Refractory Status Epilepticus Associated with Anti-SSA (anti-Ro) Antibodies
Bibliographic record
Abstract
There have been frequent recent descriptions of limbic encephalitis and medically refractory epilepsy secondary to antibodies to a broad variety of neuronal antigens, including Nmethyl-D-aspartate receptors (NMDAR), glutamic acid decarboxylase (GAD) and voltage-gated potassium channels (VGKC). 1 As a result, an intensive search for autoantibodies has become part of the diagnostic evaluation for unexplained refractory status epilepticus.We present two cases of sudden, severe limbic encephalitis and medically refractory status epilepticus associated with anti-SSA (Sjogren's-syndrome antigen A) antibodies, and discuss the potential implications of the diagnosis and management of new onset, cryptogenic status epilepticus. CASE REPORTS Patient #1A 27-year-old Hispanic woman with an unremarkable medical history presented with a two-day history of malaise, headache, fatigue and fever.On the day of admission she had frequent generalized tonic-clonic seizures.She was treated with lorazepam and phenytoin but continued to have seizures.She was intubated and placed on a continuous propofol infusion.A lumbar puncture demonstrated a mild lymphocytic pleocytosis and she was empirically started on acyclovir, ceftriaxone and vancomycin.Seizures continued, and carbamazepine was added to her regimen but a rash developed and it was discontinued.She was transferred to an academic medical center one week later for management of refractory status epilepticus.An electroencephalogram (EEG) performed on transfer showed nearly continuous generalized and bilaterally independent epileptiform discharges at 2-4 Hz, consistent with electrographic status epilepticus.Midazolam and pentobarbital continuous infusions were added and titrated to achieve EEG burst suppression.A magnetic resonance image (MRI), performed on transfer, revealed bilateral mesial temporal fluid-attenuated inversion recovery (FLAIR) signal abnormalities with increased volume of both hippocampi (Figure).Lumbar puncture revealed 12 WBCs per mm 3 , normal protein and glucose, and positive oligoclonal bands.Her antinuclear antibody titer was elevated at > 1:160 (normal <1:40).SS-A (Ro) and SS-B (La) antibodies were highly elevated at 15616 and 1664 U/mL (normal <20 U/mL), respectively.Remaining evaluation for infectious, autoimmune and paraneoplastic abnormalities was negative (Table ).Computed tomography of the chest, abdomen and pelvis and pelvic ultrasound revealed no abnormalities.She was treated with a course of intravenous methylprenisolone 1gm/day for five days followed by plasma Refractory Status Epilepticus Associated with Anti-SSA (anti-Ro) Antibodies
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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 | 0.002 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.002 | 0.001 |
| Insufficient payload (model declined to judge) | 0.004 | 0.001 |
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".