脂质沉积性肌病的临床、神经电生理和骨骼肌病理分析

目的 探讨脂质沉积性肌病(LSM)的临床表现、神经电生理及肌肉病理特点.方法 回顾分析16例LSM的临床表现、肌电图和神经传导、肌肉活检病理改变.结果 LSM主要临床特点为亚急性或慢性起病,以近端肌无力为主,症状呈波动性,肌无力重而肌萎缩轻.血清肌酶有不同程度的升高,肌电图多为肌源性损害,激素、核黄素治疗有效.临床上容易误诊为多发性肌炎、肌营养不良症、心肌炎、胃肠道疾病等.肌肉病理学特点为肌纤维内可见大量均匀的小筛孔样空泡,部分空泡融合成大泡或形成裂隙状.ORO染色证实筛孔样空泡被大量红染的脂肪颗粒充填,受累肌纤维以Ⅰ型纤维为主.4例患者行电镜检查可见肌原纤维间有大量脂滴沉积,其中1例伴有异常线粒体增多.结论 LSM是一种以易疲劳和肌无力为主要临床表现的脂质代谢障碍性肌病,肌无力较重而肌萎缩轻.神经电生理改变相对较轻,部分患者肌电图为肌源性损害.激素、核黄素治疗可获得良好疗效,肌肉活检病理学检查是诊断LSM的重要手段.     

原发性肉碱缺乏致脂质沉积性肌病的临床与病理特点

目的 分析原发性肉碱缺乏致脂质沉积性肌病（LSM）的临床与病理特点。方法 回顾性分析4例可能LSM患者的临床资料。结果 本组患者为亚急性或慢性起病，主要表现为近端肌无力，疲劳不能耐受；血清肌酶有不同程度的升高；肌电图示肌源性损害；病理检查示肌纤维内可见大量细小空泡和裂隙形成；MGT染色无破碎红纤维，油红O染色显示空泡为大量脂滴充填；受累纤维以Ⅰ型纤维为主。电镜证实肌纤维内脂滴堆积，可伴有线粒体的轻度增多。改善能量和糖皮质激素治疗有效。结论原发性肉碱缺乏致LSM是一种以易疲劳和肌无力为主要临床表现的脂质代谢障碍性肌病，病理改变以肌纤维内脂滴堆积为主，一般不伴有线粒体结构的明显异常。糖皮质激素治疗可获得良好疗效。     

脂质沉积性肌病的临床和病理特点

目的总结脂质沉积性肌病（lipid storage myopathy,LSM）的临床和病理特点,为早期诊断和治疗提供参考。方法对5例脂质沉积性肌病患者的临床资料进行回顾性分析。结果 5例脂质沉积性肌病患者均为慢性或亚急性起病,主要表现为不同程度的肌无力和对运动不耐受、血清肌酶均升高、神经电生理检查显示肌源性损害,病理检查发现肌纤维内空泡样变,脂滴明显增多,脂滴空泡呈＂串珠＂样排列。给予能量支持、低脂饮食、糖皮质激素等治疗后患者的临床症状好转。结论脂质沉积性肌病的确诊依靠肌肉活检,该病预后良好,及时的诊断和综合治疗可明显改善患者的生活质量。     

脂质沉积性肌病诊断及误诊分析

目的分析脂质沉积性肌病（LSM）的临床及病理特点,探讨脂质沉积性肌病被误诊的原因。方法分析8例确诊为脂质沉积性肌病患者的临床特点、实验室检查、神经电生理及病理学资料。结果LSM的临床特点是四肢近端肌无力和运动不耐受;肌酶谱轻中度甚至重度升高;肌电图表现肌源性损害、神经源性损害或混合性损害;肌肉病理学检查显示肌纤维中大小不等的空泡形成;7例0R0染色显示Ⅰ型肌纤维中大量脂质颗粒沉积,1例显示脂肪成分正常者电镜检查可见肌纤维内大量脂滴沉积,呈串珠状排列。2例进行神经活检,1例正常,1例光镜下部分髓鞘变薄,崩解脱失。电镜下髓鞘板层分离,雪旺氏细胞内出现脂滴沉积。确诊的LSM曾被误诊为多发性肌炎、肢带型肌营养不良、脊肌萎缩症、胶原血管病、慢性吉兰-巴雷综合征、病毒性心肌炎。结论LSM很易被误诊为其他肌病或神经源性肌病,肌肉活检及组化OR0染色可能染色不成功造成阴性结果,电镜检查是诊断LSM的主要依据。     

