A multinational consensus on dysphagia in Parkinson's disease: screening,diagnosis and prognostic value

Journal of Neurology - Parkinson’s disease (PD) is a neurodegenerative disorder characterized by a combination of motor and non-motor dysfunction. Dysphagia is a common symptom in PD, though...     

Effect of delayed blood processing on the yield of factor VIII in cryoprecipitate and factor VIII concentrate

Current standards for the preparation of factor VIII (FVIII) concentrates from human plasma recommend separation of plasma from red cells (RBCs) within 6 hours of blood donation, thereby reducing the volume of plasma from donated whole blood available for processing to FVIII concentrate. The decay of FVIII clotting activity (FVIII:C) in whole blood and plasma stored at 22 and 4 degrees C and the recovery of FVIII:C in cryoprecipitate and FVIII concentrate prepared from plasma separated from whole blood stored overnight at 4 degrees C were investigated. In whole blood stored at 22 degrees C and plasma stored at either 4 or 22 degrees C, 90 percent of the original FVIII:C was present at 6 hours, 80 percent at 12 hours, and 65 to 70 percent at 18 hours. At these times lower levels of FVIII:C were recovered from whole blood stored at 4 degrees C, that is, 84, 68, and 56 percent, respectively. In cryoprecipitates prepared from plasma separated from RBCs after 18 hours' storage at 4 degrees C (18-hour plasma), 43 percent of FVIII:C activity was recovered, as compared with 61 percent recovered from standard plasma separated within 6 hours of donation (6-hour plasma), p less than 0.05. With large-scale preparation of FVIII concentrates, however, the yield of FVIII:C was similar whether 18- or 6-hour plasma was used. Thus FVIII concentrates--but not cryoprecipitates--can be prepared from plasma separated from whole blood stored at 4 degrees C for up to 18 hours without undue loss of potency.     

The enhancer-like sequence 3' to the A gamma gene is polymorphic in human populations

Cloning and sequencing of the enhancer 3' of the A gamma globin gene of a particularly low G gamma and HbF sickle cell anemia (SCA) patient unexpectedly revealed three base changes (T----C, C----A, and A----G at sites +2285, +2460, and +2676) previously associated with the Seattle- type HPFH, thus leading the authors to suspect that the three mutations were polymorphic. The determination of the incidence of the mutations among various ethnic groups allowed the authors to conclude that this is a widely spread polymorphism, thus excluding any role of these base changes in the determination of the hereditary persistence of fetal hemoglobin (HPFH) phenotype. The origin of these three mutations is not clear because they appear linked, and the same bases (C, A, G) are found in homologous position in the 3' of the normal G gamma gene. As C, A, G at positions +2285, +2460, and +2676 are found with a 100% frequency in African SS patients and presumably among normal Africans (to explain the extremely high frequency among normal American blacks), it is likely that this was the sequence preceding the division of races. The presence of T, C, and A at the same positions apparently occurred after the divergence between blacks and the other races, that is, within the last 1 million years.     

Laparoendoscopic single-site surgery appendectomy

Background  

Laparoscopic appendectomy via the three-trocar technique is widely used for appendectomy. This report describes the initial experience with laparoendoscopic single-site surgery (LESS) appendectomy.     

A PRESsing Dissection

Background  

Intracranial dissections have been associated with baroreceptor reflex failure. When this occurs labile hypertension may be observed with its own complications, including but not limited to the clinico-radiographic entity, posterior reversible encephalopathy syndrome (PRES).     

Loss of A5 noradrenergic neurons in multiple system atrophy

The A5 noradrenergic group of the pons projects on the medulla and spinal cord and contributes to control of sympathetic activity and respiration. Because these functions are affected in multiple system atrophy (MSA), we sought to determine whether there was involvement of A5 neurons in this disorder. We counted tyrosine-hydroxylase (TH) immunoreactive neurons in the A5 area and locus ceruleus in the pons obtained from six patients with clinical and neuropathological diagnoses of MSA and six age-matched controls using stereological methods. There was a severe loss of A5 neurons in MSA (total cell number 3,215 +/- 258 in controls and 321 +/- 62 in MSA, P < 0.001). The severity of cell loss was comparable to that of locus ceruleus neurons in these cases. Our results indicate that A5 neurons are severely affected in MSA and this may contribute to some of the manifestations of this synucleinopathy.     

Neurodegeneration involving putative respiratory neurons in Perry syndrome

The objective of this study was to assess the potential involvement of ventral medullary neurons implicated in respiratory rhythmogenesis and chemosensitivity in a patient with Perry syndrome (autosomal dominant parkinsonism associated with depression, weight loss and central hypoventilation). Previous neuropathologic reports in Perry syndrome demonstrated neuronal loss in the substantia nigra with no or few Lewy bodies and no tau inclusions. Neurons in the pre-Bötzinger complex (preBötC) of the ventrolateral medulla, identified by their immunoreactivity for neurokinin-1 receptors (NK-1R), play an essential role in respiratory rhythmogenesis and serotonergic neurons in the medullary raphe in respiratory chemosensitivity, but their potential involvement in Perry syndrome has not yet been addressed. We conducted clinical and neuropathologic studies including immunohistochemistry examination in a new autopsied case clinically diagnosed as Perry syndrome. Our patient presented with parkinsonism at age 41. Subsequently, all cardinal features of Perry syndrome developed. He died of respiratory failure and sepsis at age 46. Hematoxylin-eosin staining revealed no significant pathology in the medulla. However, NK-1R, tyrosine hydroxylase (TH) and tryptophan hydroxylase (TrOH) immunoreactive neurons were significantly reduced in the ventrolateral medulla compared to controls. There was also loss of serotonergic neurons in the medullary raphe and ventral medullary surface. Severe neuronal loss in the substantia nigra, without alpha-synuclein or tau pathology but with loss of NK-1R and TH immunoreactive neurons in the ventrolateral medulla, and loss of serotonergic neurons in the medullary raphe and ventrolateral medulla may be a pathologic hallmark of Perry syndrome.