The neurophysiological mechanisms for persisting impairment of motor function after Guillain-Barr syndrome (GBS) were assessed in 37 unselected patients 1- 13 years after diagnosis. For evaluation of reinnervation a...The neurophysiological mechanisms for persisting impairment of motor function after Guillain-Barr syndrome (GBS) were assessed in 37 unselected patients 1- 13 years after diagnosis. For evaluation of reinnervation and axonal loss, macroelectromyography (macro-EMG) including measurement of fiber density (FD) was performed. Data from neuropathy symptom score, neuropathy disability score, nerve conduction studies, and quantitative sensory examination were ranked and s ummed to a neuropathy rank sum score (NRSS). The isokinetic muscle strength at t he ankle was measured. Signs of axonal loss with increase of either macro motor unit potential (macro-MUP) amplitude or FD occurred in 76% of patients. The macro-MUP amplitude correlated with muscle strength and with NRSS. Patients wi th evidence of residual neuropathy had increased macro-MUP amplitude and FD as well as decreased muscle strength compared to patients without evidence of res idual neuropathy. We conclude that axonal loss takes place in a substantial numb er of GBS patients and is associated with permanent muscle weakness caused by in sufficient reinnervation. Possible patterns of pathology are discussed in relati on to the macro-EMG findings.展开更多
The physiology of isolated partially anomalous pulmonary venous connection of a single pulmonary vein has yet to be fully characterized. This study assessed the magnitude of the left-to-right shunt and right ventricul...The physiology of isolated partially anomalous pulmonary venous connection of a single pulmonary vein has yet to be fully characterized. This study assessed the magnitude of the left-to-right shunt and right ventricular(RV) dilation from a single anomalous pulmonary vein using cardiac magnetic resonance imaging. Subjects with >1 anomalous pulmonary vein or associated lesions, including atrial septal defects, were excluded. In the 6 subjects identified, the median pulmonary-to-systemic flow ratio was 1.55(range 1.3 to 1.6). The mean RV end-diastolic volume indexed to body surface area in the subjects was significantly larger than in a normal reference cohort(108±16 vs 78±18 cm3/m2, p=0.0009) and greater than the upper limit of normal in all 6 subjects. Older age did not correlate with increased magnitude of shunting(r=0.3, p=0.5), but increased age did correlate with RV end-diastolic volume indexed to body surface area(r=0.96, p=0.01). Isolated partially anomalous pulmonary venous connection with only 1 vein connecting anomalously results in a modest left-to-right shunt and mild RV dilation.展开更多
PURPOSE: Solitary ulcer syndrome is a rare condition characterized by inflammation and chronic ulcer of the rectal wall in patients suffering from outlet constipation. Despite similar surgical options (rectopexy, ante...PURPOSE: Solitary ulcer syndrome is a rare condition characterized by inflammation and chronic ulcer of the rectal wall in patients suffering from outlet constipation. Despite similar surgical options (rectopexy, anterior resection), solitary ulcer syndrome may differ from overt rectal prolapse with regard to symptoms and pathogenesis. The present work analyzed differences between these conditions in a case-control physiology study. METHODS: From 1997 to 2002, 931 consecutive subjects were investigated in a single physiology unit for anorectal functional disorders. Standardized questionnaires, anorectal physiology, and evacuation proctography were included in a prospective database. Diagnosis of solitary ulcer syndrome was based on both symptoms and anatomic features in 25 subjects with no overt rectal prolapse (21 females and 4 males; mean age, 37.2 ±15.7 years) and no past history of anorectal surgery. They were compared with age-matched and gender-matched subjects: 25 with outlet constipation (also matched on degree of internal procidentia), 25 with overt rectal prolapse without any mucosal change, and 14 with overt rectal prolapse and mucosal changes. RESULTS: Subjects with solitary ulcer syndrome reported symptomatic levels (digitations, pain, incontinence) similar to those of patients with outlet constipation, but they had significantly more constipation and less incontinence than patients with overt rectal prolapse. Compared with each of the three control groups (dyschezia, rectal prolapse without mucosal change, and rectal prolapse with mucosal change), subjects with solitary ulcer syndrome more frequently had an increasing anal pressure at strain (15 vs. 5, 3, and 1, respectively ;P < 0.01) and a paradoxical puborectalis contraction (15 vs. 9, 1, and 1, respectively; P < 0.05). With respect to evacuating proctography, complete rectal emptying was achieved less frequently in this group (5 vs. 12, 23, and 10, respectively; P < 0.05). Compared with patients with overt rectal prolapse, mean resting and squeezing anal pressures were significantly higher in both groups of subjects with solitary ulcer syndrome and with outlet constipation. Prevalence and levels of anatomic disorders (perineal descent, rectocele) did not differ among the four groups except for rectal prolapse grade and prevalence of enterocele (higher in overt rectal prolapse group). Interestingly, and despite matched controls for degree of intussusception, individuals with solitary ulcer syndrome had circular internal procidentia more often compared with those suffering from outlet constipation without mucosal lesions (15 vs. 8, P < 0.05). CONCLUSION: This case-controlled study quantifies functional anal disorders in patients suffering from solitary ulcer syndrome. Despite no proven etiologic factor, sphincter-obstructed defecation and circular internal procidentia both may play an important part in the pathogenesis and an exclusive surgical approach may not be appropriate in this context.展开更多
