Mutism Caused by Severe Demyelination in a Patient With Marchiafava-Bignami Disease.

BACKGROUND: Marchiafava-Bignami (MB) disease is a rare disorder that causes primary degeneration of the corpus callosum. It is associated with chronic alcohol consumption caused by either a toxic or nutritional etiology. CASE REPORT: We report a case of a 54-year-old woman who presented to our emergency department with complete mutism caused by MB disease that completely resolved with intravenous thiamine and dextrose therapy. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Emergency physicians frequently encounter patients with alcohol abuse and its complications. We report a rare presentation of a potential alcohol-related spectrum disease that may be encountered by an emergency physician. Early diagnosis and prompt management are critical to potentially reversing the disease, and this case shows the importance of including this disease in the differential diagnosis in patients with speech difficulty and alcohol abuse.

Bitter bottle gourd (Lagenaria siceraria) toxicity.

BACKGROUND: Bottle gourd (Lagenaria siceraria) is an edible plant in the Cucurbitaceae family. When extremely bitter, ingestion of bottle gourd can cause rapid onset diarrhea, vomiting, gastrointestinal bleeding, and hypotension due to release of a substance named cucurbitacin. OBJECTIVE: Our aim was to increase physician awareness of cucurbitacin poisoning in order to facilitate accurate diagnosis and appropriate management. CASE REPORT: Five adult patients presented with nausea, vomiting, and diarrhea within 5 to 25 min of ingesting cooked bitter bottle gourd. One patient developed severe diarrhea, hematemesis, and hypotension requiring hospitalization. All patients improved within a few days with intravenous fluids and proton pump inhibitors. To our knowledge, this is the first reported group of patients with toxicity due to ingestion of bottle gourd in the United States (US). CONCLUSIONS: Physicians should be suspicious of cucurbitacin toxicity in patients who present with symptoms within minutes of ingestion of a plant in the Cucurbitaceae family. Patients should be asked if the plant tasted unusually bitter. The most common symptoms include diarrhea and hematemesis. More than half of patients develop hypotension. There is no known antidote for bottle gourd poisoning; treatment is supportive. Proton pump inhibitors should be given to patients with gastrointestinal mucosal injury.

Smoking among rural and urban young women in China.

METHODS: A survey of 11 095 urban and rural women attending high school or college, aged 14-24 years, in 6 Chinese provinces was conducted. Ever-smoking (ES), current smoking, established smoking and intention to smoke frequencies were calculated. Bivariate analyses were conducted to identify possible correlates of smoking, and those found to be significant at the p=0.1 level were included in a multivariate logistical regression model to obtain adjusted OR for correlates of ES. RESULTS: ES prevalence was 20.1%, with urban female students (UFS) at 22.0% and rural female students (RFS) at 19.0% (p<0.01). Established smoking prevalence was higher among UFS than RFS (2.4% vs 0.9%, p<0.01). Similarly, current smoking prevalence was higher among UFS than RFS (4.2% vs 1.9%, p<0.01). The intention to smoke prevalence was higher among UFS than RFS (3.5% vs 1.7%, p<0.01). The majority were aware that smoking was harmful to health, but were less aware of specific diseases associated with smoking. ES was associated with awareness of cigarettes made for women (OR, 1.66, 95% CI 1.49 to 1.86) and thinking that they were less harmful than other cigarettes (OR 1.54, 95% CI 1.33 to 1.79). The strongest correlate of ES was low refusal self-efficacy (OR 6.35, 95% CI 5.32 to 7.57). CONCLUSIONS: This is the first report that having heard of women's cigarettes is a correlate of smoking among young Chinese women. ES among young Chinese women has increased in the last decade, and thus, specific prevention strategies need to be developed to prevent the tobacco epidemic from spreading among this vulnerable population.

Two novel STK11 mutations in three Chinese families with Peutz-Jeghers syndrome.

