Relation of QRS duration to mortality in a community-based cohort with hypertrophic cardiomyopathy

A prolonged QRS duration on the standard electrocardiogram is associated with an increased risk of cardiovascular death in cardiomyopathies of different origin. However, the relation between QRS duration and prognosis in hypertrophic cardiomyopathy (HC) remains undefined. We assessed the relation between QRS duration and cardiovascular death in 241 consecutive patients with HC. The study cohort was divided into 2 groups according to QRS duration: <120 and > or =120 ms. Of the 241 patients, 191 (79%) had a QRS duration <120 ms and 50 (21%) a QRS duration > or =120 ms. During a mean follow-up of 7.9 +/- 5.1 years, 35 patients died of cardiovascular causes related to HC. Of these 35 patients, 13 (6%) had a QRS duration <120 ms and 22 (43%) had a QRS duration > or =120 ms (p <0.01). Risk of cardiovascular death was significantly higher in patients with a QRS duration > or =120 ms than in those with a QRS duration <120 ms (relative risk 5.2, p <0.0001). At 8-year follow-up, cumulative risks of HC-related death were 7.1% in patients with a QRS duration <120 ms and 55% in those with a QRS duration > or =120 ms. Multivariate analysis confirmed that a QRS duration > or =120 ms was independently associated with an increased risk of cardiovascular death (hazard ratio 3.2, p = 0.007). New York Heart Association functional class III/IV was the only other clinical variable significantly and independently associated with an increased risk of cardiovascular death. In conclusion, in patients with HC, QRS duration on standard electrocardiogram is directly related to cardiovascular mortality, and a QRS duration > or =120 ms is a strong and independent predictor of prognosis.     

Effect of the 50 bp deletion polymorphism in the SOD1 promoter on SOD1 mRNA levels in Italian ALS patients

The genetic association between homozygosity for a 50 bp deletion polymorphism in the SOD1 promoter, 1684 bp upstream of the ATG, and an increased age of symptom onset was observed in various populations of ALS patients. Moreover, it has been demonstrated that this deletion reduces SOD1 expression in vitro. The objective of the present study was to test whether the observed association is replicated in patients from an Italian population and to check whether the deletion correlates with reduced SOD1 mRNA expression in vivo. Genomic DNA from 235 Italian SALS cases and 245 age- and sex-matched donors from the same ethnic background was screened for the 50 bp SOD1 promoter deletion by real time PCR assays. No differences were observed between ALS patients and controls for the frequency of both the alleles (D=deleted, N=non-deleted; p=0.95) and genotypes (p=0.90). Furthermore, stratification of the ALS samples showed that this variation was not associated with increased age of onset in ND and DD patients in comparison to NN patients (p=0.48). Finally, we performed real-time RT-PCR to quantify SOD1 mRNA levels in 48 patients and we did not find a relevant difference among the three sub-groups of genotypes (p=0.30). Our data suggest that the studied polymorphism does not modulate SOD1 mRNA level and disease phenotype in an Italian population.     

T-cell tumor necrosis factor-alpha receptor binding in myasthenic patients.

Myasthenia gravis (MG) is a T-cell-dependent and antibody-mediated autoimmune disease of the neuromuscular junction, in which the cytokine network may be deranged. Specific receptors for tumor necrosis factor (TNF)-alpha, a cytokine with several effects on the neuroimmune system, were found on human lymphocytes. In the present study, we assayed TNF-alpha binding on peripheral blood T-cells from MG patients, finding that T-cells from patients have significantly more TNF-alpha receptors than those from controls (Bmax: 654 +/- 12 vs. 133 +/- 4 (mean +/- SEM) receptors/cell). Such TNF-alpha binding sites are of the same type in patients and healthy subjects (Kd: 68.7 +/- 4.3 vs. 70.1 +/- 4.8 (mean +/- SEM) pM). The enhanced T-cell TNF-alpha binding is due to an increased number of TNF-alpha receptors on T-helper lymphocytes. These results are discussed in terms of MG immunopathogenesis, since it has been reported that activated T-cells have increased amounts of TNF-alpha receptors.     

Acute subdural hematoma of arteriolar origin. Traumatic or spontaneous?

