Bibliographic record
Abstract
Primary lateral sclerosis (PLS) is a controversial form of motor neuron disease (MND), with uncertainty whether it represents a distinct clinico-pathological entity or is simply a variant of classical amyotrophic lateral sclerosis (ALS). Neuropathological studies provide an opportunity to investigate these issues; however, there have been very few published descriptions of postmortem findings in clinically defined PLS, using modern techniques. Here, we report the neuropathological features of seven cases of PLS with age at onset ranging from 47 to 73 years and disease duration from 3.5 to 35 years. All cases showed chronic degeneration of the primary motor cortex and/or the corticospinal tracts with preservation of lower motor neurons (LMN). All five cases, in which motor cortex was available, had TDP-43 immunoreactive (TDP-ir) cortical pathology. In all seven cases, TDP-ir inclusions were also present in LMN; however, these were always rare, averaging less than one inclusion per tissue section. The finding of TDP-ir pathology in all our cases suggests that PLS and ALS are closely related conditions. Importantly however, the extremely minor involvement of LMN, even after very long disease duration in some cases, suggests that PLS is a distinct form of MND in which LMN are spared or protected.
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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) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.000 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.001 |
| 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".