近年来,由于现代医学的进步和医疗条件的改善,特别是重症监护病房(ICU)的发展,为ICU中危重病患者提供了更好的条件来维持生命,使患者有更长的时间住在ICU,也使更多的脓毒(sepsis)和多脏器功能障碍综合征(MODS)得以生存,同时危重病性多...近年来,由于现代医学的进步和医疗条件的改善,特别是重症监护病房(ICU)的发展,为ICU中危重病患者提供了更好的条件来维持生命,使患者有更长的时间住在ICU,也使更多的脓毒(sepsis)和多脏器功能障碍综合征(MODS)得以生存,同时危重病性多发性神经病与肌病(critical il ness polyneurop-athy and myopathy,CIPNM)患者也逐渐增多。危重病性多发性神经病与肌病是是继发于重症监护患者的一大类神经肌肉病变,多由于败血症、多脏器功能衰竭以及长时间机械通气等引起急性、轴索变性疾病,甚至可累及神经肌肉接头和肌肉,表现为肌肉萎缩和坏死。根据累及部位分为危重病性多发性周围神经病(Critical il ness polynenropathy,CIP)与危重病性肌病(critical illness myopathy,CIM)。由于ICU医师一般多注重心、肺、脑等病变的处理,很少关注患者的周围神经和肌肉情况,往往要等到患者长时间不能脱离呼吸机或意识恢复后,出现明显四肢无力和腱反射消失时才引起医生注意,因此神经肌肉并发症常被漏诊。现就院ICU2012年8月~2014年6月收治并诊断的7例危重病性多发性神经病与肌病进行分析。展开更多
To determine the mechanisms underlying the changes in collagen metabolism resp onsible for muscle fibrosis in patients with neuromuscular diseases, the synthes is and degradation of collagens was studied in muscles of...To determine the mechanisms underlying the changes in collagen metabolism resp onsible for muscle fibrosis in patients with neuromuscular diseases, the synthes is and degradation of collagens was studied in muscles of patients with polyneur opathy and noninflammatory myopathies. The mRNA levels for type I, III, and IV c ollagens and immunohistochemical staining intensities for collagen propeptides a nd telopeptides were increased in polyneuropathy, suggesting enhanced synthesis of collagens. In myopathy, the mRNA levels were at the control level. Matrix met alloproteinase (MMP) 2 mRNA level was increased in polyneuropathy, although the quantity of proMMP 2 was not changed. An increase in type IV collagen concentr ation and proMMP 9 expression was observed in polyneuropathy but not in myopath ies. Our results suggest that intramuscular accumulation of type IV collagen occ urs in polyneuropathy, possibly leading to thickening of the capillary and muscl e fiber basement membranes. This may complicate the transportation of nutrients and cellular excreta between blood and muscle cells.展开更多
We report a 51-year-old alcoholic man with a 10-year history of cervical lipomas and progressive symmetrical sensory neuropathy, initially diagnosed with Madelung’s disease, an idiopathic syndrome often attributed to...We report a 51-year-old alcoholic man with a 10-year history of cervical lipomas and progressive symmetrical sensory neuropathy, initially diagnosed with Madelung’s disease, an idiopathic syndrome often attributed to chronic alcoholism. The eventual development of proximal weakness led to pathological and genetic testing which identified a A8344G mutation in the mitochondrial tRNA lysine gene, associated with MERRF (myoclonic epilepsy with ragged-red fibers). This case demonstrates how the varied terminology for this syndrome has resulted in a lack of consistent recognition and assessment for mitochondrial cytopathy.展开更多
Although paresthesias of the distal lower limbs are characteristic features of polyneuropathy, they may also herald the presence of a focal neuropathy, polyra diculopathy, or myelopathy. Electromyography and nerve con...Although paresthesias of the distal lower limbs are characteristic features of polyneuropathy, they may also herald the presence of a focal neuropathy, polyra diculopathy, or myelopathy. Electromyography and nerve conduction studies (EMG/N CS) are widely used in the evaluation of such symptoms, but their utility has no t been subjected to vigorous scrutiny. We investigated the clinical impact of th e electrodiagnostic consultation in assessing suspected polyneuropathy. When com pared with the clinical impression, the result of the electrodiagnostic consulta tion was confirmatory in only 39%of all patients, and changed the diagnosis or uncovered an additional diagnosis in 43%. An alternative diagnosis was likely w hen either weakness was present (75%) or the Achilles stretch reflex was preser ved (48%). These data support the use of EMG/NCS in the diagnostic evaluation o f patients presenting with distal paresthesias, especially in those with preserv ed Achilles reflexes or motor deficits.展开更多
