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Record W4283382664 · doi:10.1017/cjn.2022.183

P.087 The landscape of paediatric tuberous sclerosis complex (TSC) neurological care in canada: results from a national survey

2022· article· en· W4283382664 on OpenAlexaffvenueabout
M Nabavi Nouri, M Zak, R Whitney, D Haile

Bibliographic record

VenueCanadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques · 2022
Typearticle
Languageen
FieldMedicine
TopicTuberous Sclerosis Complex Research
Canadian institutionsSystems, Applications & Products in Data Processing (Canada)Toronto Public Health
Fundersnot available
KeywordsTuberous sclerosisMedicineEpilepsyVigabatrinNeurological problemsPediatricsPsychiatry

Abstract

fetched live from OpenAlex

Background: Tuberous Sclerosis Complex (TSC) is a genetic disease that affects multiple body systems with the neurological manifestations causing the greatest disease burden. The objective of this study was to understand the scope of neurological TSC care delivery across Canada. Methods: A survey was developed after literature review and discussion amongst two Paediatric Epileptologists and one Nurse Practitioner with expertise in TSC. Canadian Paediatric Neurologists participated via an anonymous web-based survey through the Canadian League Against Epilepsy (CLAE) and Canadian Neurological Sciences Federation (CNSF). Results: Fifty-eight responses were received. A dedicated TSC clinic was reported by 24% (n=14). Sixty percent (n= 35) reported preforming serial screening EEG monitoring in infants and 58% (n= 34) started prophylactic therapy when EEG abnormalities occurred. Vigabatrin was used in 37% (n=21). For management of drug-resistant epilepsy, surgery was reported as the preferred therapeutic option in 57% (n=32) of respondents. Barriers to treatment identified were a lack of multi-disciplinary care, unfamiliarity with new therapies and insufficient resources. Conclusions: Our findings demonstrate the variability in neurological care delivery of patients with TSC. With few dedicated TSC clinics, there is a need for the establishment of a national network to support clinical practice, research and education.

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)0.008
metaresearch head score (Gemma)0.007
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesScience and technology studies, Research integrity
Consensus categoriesScience and technology studies
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.289
Threshold uncertainty score1.000

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0080.007
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0010.000
Bibliometrics0.0010.003
Science and technology studies0.0030.004
Scholarly communication0.0000.000
Open science0.0030.000
Research integrity0.0000.003
Insufficient payload (model declined to judge)0.0010.000

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 head0.119
GPT teacher head0.291
Teacher spread0.172 · 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; both teacher heads agree on what is shown here.

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

Citations0
Published2022
Admission routes3
Has abstractyes

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Same venueCanadian Journal of Neurological Sciences / Journal Canadien des Sciences NeurologiquesSame topicTuberous Sclerosis Complex ResearchFrench-language works237,207