We retrospectively reviewed the hospital records of 53 patients admitted for 73 episodes of myasthenic crisis at Columbia-Presbyterian Medical Center over a period of 12 years, from 1983 to 1994. Median age at the onset of first crisis was 55 (range, 20 to 82), the ratio of women to men was 2:1, and the median interval from onset of symptoms to first crisis was 8 months. Infection (usually pneumonia or upper respiratory infection) was the most common precipitating factor (38%), followed by no obvious cause (30%) and aspiration (10%). Twenty-five percent of patients were extubated at 7 days, 50% at 13 days, and 75% at 31 days; the longest crisis exceeded 5 months. Using survival analysis and backward stepwise Cox regression, we identified three independent predictors of prolonged intubation: (1) pre-intubation serum bicarbonate > or = 30 mg/dl (p = 0.0004, relative hazard 4.5), (2) peak vital capacity day 1 to 6 post-intubation < 25 ml/kg (p = 0.001, relative hazard 2.9), and (3) age > 50 (p = 0.01, relative hazard 2.4). The proportion of patients intubated longer than 2 weeks was 0% among those with no risk factors, 21% with one risk factor, 46% with two risk factors, and 88% with three risk factors (p = 0.0004). Complications independently associated with prolonged intubation included atelectasis (p = 0.002), anemia treated with transfusion (p = 0.03), Clostridium difficile infection (p = 0.01), and congestive heart failure (p = 0.03). Three episodes of crisis were fatal, for a mortality rate of 4% (3/73); four additional patients died after extubation. All seven deaths were due to overwhelming medical comorbidity. Over half of those who survived were functionally dependent (home or institutionalized) at discharge. In addition to prospective controlled studies of immunotherapies, the prevention and treatment of medical complications offers the best opportunity for further improving the outcome of myasthenic crisis.

a-Tropomyosin exons 2 and 3 are spliced in a mutually exclusive manner. Exon 3 is included as the default exon in the mRNA of most cell types, whereas exon 2 is only included in the mRNA of smooth muscle cells. The primary determinant for the default selection of exon 3 is the branchpoint/polypyrimidine tract. This element upstream of exon 3 clearly and effectively outcompetes the corresponding element upstream of exon 2. To identify trans-acting factors that bind to this important cis element, we used UV cross-linking to identify a 57-kD protein whose binding characteristics directly correlate with 3'-splice-site selection in cis-competition splicing assays. This protein appears to be identical to polypyrimidine tract-binding protein. In this report we have used oligonucleotides derived from peptide sequences to isolate and sequence cDNA clones encoding this 57.2-kD protein. The primary sequence reveals a novel protein with significant homology to other RNA-binding proteins. Expression of the mRNA is detected in all tissues and cells examined, although its levels exhibit tissue-specific and developmental regulation. Using a biochemical complementation assay, we have found that this protein, along with a 100-kD protein, exists as part of a large complex that is required to rescue splicing from depleted nuclear extracts.

We have determined the amino acid sequence of the Ca2+‐dependent cell adhesion molecule uvomorulin as it appears on the cell surface. The extracellular part of the molecule exhibits three internally repeated domains of 112 residues which are most likely generated by gene duplication. Each of the repeated domains contains two highly conserved units which could represent putative Ca2+‐binding sites. Secondary structure predictions suggest that the putative Ca2+‐binding units are located in external loops at the surface of the protein. The protein sequence exhibits a single membrane‐spanning region and a cytoplasmic domain. Sequence comparison reveals extensive homology to the chicken L‐CAM. Both uvomorulin and L‐CAM are identical in 65% of their entire amino acid sequence suggesting a common origin for both CAMs.

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The authors identified predictors of functional disability and mortality after status epilepticus in a multivariate analysis of 83 episodes in 74 patients. Twenty-one percent (14/85) of episodes were fatal. Increased age (OR = 1.1; 95% CI, 1.0 to 1.1) and acute symptomatic seizures (OR = 6.0; 95% CI, 1.2 to 30.3) were predictors of mortality. Functional outcome at discharge deteriorated in 23% (16/69) of nonfatal episodes. Increased length of hospitalization (OR = 1.04; 95% CI, 1.0 to 1.1) and acute symptomatic seizures (OR = 3.9; 95% CI, 1.0 to 14.7) were predictors of functional disability.