Objective: Polyneuropathy, organomegaly, endocrinopathy,M protein, and skin ch anges (POEMS) syndrome is a rare cause of polyneuropathy. Although the polyneuro pathy component is essential for the diagnosis of the dis...Objective: Polyneuropathy, organomegaly, endocrinopathy,M protein, and skin ch anges (POEMS) syndrome is a rare cause of polyneuropathy. Although the polyneuro pathy component is essential for the diagnosis of the disease, the pattern of as sociated electrodiagnostic abnormalities has not been characterized in detail. T he purpose of this study was to elucidate the features of nerve conduction abnor malities in POEMS syndrome.Methods: We reviewed the medical records and nerve co nduction studies(NCS) of 12 consecutive patients with POEMS.Results: A total of 68 motor and 46 sensory nerves were examined.Compound muscle action potentials(C MAPs) and sensory nerve action potentials(SNAPs) were not elicited in lower limb s more frequently compared with in upper limbs (P < 0.05, in both motor and sens ory nerves), and the CMAP amplitude was more attenuated in lower limbs than in u pper limbs (P < 0.05). Abnormal conduction slowing was frequently observed in mo tor (95%) and sensory (76%) nerves. Distal motor latencies were abnormally pro longed in 75%of the motor nerves,and terminal latency indices were significant - ly higher in patients than in normal controls (P < 0.05). Conclusions: NCS in POEMS syndrome showed characteristic patterns, in which conduction abnormalities were more frequently and severely affected in the lower limbs, and more predomi nantly in the intermediate nerve segments than in the distal portions. Significa nce:The recognition of these characteristic patterns may be helpful for the earl y diagnosis of polyneuropathy in POEMS syndrome.展开更多
盐度对鱼类生态生理学作用的直接效应是引起鱼体对渗透压的调节,间接影响则表现为对鱼体与环境间物质交换与能量流动的影响(De Silva et al.,1976)。自上世纪50年代末开始,盐度一直是鱼类生理学和实验生物学的重要指标。为此,众多专家...盐度对鱼类生态生理学作用的直接效应是引起鱼体对渗透压的调节,间接影响则表现为对鱼体与环境间物质交换与能量流动的影响(De Silva et al.,1976)。自上世纪50年代末开始,盐度一直是鱼类生理学和实验生物学的重要指标。为此,众多专家就广盐性洄游种类鲑鱼生长与盐度的关系(Otto,1971;Shaw et al.,1975;MacLeod,1977;展开更多
文摘The neurophysiological mechanisms for persisting impairment of motor function after Guillain-Barr syndrome (GBS) were assessed in 37 unselected patients 1- 13 years after diagnosis. For evaluation of reinnervation and axonal loss, macroelectromyography (macro-EMG) including measurement of fiber density (FD) was performed. Data from neuropathy symptom score, neuropathy disability score, nerve conduction studies, and quantitative sensory examination were ranked and s ummed to a neuropathy rank sum score (NRSS). The isokinetic muscle strength at t he ankle was measured. Signs of axonal loss with increase of either macro motor unit potential (macro-MUP) amplitude or FD occurred in 76% of patients. The macro-MUP amplitude correlated with muscle strength and with NRSS. Patients wi th evidence of residual neuropathy had increased macro-MUP amplitude and FD as well as decreased muscle strength compared to patients without evidence of res idual neuropathy. We conclude that axonal loss takes place in a substantial numb er of GBS patients and is associated with permanent muscle weakness caused by in sufficient reinnervation. Possible patterns of pathology are discussed in relati on to the macro-EMG findings.
文摘The physiology of isolated partially anomalous pulmonary venous connection of a single pulmonary vein has yet to be fully characterized. This study assessed the magnitude of the left-to-right shunt and right ventricular(RV) dilation from a single anomalous pulmonary vein using cardiac magnetic resonance imaging. Subjects with >1 anomalous pulmonary vein or associated lesions, including atrial septal defects, were excluded. In the 6 subjects identified, the median pulmonary-to-systemic flow ratio was 1.55(range 1.3 to 1.6). The mean RV end-diastolic volume indexed to body surface area in the subjects was significantly larger than in a normal reference cohort(108±16 vs 78±18 cm3/m2, p=0.0009) and greater than the upper limit of normal in all 6 subjects. Older age did not correlate with increased magnitude of shunting(r=0.3, p=0.5), but increased age did correlate with RV end-diastolic volume indexed to body surface area(r=0.96, p=0.01). Isolated partially anomalous pulmonary venous connection with only 1 vein connecting anomalously results in a modest left-to-right shunt and mild RV dilation.
