Autosomal dominant primary lateral sclerosis
Bibliographic record
Abstract
Diseases predominantly affecting the upper motor neuron include primary lateral sclerosis (PLS) and the hereditary spastic parapareses. PLS is a disorder characterized by spinal and bulbar spasticity due to degeneration of the upper motor neuron. We describe here a French Canadian family with progressive involvement of the upper motor neuron that closely mimics the features of PLS. Subjects were referred to the study protocol by their treating physician. Prior to their participation in the study, they signed a consent form approved by the local ethics review board. At least two neurologists performed a thorough neurologic examination on all affected and unaffected family members. Additionally, electrophysiologic studies were performed on most patients by the same electrophysiologist and were validated by a second electrophysiologist. For genetic studies, blood samples were collected from 18 individuals, including 8 affected members in two generations of the family. All eight living affected individuals were available for detailed clinical assessment, while four deceased individuals were considered affected based on reliable history obtained from their children. The …
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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) | 0.000 | 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 | 0.001 |
| 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".