Objective: To analyze the impact of inflammation and negative nitrogen balance (NBAL) on nutritional status and outcomes after subarachnoid hemorrhage (SAH).Methods: This was a prospective observational study of SAH patients admitted between May 2008 and June 2012. Measurements of C-reactive protein (CRP), transthyretin (TTR), resting energy expenditure (REE), and NBAL (g/day) were performed over 4 preset time periods during the first 14 postbleed days (PBD) in addition to daily caloric intake. Factors associated with REE and NBAL were analyzed with multivariable linear regression. Hospital-acquired infections (HAI) were tracked daily for time-to-event analyses. Poor outcome at 3 months was defined as a modified Rankin Scale score $4 and assessed by multivariable logistic regression.Results: There were 229 patients with an average age of 55 6 15 years. Higher REE was associated with younger age (p 5 0.02), male sex (p , 0.001), higher Hunt Hess grade (p 5 0.001), and higher modified Fisher score (p 5 0.01). Negative NBAL was associated with lower caloric intake (p , 0.001), higher body mass index (p , 0.001), aneurysm clipping (p 5 0.03), and higher CRP:TTR ratio (p 5 0.03). HAIs developed in 53 (23%) patients on mean PBD 8 6 3. Older age (p 5 0.002), higher Hunt Hess (p , 0.001), lower caloric intake (p 5 0.001), and negative NBAL (p 5 0.04) predicted time to first HAI. Poor outcome at 3 months was associated with higher Hunt Hess grade (p , 0.001), older age (p , 0.001), negative NBAL (p 5 0.01), HAI (p 5 0.03), higher CRP:TTR ratio (p 5 0.04), higher body mass index (p 5 0.03), and delayed cerebral ischemia (p 5 0.04).Conclusions: Negative NBAL after SAH is influenced by inflammation and associated with an increased risk of HAI and poor outcome. Underfeeding and systemic inflammation are potential modifiable risk factors for negative NBAL and poor outcome after SAH. Neurology ® 2015;84:680-687 GLOSSARY CRP 5 C-reactive protein; DCI 5 delayed cerebral ischemia; HAI 5 hospital-acquired infection; ICU 5 intensive care unit; IDC 5 indirect calorimetry; mRS 5 modified Rankin Scale; NBAL 5 nitrogen balance; PBD 5 postbleed day; REE 5 resting energy expenditure; SAH 5 subarachnoid hemorrhage; SHOP 5 SAH outcomes project; TTR 5 transthyretin; UUN 5 urine urea nitrogen.Aneurysmal subarachnoid hemorrhage (SAH) is a significant contributor to all stroke-related potential years of life lost before age 65 years.1 Much of this is attributed to delayed cerebral ischemia (DCI).2 However, recent studies have found that both medical and infectious complications are significant independent contributors to morbidity and mortality after SAH.3-5 We previously found an association between poor nutritional status and infectious complications acutely after SAH. 6 Malnutrition has been associated with impaired immunologic function leading to increased rates of infection. 7 An assessment of nutritional profiles measured by indirect calorimetry (IDC) found SAH patients to have average resting energy expenditure (REE) rates ...

Five cases of presumed nicotine withdrawal delirium among brain-injured patients treated in a neurologic intensive care unit are presented. Each patient had a history of heavy tobacco use and experienced dramatic and sustained clinical improvement within hours of transdermal nicotine replacement. These preliminary observations suggest that nicotine withdrawal may be an under-recognized cause of delirium in patients with acute brain injury.

In contrast to other retrospective multisurgeon series, our retrospective single-surgeon series suggests that microsurgical fenestration of the lamina terminalis may not reduce the incidence of shunt-dependent hydrocephalus or cerebral vasospasm after aneurysmal subarachnoid hemorrhage. A prospective multicenter trial is needed to definitively address the use of this maneuver.