Acute subdural hematomas are usually related to the rupture of a bridging vein with or without tearing of brain matter. In some instances the origin of the hemorrhage is due to the shearing of a cortical arteriole bleeding directly into the subdural space, generally after a minor head trauma. Ten cases were reviewed at Geneva's Neurosurgical clinic over a period of thirteen years (1973-1986). They represent less than 5% of all acute and subacute subdural hematomas treated during that period. Subdural hematomas of venous origin are usually encountered on previously normal brain whereas those of arteriolar origin are thought to result from the disruption of a small arteriolar knuckle adhering to the arachnoid and dura. While the majority of arteriolar hematomas reported here and in the literature are of traumatic origin, some are undoubtedly spontaneous. The clinical features of these hematomas are characterized by a high incidence of falsely localising motor signs, presumably due to the so called Kernohan's phenomenon. Their treatment requires a wide exposure, allowing identification and coagulation of the bleeding source located in most instances around the Sylvian fissure. Although a rare entity, the existence of arteriolar subdural hematomas must be emphasized, since any delay in their treatment may result in a dramatic outcome.     

Are commercial warming kits interchangeable for vitrified human blastocysts? Further evidence for the adoption of a Universal Warming protocol

Journal of Assisted Reproduction and Genetics - To study whether a new combination of different warming kits is clinically effective for vitrified human blastocysts. This is a longitudinal cohort...     

Superposition of ballistic on steady contractions in man

Summary In a visual reaction time task, human subjects superimposed isometric ballistic contractions on a maintained activity in the soleus or anterior tibial muscle. Since there were good reasons to believe that the supraspinal motor commands for the ballistic contractions were independent of those for the background activity, the interaction between the motor commands for the ballistic and for the steady contractions could be studied at the spinal level. If ballistic and steady contractions were in the same direction, the EMG burst and torque changes associated with the ballistic contraction were nearly constant irrespective of the maintained steady flexion force. This was true if a muscle was activated to about 5% of its maximum force as the soleus muscle during plantar flexions and if it was activated to about 40% of its maximum force as the anterior tibial muscle during dorsal flexions. If ballistic and steady contractions were in opposite directions the torque changes related to the ballistic contraction increased linearly with the background activity. This relation was caused by a reduction in antagonist activity starting about 50 ms before the agonist EMG burst and not by an increased agonist burst, the latter remaining independent of background activity. These results imply that the input — output relationship of the motoneuronal pool is nearly linear. The functional basis of this relation is the size principle which is valid during continous and ballistic contractions. The number of motor units recruited for the ballistic contraction is adjusted according to their force such that the contraction amplitude remains constant.     

Platelet phenolsulphotransferase activity in patients with dementia of Alzheimer type

Human blood platelets are more and more regarded as a reliable model of nerve cells, and some biochemical alterations found in platelet enzyme activities and receptor binding parameters may be related to analogous changes occuring in the central nervous system. Platelet phenol-sulphotransferase activity has been evaluated in some neurological disorders, such as Parkinson's disease and headache. In the present work we determined phenolsulphotransferase activity in platelets of 36 patients with dementia of Alzheimer type, and compared such activity values with those of 36 sex- and age-matched healthy controls. Patients showed a significantly higher platelet phenolsulphotransferase activity than control subjects, both with dopamine and with phenol as substrates. Moreover, a significant positive correlation was found between platelet enzyme activity and severity of illness. These findings are discussed in terms of the biochemical abnormalities found in the central nervous system of demented subjects, and in terms of the possible pathogenetic involvement of an altered phenolsulphotransferase activity (and, in general, of a modified monoamine metabolism) in such derangements of biochemical systems often occuring in dementia of Alzheimer type.     

Sudden cervical pain: spontaneous cervical epidural hematoma

Three cases of cervical epidural hematoma are reported. Acute neck pain usually associated with a mild effort, closely followed by radicular pain and a neurologic deficit below the lesion is the typical presentation of this extremely rare and difficult diagnosis. As prognosis depends on preoperative neurologic state, the authors emphasize the importance of prompt identification of this lesion. The diagnosis is confirmed by computed tomography, and emergency neurosurgical laminectomy is mandatory.     

Platelet and Lymphocyte Benzodiazepine Binding in Patients with Alzheimer''s Disease

Peripheral blood cells, such as platelets or lymphocytes, have been studied in the investigation of systemic derangements and central nervous system biochemical changes occurring in several neuropsychiatric disorders. In the present work, assaying platelet and lymphocyte peripheral-type benzodiazepine binding in patients with Alzheimer's disease (AD) and healthy controls, we found a significantly reduced number of cell receptors in patients' platelets and lymphocytes. These results are discussed with reference to central nervous system biochemical abnormalities in AD. Moreover, the lymphocyte binding data may represent an impairment of the immune response in AD, since lymphocyte surface peripheral-type benzodiazepine receptors seem to be related to immune function.