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Language functions in Huntington's disease
Podoll K, Caspary P, Lange HW, Noth J.. Brain : a journal of neurology, 1988-12-01
Topic: Brain proteins, microglia and lab models
A comprehensive language test battery (Aachen Aphasia Test) was administered to 45 patients in the early, middle or later stages of Huntington's disease (HD) and to 20 control subjects. In spontaneous speech, many HD patients exhibited a loss of conversational initiative. Dysarthria was a common finding. Reading skills were found to be impaired mainly as a consequence of dysarthria; some HD patients displayed visual dyslexia. In addition to the characteristic disturbances of writing skills due to the choreiform movement disorder, the writing of HD patients with advanced dementia indicated constructional dysgraphia, characterized by frequent omissions, perseverations and substitutions. HD patients exhibited no evidence of word-finding difficulty or other semantic deficits in spontaneous speech. There was, however, a marked impairment in visual confrontation naming, with a significant rise in naming error rate as the disease progressed in severity. In most instances, the inappropriate names referred to an object visually similar to the target object, suggesting that visual misperception is the major cause of the naming disorder in HD. Syntactical structure of spontaneous speech was typically reduced to short, simple sentence construction. Verbal stereotypes were only rarely encountered and occurred late in the course of the disease. Tests of language comprehension reflected the general degree of dementia. It is concluded that there are no primary language changes in HD. Instead, a variety of language impairments develop secondary to other neurological and neuropsychological changes.
Key findings
- There was, however, a marked impairment in visual confrontation naming, with a significant rise in naming error rate as the disease progressed in severity.
- In most instances, the inappropriate names referred to an object visually similar to the target object, suggesting that visual misperception is the major cause of the naming disorder in HD.
- It is concluded that there are no primary language changes in HD.
Why it matters
In 45 patients with Huntington's disease, language problems such as naming errors appeared to come mainly from visual misperception and movement-related speech difficulties rather than primary language changes. This concerns Huntington's disease and does not address treating, curing or preventing Alzheimer's disease.
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Study details
- Participants45
- Evidence strengthLow
How this study scored 45/100
Published in a leading specialty journal. Scored zero for study design, effect reported and randomised / blinded because the record does not state it.
| Component | Points | Why |
|---|---|---|
| Journal tier | 9 / 10 | Tier 2 — leading specialty journal (Brain : a journal of neurology) |
| Journal's citation record | 4 / 4 | Brain: journal h-index 503 (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 | 1988 — older than 20 years, still counted |
| Sample size | 1 / 4 | 45 participants |
| 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 (participant count stated) |
| Randomised / blinded | 0 / 2 | No randomisation or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 5 / 7 | Cited 118 times (OpenAlex, 2026-10-10) |
| Citations per year | 1 / 5 | 3.0 citations a year over 40 year(s) since 1988 |
| Impact for its field and year | 1 / 6 | Cited 0.3× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 1 / 6 | Cited by 1 other studies in this library |
| Still being cited | 1 / 4 | Cited 5 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 3 / 4 | Last-listed author J. Noth: h-index 51 (OpenAlex, whole career) |
| Institution's track record | 1 / 3 | Heinrich Heine University Düsseldorf: institution h-index 794 (OpenAlex) |
| Multi-centre collaboration | 1 / 3 | OpenAlex lists 2 institution(s) in 1 country |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 2974745) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Total | 45 / 100 | — |
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Read the original
May need a subscription OpenAlex found no free copy of the full text (checked 6 Feb 1020), so the publisher may charge for it. The PubMed abstract is free.
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
Podoll, K., Caspary, P., Lange, H. W., & Noth, J. (1988). Language functions in Huntington's disease. Brain : a journal of neurology. https://doi.org/10.1093/brain/111.6.1475
Vancouver
Podoll K, Caspary P, Lange HW, Noth J. Language functions in Huntington's disease. Brain : a journal of neurology. 1988. doi:10.1093/brain/111.6.1475. PMID: 2974745.
BibTeX
@article{podoll1988-2974745,
author = {Podoll, K. and Caspary, P. and Lange, H. W. and Noth, J.},
title = {{Language functions in Huntington's disease}},
journal = {Brain : a journal of neurology},
year = {1988},
doi = {10.1093/brain/111.6.1475},
pmid = {2974745},
url = {https://doi.org/10.1093/brain/111.6.1475}
}
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