Consultant’s corner: the “breakthrough” seizure

Seizures are a common cause of emergency department visits. This article provides an algorithm for evaluation and management of the patient with a known seizure disorder who presents with a breakthrough event. If the seizure represents a significant deviation from the usual pattern or the patient has an established pattern of episodes of status epilepticus, early involvement of the consultant neurologist is warranted. Otherwise, the first step is to search for provocative influences such as fever, intercurrent illness, or non-compliance with prescribed anti-epileptic drugs. Measurement of drug levels will guide the next rational phase of management. The overwhelming majority of patients can be managed following this logical framework.     

Fatal pneumothorax in “BCNU lung”

A patient with recurrent glioma treated with BCNU developed pneumothorax and interstitial pulmonary fibrosis. She died from “BCNU lung” and its complications, although she was free of her initial disease.     

Multifactorial analysis of opsoclonus‐myoclonus syndrome etiology (“Tumor” vs. “No tumor”) in a cohort of 356 US children

1 Background

Pediatric opsoclonus‐myoclonus syndrome (OMS) presents a paradox of etiopathogenesis: A neuroblastic tumor (NB) is found in only one half of the cases, the others are ascribed to infections or designated as idiopathic.

2 Method

From an IRB‐approved observational study of 356 US children with OMS, secondary analysis of “etiology” and related factors was performed on a well‐characterized cohort. The “Tumor” (n = 173) and “No Tumor” groups (n = 183), as defined radiologically, were compared according to multiple factors considered potentially differentiating. Data were analyzed retrospectively using parametric and nonparametric tests as indicated.

3 Results

Patients with NB were not distinguishable by prodromal symptoms, OMS onset age, gender, race/ethnicity, OMS severity, rank order of neurological sign appearance, or geographic distribution. Various CSF immunologic biomarker abnormalities of OMS did not vary in the presence or absence of a detectable tumor: frequency of six lymphocyte subsets, or concentrations of 18 cytokines/chemokines, cytokine antagonists, chemokine receptors, cell adhesion molecules, or neuronal/glial markers. Prior responsiveness to conventional immunotherapy was not contingent on tumor/no tumor designation.

4 Conclusions

Multiple convergent factors provide compelling empirical evidence and rationalize the concept that OMS is one neurological disorder, regardless of apparent etiology. Limitations to the current clinical etiologic classifications as paraneoplastic, parainfectious/post‐infectious, and idiopathic etiology require antigen‐based biological solutions to tease out the molecular pathophysiology of viral/tumoral mechanisms. Systematic studies, regardless of presumed etiology, will be necessary to find the highest‐yield combination of imaging approaches, screening for infectious agents, and new biomarkers. Two testable hypotheses for future research are presented.