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Record W4252424258 · doi:10.5334/tohm.372

The Movement Disorder of Brain-Lung-Thyroid Syndrome Can be Responsive to Methylphenidate

2017· article· en· W4252424258 on OpenAlexafffund
Laurence Gauquelin, Luan T. Tran, Sylvain Chouinard, Geneviève Bernard

Bibliographic record

VenueTremor and Other Hyperkinetic Movements · 2017
Typearticle
Languageen
FieldMedicine
TopicNeurological and metabolic disorders
Canadian institutionsMontreal Children's HospitalMcGill University Health Centre
FundersActelion PharmaceuticalsFonds de Recherche du Québec - SantéCanadian Institutes of Health ResearchAllerganIpsen
KeywordsMethylphenidateThyroidMedicineLungNeuroscienceInternal medicinePsychologyAttention deficit hyperactivity disorderPsychiatry

Abstract

fetched live from OpenAlex

Benign hereditary chorea is a rare disorder characterized by childhoodonset, non-progressive chorea, with or without associated respiratory and thyroid dysfunction.1 It is referred to as ‘‘brain-lung-thyroid’’ syndrome when all three systems are involved.2 It is caused by autosomal dominant mutations in the NKX2-1 gene (previously TITF-1), on chromosome 14.3 It is a genetically heterogeneous condition, with over 30 different causative mutations identified.4 Other neurological manifestations of benign hereditary chorea and NKX2-1-related disorders include dystonia, myoclonus, tics, tremor, dysarthria, ataxia, hypotonia, and motor developmental delay. Neuropsychiatric symptoms such as attention deficit hyperactivity disorder (ADHD), have also been reported.1,5 Pharmacologic treatment of chorea and other abnormal movements in NKX2-1-related disorders has been disappointing. It typically involves levodopa or tetrabenazine; however, side effects are often limiting.5 We report and illustrate the case of a young female patient with brain-lung-thyroid syndrome and an immediate improvement of her involuntary movements with methylphenidate (Video 1).

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 distilled prediction

Teacher imitation

Not 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.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.000
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.358
Threshold uncertainty score0.498

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.000
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.000
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0000.000

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 head0.023
GPT teacher head0.298
Teacher spread0.275 · 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 teacher head, not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
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

Citations3
Published2017
Admission routes2
Has abstractyes

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