DM1 Patients with Small CTG Expansions are also at Risk of Severe Conduction Abnormalities
Bibliographic record
Abstract
BACKGROUND AND OBJECTIVES: A high risk of cardiac arrhythmias was reported in myotonic dystrophy type 1 (DM1). The purpose of the study was to evaluate the risk of severe electrocardiographic abnormalities in DM1 patients with small CTG expansions. METHODS: We assessed the ECG done at DM1 diagnosis for 127 patients with ≤200 CTG repeats and for 82 of them who had ≥1 ECG over a period of follow-up of 11.7 ± 7.6 years (mean ± SD). Criteria of severe ECG abnormality are at least one of the following features: PR interval ≥240 msec, QRS duration ≥120 msec, second-degree or third-degree atrioventricular block, atrial fibrillation or flutter, insertion of a pacemaker or cardioverter-defibrillator. RESULTS: At baseline, ECG was normal for 109 patients out of 127 (85.8%) and only 4 patients (3.1%) presented severe ECG abnormalities. At follow-up, 46 patients out of 82 (56.1%) had a normal ECG and 25 (30.5%) developed severe ECG abnormalities (p < 0.0001) including 6 of them who needed permanent pacemaker insertion. There were also 3 sudden deaths during the follow-up period. Using multivariate Cox regression analysis, age at entry (relative risk RR, 1.05; (95% CI 1.01-1.08; p = 0.012) and muscular weakness (MIRS) at the entry (RR, 2.03; 95% CI 1.28-3.22; p = 0.003) were significant risk factors for the development of severe ECG abnormality. CONCLUSIONS: An increased risk of severe ECG abnormalitiesy and cardiac events is observed even in DM1 patients with small CTG expansions and warrants close cardiac follow-up similar to DM1 patients with larger CTG expansions.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.003 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one teacher head, not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".