Long-term survival, prognosis, and life-care planning for 29 patients with chronic locked-in syndrome.

We present a life-table analysis of a cohort of 29 locked-in syndrome (LIS) patients followed for a minimum of five years, and we report on the status of the chronic LIS patient. Twenty-nine LIS patients who remained locked-in for more than one year were identified. Inpatient charts were reviewed for demographic, medical, and functional data. Telephone followup was obtained to examine medical complications after discharge, survival, neurologic recovery, care issues, and permanent disposition. A life-table analysis was performed on survival data. Cerebrovascular disease was the most common cause of LIS. Survival ranged from 2.02 to 18.15 years. Twenty of the 26 patients available for five-year followup survived; hence, five-year survival was 81%. An alternative method of communication and emotional stress for the patient's caregiver was the key issue in patient care. Most patients were cared for in their own homes. Although minimal late neurologic recovery occurs in chronic LIS, survival may, nonetheless, be prolonged with adequate supportive care. Modern computerized technology offers LIS patients the ability to interact with their environment. This information may assist physicians in making ethical and long-term care decisions with the patient rather than for the patient with LIS.     

Two novel β-thalassemia alleles: Poly A signal (AATAAA→AAAA) and −92 C→T

β-Thalassemia (β-thal) is a hereditary anemia caused by mutations in the β-globin gene encoding the β-globin chain of hemoglobin A. In its homozygous form, the disease is characterized by a hemolytic hypochromic anemia, hepatosplenomegaly, skeletal deformation, mongoloid facies, and cardiac enlargement. The heterozygous form is usually asymptomatic but is sometimes associated with moderate anemia and splenomegaly. More than 100 β-thal mutations have been detected in the β-globin gene (Kazazian, 1990). Here we report two new β-thal mutations and observation of another rare allele, which was found previously in a single individual. © 1995 Wiley-Liss, Inc.     

IgE-mediated release of rat mast cell protease II, beta-hexosaminidase and leukotriene C4 from cultured bone marrow-derived rat mast cells.

Functional characteristics of cultured bone marrow-derived rat mast cells (BMMC) were studied. BMMC were shown to release in a time- and dose-dependent fashion the mucosal mast cell (MMC)-specific enzyme, rat mast cell protease II (RMCPII), following IgE-mediated activation in vitro. RMCPII release was temporally associated with that of the mast cell granule-derived enzyme, beta-hexosaminidase (beta-hex). Release of the pre-formed granule constituents, RMCPII and beta-hex, was associated with the generation of the membrane-derived lipid mediator, leukotriene C4 (LTC4) and, in older cultures, substantial amounts were generated (25.2 ng/10(6) BMMC). Absolute amounts of RMCPII, beta-hex and LTC4 released were dependent upon the age of the BMMC. These results extend our previous observations on the staining properties and protease content of rat BMMC and provide evidence that these cells are functionally, as well as histochemically, analogous to the MMC subset, which is so prominent during intestinal nematode infections in rats.     

Computerized photo-plethysmography of the finger

A microcomputer system for studying photo-plethysmography of the finger (PPF) was designed and applied to 50 non-premedicated healthy boys (one to ten years old) undergoing general anaesthesia (halothane in 70% N₂O, with mechanical ventilation) for outpatient inguinal hernia repair. The purpose of this study was to assess the accuracy of computerized estimations of the photo-plethysmographic (arterial waves) amplitude and to evaluate whether or not PPF allows discrimination between two different surgical stimuli (skin incision, and manipulation of the spermatic cord). When anaesthesia was stable for at least five minutes (end-tidal halothane=1.25–1.5%;PetCO₂=32–38 mmHg; SpO₂≥98%; rectal temperature=36.3–37°C; ambient operating room temperature=20–21°C), and immediately before the skin incision, computerized estimations of the photo-plethysmographic (arterial waves) amplitudes (PPA) were recorded and saved for later comparison with direct (manual) measurements of the plethysmographic tracing, using an arbitrary scale of 0–255 units. Also, the values of PPA, systolic blood pressure, and pulse rate recorded immediately before the skin incision were later compared with the maximum changes in these same values recorded 30–90 sec after skin incision, and 30–90 sec after manipulation (traction + dissection) of the spermatic cord. Six boys (three to ten years old) stayed quiet enough, during induction of anaesthesia by mask, to allow regression analysis of PPA, systolic blood pressure, and pulse rate (Y) on end-tidal halothane/70% N₂O (X). Computerized estimations tended to give a higher reading, by between 0.2 to 0.8 units, than direct measurements. Spearman and Kendall correlations showed that computerized and direct measurements were associated (P<0.0001), the Kolmogorov-Smirnov’s test revealed that the two distributions were identical (P=1), the mean difference between computerized and direct estimations of the PPA was 0.52±1.08 units, and the limits of agreement (?1.6 and 2.6 units) were small enough to be confident that computerized (automatic) estimations of PPA can be used for clinical purposes. Skin incision caused a smaller decrease of PPA (24%) than manipulation of the spermatic cord (37%). Changes in PPA were more pronounced than changes in systolic blood pressure or pulse rate (P<0.05). Linear regressions and Fisher’s exact test (two-tailed) showed that, during induction of anaesthesia with halothane in 70% N₂O by mask (n=6), changes in end-tidal halothane concentration were related more to changes in PPA than to changes in systolic blood pressure and/or in pulse rate (P<0.05). In conclusion, computerized PPF allows discrimination between two different surgical stimuli, provides quantification of the sympathetic response to preoperative anxiety, and may be useful for studying pre-anaesthetic sedation.     

Synchronous cortical gamma-band activity in task-relevant cognition

Widespread synchronous oscillatory activity, particularly in the gamma ('40 Hz') band, has been postulated to exist in the brain as a mechanism underlying binding. A new method of examining phase synchronicity across multiple electrode sites in specific EEG frequency bands as a function of time was employed, in a conventional cognitive ERP paradigm in 40 normal subjects. A significant late post-stimulus gamma synchronicity response occurred for task-relevant stimuli, whereas for task-irrelevant stimuli no such response was evident. However, an early response was seen for both task-relevant and irrelevant stimuli. This is the first empirical demonstration that widespread synchronous high frequency oscillations occur in humans in relation to cognition.