脂质沉积性肌病的临床与病理分析

目的探讨脂质沉积性肌病(lipidstoragemyopathy,LSM)的临床表现及病理学特点。方法回顾分析7例LSM的临床表现、肌电图及肌肉活检病理改变。结果LSM的主要临床特点为亚急性或慢性起病,近端肌无力,症状呈波动性,血清肌酶有不同程度的升高,多数病人肌电图示肌源性损害,激素治疗有效。病理学特点为肌纤维内可见大量均匀的小筛孔样空泡,部分空泡融合成大泡或形成裂隙状。ORO染色证实筛孔样空泡为大量红染的脂肪颗粒充填,部分肌纤维破裂,伴脂肪颗粒堆积。受累纤维以Ⅰ型纤维为主。电镜证实肌纤维内脂滴堆积,其中1例伴有异常线粒体增多。结论LSM是一种以易疲劳和肌无力为主要临床表现的脂质代谢障碍性肌病,激素治疗可获得良好疗效。肌肉活检病理学检查是诊断LSM的重要手段。     

脂质沉积性肌病临床特征(附10例报告)

目的总结脂质沉积性肌病(lipid storage myopathy,LSM)的临床特点,以提高对此疾病的认识。方法回顾性分析10例LSM患者资料并复习文献。结果本组患者平均起病年龄(21.7±10.2)岁,呈亚急性或慢性起病,病情缓慢进展加重或出现缓解复发,主要表现为运动耐受差和不同程度的肌无力、肌痛或触痛、腱反射减弱或消失、肌萎缩,肌酶轻-中度升高,神经电生理显示肌源性损害或者混合性损害。肌肉病理学显示肌纤维间、肌膜下、胞核周围大量排列成串或成团的脂滴,无炎性细胞浸润。糖皮质激素治疗可以改善症状。结论LSM是以肌无力和肌疲劳为主要表现的肌纤维内脂肪代谢障碍病。肌肉病理检查有助于确定诊断。     

脂质沉积性肌病的临床与病理分析

目的 探讨脂质沉积性肌病(lipid storage myopathy，LSM)的临床表现及病理学特点。方法 回顾分析7例LSM的临床表现、肌电图及肌肉活检病理改变。结果 LSM的主要临床特点为亚急性或慢性起病，近端肌无力，症状呈波动性，血清肌酶有不同程度的升高，多数病人肌电图示肌源性损害，激素治疗有效。病理学特点为肌纤维内可见大量均匀的小筛孔样空泡，部分空泡融合成大泡或形成裂隙状。ORO染色证实筛孔样空泡为大量红染的脂肪颗粒充填，部分肌纤维破裂，伴脂肪颗粒堆积。受累纤雏以Ⅰ型纤雏为主。电镜证实肌纤维内脂滴堆积，其中1例伴有异常线粒体增多。结论 LSM是一种以易疲劳和肌无力为主要临床表现的脂质代谢障碍性肌病，激素治疗可获得良好疗效。肌肉活检病理学检查是诊断LSM的重要手段。     

脂质沉积性肌病合并横纹肌溶解的临床与病理特征(附1例报告)

目的 研究脂质沉积性肌病(lipid storage myopathies,LSM)合并横纹肌溶解( rhabdomyolysis,RM)的临床与病理学特征.方法 回顾性分析1例LSM合并RM患者的临床和病理学资料.结果 本例患者青年起病,主要表现为运动不耐受,上消化道出血后病情加重,出现四肢近端肌无力,咀嚼、抬头困难,肌痛明显,一过性棕色尿.肌酸激酶(creatine kinase,CK)显著升高.肌电图示:肌源性损害.病理检查示:HE染色见大量筛状空泡纤维和坏死肌纤维,油红"O"(ORO)染色见脂滴显著增加.经糖皮质激素和左旋肉碱等治疗后,患者症状迅速缓解.结论 LSM患者短时间内出现肌无力明显加重伴棕色尿及CK显著升高,应考虑合并RM,肌活检示肌纤维内脂滴显著增加并出现大量坏死肌纤维是其肌肉病理特征.     

