Successful Treatment of Super-Refractory Status Epilepticus With Cannabis Oil in a Patient With Neuronal Ceroid Lipofuscinosis
Bibliographic record
Abstract
Herein we describe a case of a 25-year-old man diagnosed with the “variant late infantile-onset” neuronal ceroid lipofuscinosis (NCL) who has a novel mutation in the CLN6 gene located on chromosome 15. The patient developed seizures at the age of 10 years along with progressive symptoms of ataxia, spasticity, cognitive decline and visual difficulties. At the age of 25 years, the seizures were drug-resistant, and the patient was put in an anesthesia-induced coma until the use of cannabis oil in combination with Food and Drug Administration (FDA)-approved anti-seizure drugs proved to be lifesaving. Treatment for NCL currently consists of symptomatic relief of seizures, and supportive therapies for associated symptoms of behavior, language and visual difficulties. Although limited, recent data on the efficacy of cannabinoids for treatment-resistant epilepsy have been published. Since cannabis oil is not currently FDA approved for most formulations, its use in drug-resistant epilepsy is anecdotal and remains highly controversial. J Neurol Res. 2020;10(6):245-247 doi: https://doi.org/10.14740/jnr626
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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) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.000 | 0.000 |
| 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".