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Record W2547424619 · doi:10.1136/jmedgenet-2016-104148

The cerebellum and embodied semantics: evidence from a case of genetic ataxia due to <i>STUB1</i> mutations

2016· article· en· W2547424619 on OpenAlexaboutno aff
Adolfo M. García, Sofía Abrevaya, Giselle Kozono, Indira García‐Cordero, Marta Córdoba, Marcelo Kauffman, Ricardo Marcos Pautassi, Édinson Muñoz, Lucas Sedeño, Agustín Ibáñez

Bibliographic record

VenueJournal of Medical Genetics · 2016
Typearticle
Languageen
FieldNeuroscience
TopicGenetic Neurodegenerative Diseases
Canadian institutionsnot available
FundersFondo de Financiamiento de Centros de Investigación en Áreas PrioritariasComisión Nacional de Investigación Científica y TecnológicaFondo para la Investigación Científica y TecnológicaConsejo Nacional de Investigaciones Científicas y TécnicasFundación INECO
KeywordsCerebellumAtaxiaEmbodied cognitionSemantics (computer science)MutationBiologyGeneticsNeuroscienceComputer scienceGene

Abstract

fetched live from OpenAlex

Abundant research on lexicosemantic processing indicates that damage to movement-related regions (the motor and premotor cortices, Broca´s area and the basal ganglia1) distinctively impairs processing of action verbs, that is, verbs denoting bodily motion. Moreover, such deficits could be hereditary,2 suggesting an association with genetic factors. We, thus, hypothesised that genetically based deterioration of other motor regions could involve similar impairments. In particular, through a combination of structural and functional MRI (fMRI) with genetic and behavioural analysis, this case study indicates that distinctive action-verb deficits can also be linked to genetic mutations affecting the cerebellum, a key motor hub implicated in balance, posture and movement coordination. Accordingly, in line with the embodied cognition framework, our data illuminate a potential functional specialisation of the cerebellum within the lexicosemantic domain.To test our hypothesis, we profited from access to a unique case of genetic ataxia and assessed action-verb processing together with cerebellar atrophy and related functional connectivity. The patient is a 26-year-old, Spanish-speaking male, with 13 years of education and a normal score (26/30) on the Montreal Cognitive Assessment (MoCA). He was diagnosed with cerebellar ataxia plus myoclonus, and exome sequencing revealed novel compound heterozygous mutations in the STUB1 gene3 (see online supplementary appendix e-1). His condition is characterised by severe dysarthria, action and postural tremor in the upper limbs, abnormalities of manual and facial movements and progressive disturbances of balance and gait.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame machine prediction

Teacher imitation

Not 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.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.003
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Case report · Consensus signal: Case report
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.008
Threshold uncertainty score0.017

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0000.003
Meta-epidemiology (narrow)0.0020.001
Meta-epidemiology (broad)0.0010.001
Bibliometrics0.0030.001
Science and technology studies0.0010.003
Scholarly communication0.0010.001
Open science0.0010.001
Research integrity0.0040.001
Insufficient payload (model declined to judge)0.0020.000

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.

Opus teacher head0.039
GPT teacher head0.311
Teacher spread0.272 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designCase report
Domainnot available
GenreEmpirical

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".

Quick stats

Citations65
Published2016
Admission routes1
Has abstractyes

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