文摘PURPOSE: Solitary ulcer syndrome is a rare condition characterized by inflammation and chronic ulcer of the rectal wall in patients suffering from outlet constipation. Despite similar surgical options (rectopexy, anterior resection), solitary ulcer syndrome may differ from overt rectal prolapse with regard to symptoms and pathogenesis. The present work analyzed differences between these conditions in a case-control physiology study. METHODS: From 1997 to 2002, 931 consecutive subjects were investigated in a single physiology unit for anorectal functional disorders. Standardized questionnaires, anorectal physiology, and evacuation proctography were included in a prospective database. Diagnosis of solitary ulcer syndrome was based on both symptoms and anatomic features in 25 subjects with no overt rectal prolapse (21 females and 4 males; mean age, 37.2 ±15.7 years) and no past history of anorectal surgery. They were compared with age-matched and gender-matched subjects: 25 with outlet constipation (also matched on degree of internal procidentia), 25 with overt rectal prolapse without any mucosal change, and 14 with overt rectal prolapse and mucosal changes. RESULTS: Subjects with solitary ulcer syndrome reported symptomatic levels (digitations, pain, incontinence) similar to those of patients with outlet constipation, but they had significantly more constipation and less incontinence than patients with overt rectal prolapse. Compared with each of the three control groups (dyschezia, rectal prolapse without mucosal change, and rectal prolapse with mucosal change), subjects with solitary ulcer syndrome more frequently had an increasing anal pressure at strain (15 vs. 5, 3, and 1, respectively ;P < 0.01) and a paradoxical puborectalis contraction (15 vs. 9, 1, and 1, respectively; P < 0.05). With respect to evacuating proctography, complete rectal emptying was achieved less frequently in this group (5 vs. 12, 23, and 10, respectively; P < 0.05). Compared with patients with overt rectal prolapse, mean resting and squeezing anal pressures were significantly higher in both groups of subjects with solitary ulcer syndrome and with outlet constipation. Prevalence and levels of anatomic disorders (perineal descent, rectocele) did not differ among the four groups except for rectal prolapse grade and prevalence of enterocele (higher in overt rectal prolapse group). Interestingly, and despite matched controls for degree of intussusception, individuals with solitary ulcer syndrome had circular internal procidentia more often compared with those suffering from outlet constipation without mucosal lesions (15 vs. 8, P < 0.05). CONCLUSION: This case-controlled study quantifies functional anal disorders in patients suffering from solitary ulcer syndrome. Despite no proven etiologic factor, sphincter-obstructed defecation and circular internal procidentia both may play an important part in the pathogenesis and an exclusive surgical approach may not be appropriate in this context.
文摘Objective: Polyneuropathy, organomegaly, endocrinopathy,M protein, and skin ch anges (POEMS) syndrome is a rare cause of polyneuropathy. Although the polyneuro pathy component is essential for the diagnosis of the disease, the pattern of as sociated electrodiagnostic abnormalities has not been characterized in detail. T he purpose of this study was to elucidate the features of nerve conduction abnor malities in POEMS syndrome.Methods: We reviewed the medical records and nerve co nduction studies(NCS) of 12 consecutive patients with POEMS.Results: A total of 68 motor and 46 sensory nerves were examined.Compound muscle action potentials(C MAPs) and sensory nerve action potentials(SNAPs) were not elicited in lower limb s more frequently compared with in upper limbs (P < 0.05, in both motor and sens ory nerves), and the CMAP amplitude was more attenuated in lower limbs than in u pper limbs (P < 0.05). Abnormal conduction slowing was frequently observed in mo tor (95%) and sensory (76%) nerves. Distal motor latencies were abnormally pro longed in 75%of the motor nerves,and terminal latency indices were significant - ly higher in patients than in normal controls (P < 0.05). Conclusions: NCS in POEMS syndrome showed characteristic patterns, in which conduction abnormalities were more frequently and severely affected in the lower limbs, and more predomi nantly in the intermediate nerve segments than in the distal portions. Significa nce:The recognition of these characteristic patterns may be helpful for the earl y diagnosis of polyneuropathy in POEMS syndrome.
文摘盐度对鱼类生态生理学作用的直接效应是引起鱼体对渗透压的调节,间接影响则表现为对鱼体与环境间物质交换与能量流动的影响(De Silva et al.,1976)。自上世纪50年代末开始,盐度一直是鱼类生理学和实验生物学的重要指标。为此,众多专家就广盐性洄游种类鲑鱼生长与盐度的关系(Otto,1971;Shaw et al.,1975;MacLeod,1977;
