular atrophy
Neuroscience
Disease
Psychology
Atrophy
Pathology
Psychiatry
Metadata
- Type
- review
- Language
- en
- Landing Page
- https://doi.org/10.1007/s00415-019-09186-3
- https://link.springer.com/content/pdf/10.1007/s00415-019-09186-3.pdf
- OA Status
- hybrid
- Cited By
- 30
- References
- 36
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W2910508614
All OpenAlex metadata
Raw OpenAlex JSON
- OpenAlex ID
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https://openalex.org/W2910508614Canonical identifier for this work in OpenAlex
- DOI
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https://doi.org/10.1007/s00415-019-09186-3Digital Object Identifier
- Title
-
Mills’ syndrome revisitedWork title
- Type
-
reviewOpenAlex work type
- Language
-
enPrimary language
- Publication year
-
2019Year of publication
- Publication date
-
2019-01-10Full publication date if available
- Authors
-
Stephan R. Jaiser, Dipayan Mitra, Timothy L. Williams, Mark R. BakerList of authors in order
- Landing page
-
https://doi.org/10.1007/s00415-019-09186-3Publisher landing page
- PDF URL
-
https://link.springer.com/content/pdf/10.1007/s00415-019-09186-3.pdfDirect link to full text PDF
- Open access
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YesWhether a free full text is available
- OA status
-
hybridOpen access status per OpenAlex
- OA URL
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https://link.springer.com/content/pdf/10.1007/s00415-019-09186-3.pdfDirect OA link when available
- Concepts
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Amyotrophic lateral sclerosis, Upper motor neuron, Motor neuron, Lower motor neuron, Medicine, Abnormality, Neurology, Amyotrophy, Neuroradiology, Progressive muscular atrophy, Neuroscience, Disease, Psychology, Atrophy, Pathology, PsychiatryTop concepts (fields/topics) attached by OpenAlex
- Cited by
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30Total citation count in OpenAlex
- Citations by year (recent)
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2025: 8, 2024: 5, 2023: 4, 2022: 7, 2021: 5Per-year citation counts (last 5 years)
- References (count)
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36Number of works referenced by this work
- Related works (count)
-
10Other works algorithmically related by OpenAlex
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| abstract_inverted_index.contralateral | 174 |
| abstract_inverted_index.demonstrated. | 80 |
| abstract_inverted_index.distinguished | 233 |
| abstract_inverted_index.eccentrically | 66 |
| abstract_inverted_index.intermuscular | 118 |
| abstract_inverted_index.significantly | 102 |
| abstract_inverted_index.somatosensory | 84 |
| abstract_inverted_index.unremarkable, | 88 |
| abstract_inverted_index.asymmetrically | 111 |
| abstract_inverted_index.investigations | 50 |
| abstract_inverted_index.hyper-intensity | 64 |
| abstract_inverted_index.neuron-dominant | 240 |
| abstract_inverted_index.electrodiagnostic | 192 |
| cited_by_percentile_year.max | 99 |
| cited_by_percentile_year.min | 90 |
| countries_distinct_count | 1 |
| institutions_distinct_count | 4 |
| sustainable_development_goals[0].id | https://metadata.un.org/sdg/3 |
| sustainable_development_goals[0].score | 0.6800000071525574 |
| sustainable_development_goals[0].display_name | Good health and well-being |
| citation_normalized_percentile.value | 0.85590425 |
| citation_normalized_percentile.is_in_top_1_percent | False |
| citation_normalized_percentile.is_in_top_10_percent | False |