Differentiation Between Primary Lateral Sclerosis and Amyotrophic Lateral Sclerosis
Bibliographic record
Abstract
BACKGROUND: Motor neuron diseases can affect the upper motor neuron and/or the lower motor neuron. Both amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS) are motor neuron diseases, and there is much debate as to whether these are 2 separate disorders or simply 2 points on a continuum. OBJECTIVE: To determine which clinical features at onset and during follow-up could help differentiate between PLS and ALS. DESIGN: Retrospective study comparing patients with a diagnosis of PLS or ALS for differences in symptoms or signs at disease onset and during follow-up. SETTING: Tertiary referral center. Patients Six hundred sixty-one patients with ALS and 43 patients with PLS were included in the study. RESULTS: At presentation, stiffness was the only symptom that was significantly different between patients with PLS and patients with ALS (observed in 47% and 4% of patients, respectively; P<.001). During follow-up, limb wasting was rare in patients with PLS (2%, compared with 100% in patients with ALS; P<.001). Disease duration was significantly longer in patients with PLS compared with patients with ALS (mean +/- SD, 11.2 +/- 6.1 vs 3.8 +/- 4.2 years, respectively; P<.001). During the 16 years of follow-up, the mortality rate was significantly lower in patients with PLS compared with patients with ALS (only 33% vs 89%, respectively; P<.001). CONCLUSION: Our findings suggest that a patient presenting with spasticity who does not develop wasting within 3 years most likely has PLS.
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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 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.001 |
| 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".