脂质沉积性肌病18例临床分析

目的分析临床上少见的脂质沉积性肌病患者的临床表现和病理特征。方法收集我院经光镜和/或电镜检查后确诊的脂质沉积性肌病患者18例,分析其临床表现、肌电图、心肌酶学以及肌肉组织病理活检特点。结果脂质沉积性肌病的主要临床特点为四肢近端肌无力和对运动不耐受;血清肌酶普遍升高;肌电图可呈典型肌源性损害改变;肌肉活检均可见脂质沉积特征性改变。病理示肌纤维内可见大量细小空泡或裂隙形成,油红O染色显示肌纤维内脂滴含量明显增多。结论 LSM临床表现为非特异性肌无力,病理改变以肌纤维内脂滴沉积为主,小剂量泼尼松龙治疗有效。     

误诊为多发性肌炎的脂质沉积性肌病——附3例报告

目的探讨脂质沉积性肌病(lipid storage myopathy,LSM)的临床特征、病理改变、代谢异常及治疗。方法回顾性分析我院确诊的3例LSM患者的临床表现、肌肉病理所见及血、尿代谢检查结果 ,结合文献报道总结LSM的临床特点和发病机制,并对多发性肌炎的诊断标准进行讨论。结果 3例患者均有对称性肌无力和/或肌痛,肌酶谱增高,最初均诊断为多发性肌炎,经激素治疗可好转,但病情反复。肌肉病理显示肌纤维大小不等,大量肌纤维内可见类圆形空泡,油红O染色见肌纤维内大量红染的脂滴沉积。经调整饮食、口服维生素B2、左卡尼汀等药物治疗后,症状改善明显。结论应用Bohan/Peter标准可能导致对多发性肌炎的过度诊断。对临床表现为肌无力、肌痛的患者,不要盲目应用激素治疗,应积极进行骨骼肌活检,明确诊断。     

Mechanisms of the suppression of the nociceptive reflex of the opening of the mouth during stimulation of the structures of the limbic brain

The comparative effectiveness of the inhibitory influence of tetanic stimulation of hypothalamus, amygdala and limbic cortex on EMG-response of m. digastricus evoked by electrical stimulation of tooth pulp nociceptive afferents was studied in cats anesthetized with a mixture of chloralose and nembutal. It was found that inhibition of the EMG-component of the jaw-opening reflex is most pronounced in case of stimulation of medial and lateral region of the hypothalamus, the inhibitory effect of central and medial nuclei of the amygdala is less pronounced and the effect of the limbic cortex is the weakest. It was shown that the mechanism of the antinociceptive effect of tetanic stimulation of the hypothalamus is not related to the concomitant increase of the blood pressure. After stabilization of the blood pressure the suppressive effect of the hypothalamus remains without changes, that points out to a direct, primary, not baro-afferent mechanism of the inhibition of the activity of nociceptive neurons of the trigeminal sensory nuclei. Noradrenaline, injected intravenously, induced a large increase of the blood pressure accompanied by a pronounced inhibition of the pain reflex. Angiotensin causes the same degree of blood pressure elevation without changes in the amplitude of the EMG-response of the pain reflex. Hypothalamic and noradrenergic mechanisms for control of pain sensitivity are discussed.     

药物治疗与合并认知行为治疗对强迫症疗效的比较

目的探讨认知行为心理治疗(CBT)在强迫症(OCD)患者各亚型治疗中的有效性和规律性。方法本研究为临床对照研究。符合入组标准的强迫症患者按患者自愿原则分为两组,治疗观察3、6、12个月。疗效评定分别运用Yale-Brown强迫量表,自拟的自评好转程度量表和临床疗效评定。结果认知行为心理治疗合并药物治疗组31例,临床有效率70.9%,其中治愈率1.8%。单纯药物治疗组24例,临床有效率33.3%。Yale-Brown强迫量表和自评量表得分在6个月和12个月两组有显著差异(P<0.05)。其中强迫症亚型(怕脏型、反复检查型和反复担心型)的疗效比较,怕脏型在治疗3个月末两组间自评量表评分有显著性差异(P<0.05);反复担心型在治疗6个月末两组间Yale-Brown强迫量表总分有显著性差异(P<0.05);反复检查型两组间无统计学差异。结论认知行为心理治疗合并药物治疗强迫症的疗效明显优于单纯药物治疗。强迫症的亚型在治疗中的有效性次序为:反复担心型>怕脏型>反复检查型。     

