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Motor neuron disease-inclusion dementia presenting as cortical-basal ganglionic degeneration
Grimes DA, Bergeron CB, Lang AE.. Movement disorders : official journal of the Movement Disorder Society, 1999-07-01
Topic: Risk, prevention and care in population studies
The frontotemporal dementias are a group of relatively new and evolving clinical and pathologic entities. The predominant frontal-temporal atrophy causes a variety of clinical syndromes, usually dominated by disturbances in behavior, mood, and speech. The motor neuron disease-inclusion dementia (MNDID) subtype is characterized by the accumulation of specific intraneuronal ubiquitin-immunoreactive inclusions with the complete absence of tau immunoreactivity. We present a patient with the clinical and neuroimaging characteristics of a highly asymmetric neurodegenerative condition distinguished by limb rigidity, bradykinesia, dystonia with an alien limb phenomenon, cortical sensory findings, and limb apraxia. His premorbid diagnosis was cortical-basal ganglionic degeneration but he had the typical histologic features of a frontotemporal dementia of the MNDID subtype.
Key findings
- The frontotemporal dementias are a group of relatively new and evolving clinical and pathologic entities.
- The predominant frontal-temporal atrophy causes a variety of clinical syndromes, usually dominated by disturbances in behavior, mood, and speech.
- The motor neuron disease-inclusion dementia (MNDID) subtype is characterized by the accumulation of specific intraneuronal ubiquitin-immunoreactive inclusions with the complete absence of tau immunoreactivity.
- We present a patient with the clinical and neuroimaging characteristics of a highly asymmetric neurodegenerative condition distinguished by limb rigidity, bradykinesia, dystonia with an alien limb phenomenon, cortical sensory findings, and limb apraxia.
- His premorbid diagnosis was cortical-basal ganglionic degeneration but he had the typical histologic features of a frontotemporal dementia of the MNDID subtype.
Why it matters
This case report describes a patient with clinical and neuroimaging characteristics of cortical-basal ganglionic degeneration who was found to have the typical histologic features of frontotemporal dementia of the motor neuron disease-inclusion dementia subtype. The study did not report any outcomes related to treating, curing, or preventing Alzheimer's disease.
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Study details
- Evidence strengthLow
How this study scored 40/100
Published in a peer-reviewed journal. Scored zero for study design, sample size, effect reported and randomised / blinded because the record does not state it.
| Component | Points | Why |
|---|---|---|
| Journal tier | 7 / 10 | Tier 3 — peer-reviewed journal (Movement disorders : official journal of the Movement Disorder Society) |
| Journal's citation record | 3 / 4 | Movement Disorders: journal h-index 281 (OpenAlex, 2026-10-29) |
| Study design | 0 / 12 | No study design recorded — scores zero, not guessed (not stated in the record — scored 0) |
| Strength of evidence | 1 / 5 | Rated “low” in our summary |
| Peer-review validation | 10 / 10 | Peer-reviewed. Published as an article in a named peer-reviewed journal. |
| How recent | 1 / 5 | 1999 — older than 20 years, still counted |
| Sample size | 0 / 4 | Participant count not stated — scores zero (not stated in the record — scored 0) |
| Effect reported | 0 / 3 | No effect size reported — scores zero (not stated in the record — scored 0) |
| Studied in people | 3 / 3 | Studied in people (pubmed indexes it as a clinical or observational study) |
| Randomised / blinded | 0 / 2 | No randomisation or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 4 / 7 | Cited 58 times (OpenAlex, 2026-10-10) |
| Citations per year | 1 / 5 | 2.0 citations a year over 29 year(s) since 1999 |
| Impact for its field and year | 2 / 6 | Cited 1.9× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 2 / 6 | Cited by 3 other studies in this library |
| Still being cited | 0 / 4 | Cited 2 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 4 / 4 | Last-listed author Anthony E. Lang: h-index 168 (OpenAlex, whole career) |
| Institution's track record | 0 / 3 | Toronto Western Hospital: institution h-index 525 (OpenAlex) |
| Multi-centre collaboration | 0 / 3 | OpenAlex lists 1 institution(s) in 1 country — scores zero (affiliation lists can be incomplete) |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 10435507) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Total | 40 / 100 | — |
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Read the original
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Cite this study APA, Vancouver, BibTeX · download .bib or .ris
Built from the record alzhub holds for this paper, not looked up. Volume, issue and page numbers are not held in the record. Check the citation against the original before you use it.
APA 7th
Grimes, D. A., Bergeron, C. B., & Lang, A. E. (1999). Motor neuron disease-inclusion dementia presenting as cortical-basal ganglionic degeneration. Movement disorders : official journal of the Movement Disorder Society. https://doi.org/10.1002/1531-8257(199907)14:4<674::aid-mds1019>3.0.co;2-x
Vancouver
Grimes DA, Bergeron CB, Lang AE. Motor neuron disease-inclusion dementia presenting as cortical-basal ganglionic degeneration. Movement disorders : official journal of the Movement Disorder Society. 1999. doi:10.1002/1531-8257(199907)14:4<674::aid-mds1019>3.0.co;2-x. PMID: 10435507.
BibTeX
@article{grimes1999-10435507,
author = {Grimes, D. A. and Bergeron, C. B. and Lang, A. E.},
title = {{Motor neuron disease-inclusion dementia presenting as cortical-basal ganglionic degeneration}},
journal = {Movement disorders : official journal of the Movement Disorder Society},
year = {1999},
doi = {10.1002/1531-8257(199907)14:4<674::aid-mds1019>3.0.co;2-x},
pmid = {10435507},
url = {https://doi.org/10.1002/1531-8257(199907)14:4<674::aid-mds1019>3.0.co;2-x}
}
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