重症监护病房获得性肌无力(intensive care unit acquired weakness,ICUAW)是重症监护病房患者出现的进行性全身肢体无力,且除危重病本身外无其他原因可解释的一组临床综合征,主要表现为神经肌肉功能障碍。ICUAW的发病机制涉及中枢、周...重症监护病房获得性肌无力(intensive care unit acquired weakness,ICUAW)是重症监护病房患者出现的进行性全身肢体无力,且除危重病本身外无其他原因可解释的一组临床综合征,主要表现为神经肌肉功能障碍。ICUAW的发病机制涉及中枢、周围神经系统和肌纤维内复杂的功能、结构改变,但尚未完全明确。该文综述了目前关于ICUAW的潜在发病机制,以期对ICUAW的诊治提供新思路。展开更多
文摘近年来,由于现代医学的进步和医疗条件的改善,特别是重症监护病房(ICU)的发展,为ICU中危重病患者提供了更好的条件来维持生命,使患者有更长的时间住在ICU,也使更多的脓毒(sepsis)和多脏器功能障碍综合征(MODS)得以生存,同时危重病性多发性神经病与肌病(critical il ness polyneurop-athy and myopathy,CIPNM)患者也逐渐增多。危重病性多发性神经病与肌病是是继发于重症监护患者的一大类神经肌肉病变,多由于败血症、多脏器功能衰竭以及长时间机械通气等引起急性、轴索变性疾病,甚至可累及神经肌肉接头和肌肉,表现为肌肉萎缩和坏死。根据累及部位分为危重病性多发性周围神经病(Critical il ness polynenropathy,CIP)与危重病性肌病(critical illness myopathy,CIM)。由于ICU医师一般多注重心、肺、脑等病变的处理,很少关注患者的周围神经和肌肉情况,往往要等到患者长时间不能脱离呼吸机或意识恢复后,出现明显四肢无力和腱反射消失时才引起医生注意,因此神经肌肉并发症常被漏诊。现就院ICU2012年8月~2014年6月收治并诊断的7例危重病性多发性神经病与肌病进行分析。
文摘To determine the mechanisms underlying the changes in collagen metabolism resp onsible for muscle fibrosis in patients with neuromuscular diseases, the synthes is and degradation of collagens was studied in muscles of patients with polyneur opathy and noninflammatory myopathies. The mRNA levels for type I, III, and IV c ollagens and immunohistochemical staining intensities for collagen propeptides a nd telopeptides were increased in polyneuropathy, suggesting enhanced synthesis of collagens. In myopathy, the mRNA levels were at the control level. Matrix met alloproteinase (MMP) 2 mRNA level was increased in polyneuropathy, although the quantity of proMMP 2 was not changed. An increase in type IV collagen concentr ation and proMMP 9 expression was observed in polyneuropathy but not in myopath ies. Our results suggest that intramuscular accumulation of type IV collagen occ urs in polyneuropathy, possibly leading to thickening of the capillary and muscl e fiber basement membranes. This may complicate the transportation of nutrients and cellular excreta between blood and muscle cells.
文摘We report a 51-year-old alcoholic man with a 10-year history of cervical lipomas and progressive symmetrical sensory neuropathy, initially diagnosed with Madelung’s disease, an idiopathic syndrome often attributed to chronic alcoholism. The eventual development of proximal weakness led to pathological and genetic testing which identified a A8344G mutation in the mitochondrial tRNA lysine gene, associated with MERRF (myoclonic epilepsy with ragged-red fibers). This case demonstrates how the varied terminology for this syndrome has resulted in a lack of consistent recognition and assessment for mitochondrial cytopathy.
文摘Although paresthesias of the distal lower limbs are characteristic features of polyneuropathy, they may also herald the presence of a focal neuropathy, polyra diculopathy, or myelopathy. Electromyography and nerve conduction studies (EMG/N CS) are widely used in the evaluation of such symptoms, but their utility has no t been subjected to vigorous scrutiny. We investigated the clinical impact of th e electrodiagnostic consultation in assessing suspected polyneuropathy. When com pared with the clinical impression, the result of the electrodiagnostic consulta tion was confirmatory in only 39%of all patients, and changed the diagnosis or uncovered an additional diagnosis in 43%. An alternative diagnosis was likely w hen either weakness was present (75%) or the Achilles stretch reflex was preser ved (48%). These data support the use of EMG/NCS in the diagnostic evaluation o f patients presenting with distal paresthesias, especially in those with preserv ed Achilles reflexes or motor deficits.
文摘重症监护病房获得性肌无力(intensive care unit acquired weakness,ICUAW)是重症监护病房患者出现的进行性全身肢体无力,且除危重病本身外无其他原因可解释的一组临床综合征,主要表现为神经肌肉功能障碍。ICUAW的发病机制涉及中枢、周围神经系统和肌纤维内复杂的功能、结构改变,但尚未完全明确。该文综述了目前关于ICUAW的潜在发病机制,以期对ICUAW的诊治提供新思路。
