Kinnier Wilson’s puzzling features of amyotrophic lateral sclerosis
Bibliographic record
Abstract
It is more than 150 years since Jean-Martin Charcot (1825–1893) published the first descriptions of the adult neurodegenerative disorder amyotrophic lateral sclerosis (ALS).1 Though an unmistakable clinical syndrome in its classical form, ALS has been increasingly understood as representing a complex clinical and molecular syndrome that overlaps with frontotemporal dementia (FTD).2 Samuel Alexander Kinnier Wilson (1878–1937) (figure 1) is perhaps best known for his eponymous hepatolenticular degenerative syndrome linked to copper dysregulation.3 He is also distinguished as the founding editor, in 1920, of the Journal of Neurology & Psychopathology , which then became the Journal of Neurology, Neurosurgery and Psychiatry .4 It is, however, his two-volume textbook Neurology published posthumously in 1940 that stands as the greatest testament to the breadth of his clinical and scientific insight. Chapter 64 demonstrates his extraordinary understanding of the clinical heterogeneity of ALS, in which he highlights five ‘puzzling features’: Figure 1 Samuel Alexander Kinnier Wilson (1878–1937) was the founding editor, in 1920, of what became the Journal of Neurology, Neurosurgery and Psychiatry . 1. implication of certain afferent tracts and of ventro-lateral columns in the cord; 2. irregular degree of morbid change as between spinal nerve cell, nerve fibre and muscle, respectively; 3. similar disparity as regards Betz cell and pyramidal fibre lesions; 4. the acuteness of many cases, suggesting a toxic and diffuse process, yet the lesions appear to be systematised; 5. occasional discord between clinical and pathological findings. Intrigued by the issues raised by each of his five comments, we here discuss them in light of developments in understanding of the syndrome of ALS during the subsequent 75 years.5 ### Implication of afferent tracts and ventro-lateral columns in the cord Kinnier Wilson set out his thoughts on ALS nearly half a century after Charcot’s description. The latter recognised ALS as what he considered to be a uniquely ‘deuteropathic’ condition, one characterised by combined lower motor neuron (LMN) and …
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.004 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.002 |
| Scholarly communication | 0.001 | 0.002 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.004 | 0.002 |
| Insufficient payload (model declined to judge) | 0.001 | 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 source (direct Gemma or distilled Codex), 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".