Characteristics of the sympathetic innervation of the nictitating membrane and of the vasculature of the nose and tongue of the cat

Summary Vasomotor responses from the nasal mucosa and tongue, and contractions of the nictitating membrane, were recorded on stimulation of the cervical sympathetic or internal carotid nerves.Preganglionic sympathetic nerve fibres which elicited a membrane response possessed a lower threshold than those which evoked nasal vasoconstriction, while the latter displayed a lower threshold than fibres which evoked tongue vasoconstriction. The sympathetic vasodilator fibres to the tongue, whose activity was revealed after-receptor blockade, had a similar threshold to the vasoconstrictor fibres.Membrane contraction, nasal vasoconstriction and occasionally tongue vasoconstriction could be evoked by stimulating the internal carotid nerve. The postganglionic fibres innervating the nasal mucosa had a similar threshold to those of the nictitating membrane, which may indicate that there are small myelinated fibres innervating the mucosa.The preganglionic compound nerve action potential had four major components, S₁–S₄. S₁, S₂ and usually S₃ fibres were associated with membrane contraction; S₂, S₃ and sometimes S₁ fibres were associated with nasal vasoconstriction; and S₃, usually S₂ and occasionally S₁ fibres were associated with vasoconstriction in the tongue. It is concluded that each of these three groups of nerve fibres, but not S₄ fibres, may include fibres associated functionally with the three effectors.There was a considerable difference between the relative amplitude of the responses of the three effectors elicited by stimulation of the cervical sympathetic nerve at frequencies between 0.2 and 2 Hz. Vasoconstrictor responses were relatively larger than membrane contractions suggesting differences in the mechanisms of neurotransmission at the neuroeffector junctions.     

Development of the projection from the nucleus of the brachium of the inferior colliculus to the superior colliculus in the ferret

Neurons in the deeper layers of the superior colliculus (SC) have spatially tuned receptive fields that are arranged to form a map of auditory space. The spatial tuning of these neurons emerges gradually in an experience-dependent manner after the onset of hearing, but the relative contributions of peripheral and central factors in this process of maturation are unknown. We have studied the postnatal development of the projection to the ferret SC from the nucleus of the brachium of the inferior colliculus (nBIC), its main source of auditory input, to determine whether the emergence of auditory map topography can be attributed to anatomical rewiring of this projection. The pattern of retrograde labeling produced by injections of fluorescent microspheres in the SC on postnatal day (P) 0 and just after the age of hearing onset (P29), showed that the nBIC-SC projection is topographically organized in the rostrocaudal axis, along which sound azimuth is represented, from birth. Injections of biotinylated dextran amine-fluorescein into the nBIC at different ages (P30, 60, and 90) labeled axons with numerous terminals and en passant boutons throughout the deeper layers of the SC. This labeling covered the entire mediolateral extent of the SC, but, in keeping with the pattern of retrograde labeling following microsphere injections in the SC, was more restricted rostrocaudally. No systematic changes were observed with age. The stability of the nBIC-SC projection over this period suggests that developmental changes in auditory spatial tuning involve other processes, rather than a gross refinement of the projection from the nBIC.     

A contribution to the study of the architecture of the autonomic nervous system of the digestive tract of the rat

Summary The distribution of aminergic and non-aminergic nerve fibres to the different constituents of the wall of the digestive tract in various regions is described. Aminergic fibres synapse with all nervous perikarya. Densely interlacing networks of nerve fibres are found in both layers of the tunica muscularis and in the lamina muscularis mucosae. A finely meshed plexus is observed in relation to the wall of the blood vessels in the wall of the gut. There are many fibres connecting the muscular and the vascular plexus. No nerve fibres have been observed in direct relation to the epithelium.The functional implications of these findings are discussed.