BACKGROUND: Peutz-Jeghers syndrome (PJS) is an autosomal dominantly inherited disease. STK11/LKB1 gene germline mutations have been identified as responsible for PJS. In our study, we investigated the molecular basis of PJS and evaluated correlation between the STK11 mutations and the Chinese population. METHODS: We collected three pedigrees of PJS and screened the 9 exons and their flanking intronic sequences of STK11/LKB1 gene in the probands and normal individuals in the families using polymerase chain reaction (PCR) and direct sequencing. RESULTS: Sequencing of the STK11 gene in the probands of 3 families revealed two novel mutations (c180C-->G and c998-1002delGCAGC) in exon 1 and exon 8, respectively. The mutation of c180C-->G resulted in a premature termination codon. The other mutation, a deletion of five nucleotides (998-1002delGCAGC) in exon 8, predicted to generate a translational frameshift and a termination at codon 1070. CONCLUSIONS: The growing number of mutations in PJS pedigrees suggests the molecular basis of PJS. STK11 gene mutation can be detected in most patients with PJS.

AR2, a novel automatic muscle artifact reduction software method for ictal EEG interpretation: Validation and comparison of performance with commercially available software.

Objective: To develop a novel software method (AR2) for reducing muscle contamination of ictal scalp electroencephalogram (EEG), and validate this method on the basis of its performance in comparison to a commercially available software method (AR1) to accurately depict seizure-onset location. Methods: A blinded investigation used 23 EEG recordings of seizures from 8 patients. Each recording was uninterpretable with digital filtering because of muscle artifact and processed using AR1 and AR2 and reviewed by 26 EEG specialists. EEG readers assessed seizure-onset time, lateralization, and region, and specified confidence for each determination. The two methods were validated on the basis of the number of readers able to render assignments, confidence, the intra-class correlation (ICC), and agreement with other clinical findings. Results: Among the 23 seizures, two-thirds of the readers were able to delineate seizure-onset time in 10 of 23 using AR1, and 15 of 23 using AR2 (p<0.01). Fewer readers could lateralize seizure-onset (p<0.05). The confidence measures of the assignments were low (probable-unlikely), but increased using AR2 (p<0.05). The ICC for identifying the time of seizure-onset was 0.15 (95% confidence interval (CI), 0.11-0.18) using AR1 and 0.26 (95% CI 0.21-0.30) using AR2.  The EEG interpretations were often consistent with behavioral, neurophysiological, and neuro-radiological findings, with left sided assignments correct in 95.9% (CI 85.7-98.9%, n=4) of cases using AR2, and 91.9% (77.0-97.5%) (n=4) of cases using AR1. Conclusions: EEG artifact reduction methods for localizing seizure-onset does not result in high rates of interpretability, reader confidence, and inter-reader agreement. However, the assignments by groups of readers are often congruent with other clinical data. Utilization of the AR2 software method may improve the validity of ictal EEG artifact reduction.

A new identified complication of intracystic hemorrhage in a large pineal gland cyst.

Pineal gland cysts are typically asymptomatic, benign cysts most commonly found incidentally in adults. In rare cases, a large pineal gland cyst can be complicated by intracystic hemorrhage, which could then manifest with neurological symptoms. We report a new complication of intracystic hemorrhage in a large pineal gland cyst in a 40-year-old man with new onset seizures.

The molecular basis of odor coding in the Drosophila antenna.

We have undertaken a functional analysis of the odorant receptor repertoire in the Drosophila antenna. Each receptor was expressed in a mutant olfactory receptor neuron (ORN) used as a "decoder," and the odor response spectrum conferred by the receptor was determined in vivo by electrophysiological recordings. The spectra of these receptors were then matched to those of defined ORNs to establish a receptor-to-neuron map. In addition to the odor response spectrum, the receptors dictate the signaling mode, i.e., excitation or inhibition, and the response dynamics of the neuron. An individual receptor can mediate both excitatory and inhibitory responses to different odorants in the same cell, suggesting a model of odorant receptor transduction. Receptors vary widely in their breadth of tuning, and odorants vary widely in the number of receptors they activate. Together, these properties provide a molecular basis for odor coding by the receptor repertoire of an olfactory organ.