COLD PRESSOR TEST DEMONSTRATES RESIDUAL SYMPATHETIC CARDIOVASCULAR ACTIVATION IN FAMILIAL DYSAUTONOMIA

Objectives: Patients with polyneuropathy and antibodies to myelin-associated glycoprotein (MAG) and sulphated glucuronyl paragloboside (SGPG) differ from chronic inflammatory demyelinating polyneuropathy (CIDP) because of a slower, progressive course, symmetrical and predominantly sensory involvement of legs, predominantly distal slowing of motor conductions, and poorer response to therapy. We studied whether a wide set of electrophysiologic parameters may differentiate these two neuropathies. Methods: We reviewed the electrophysiological studies of 10 patients with anti-MAG/SGPG antibodies and 22 with CIDP examining: (1) motor conduction velocity and distal compound muscle action potential amplitude; (2) conduction block (CB) and temporal dispersion; (3) distal motor latency and terminal latency index (TLI); (4) F wave and proximal conduction time; and (5) sensory conduction and occurrence of abnormal median with normal sural sensory potential. Results: Anti-MAG/SGPG neuropathies showed: (1) more severe involvement of peroneal nerves; (2) more frequent disproportionate distal slowing of motor conductions (TLI less than or equal to 0.25) and absent sural potential; and (3) no CB. However 3/22 CIDP patients also had at least two nerves with TLI 0.25 and no CB. Conclusions: Electrophysiologic findings suggest in anti-MAG/SGPG neuropathy a length-dependent process with a likely centripetal evolution. A disproportionate slowing of conduction in distal segments of motor nerves suggests the diagnosis of anti-MAG/SGPG neuropathy, although it is not pathognomonic.     

Gastrointestinal teratomas: CT and US appearance with pathologic correlation

Gastrointestinal teratomas are uncommon, benign neoplasms that occur primarily in children. A retrospective review of five cases (two gastric, one pancreatic, one mesenteric, and one in the lesser omentum) is presented with emphasis on the computed tomographic and ultrasonographic appearances. Principal findings are a well-defined mass with separate cystic and solid components of varying proportions, discrete areas with densities similar to that of fat, or coarse, globular calcifications within the solid component. Recognition of these findings may allow the radiologist to make a correct preoperative diagnosis of teratoma.     

Cerebrospinal fluid rhinorrhea: depiction with MR cisternography in dogs

Canine cerebrospinal fluid rhinorrhea, which occurs frequently in purebred beagles, was demonstrated in two dogs on magnetic resonance images after cisternal introduction of gadolinium-DTPA dimeglumine.     

In vivo and in vitro binding of C4 molecules on red cells: a correlation of numbers of molecules and agglutination

The fourth component of human complement (C4) is one that is essential to the antibody-mediated classical activation pathway. C4d, present on all normal and most patient red cells (RBCs), may be detected by the human antisera anti-Rodgers (Rg) and -Chido (Ch). A study has been made of the Rg/Ch antigens on normal and patient RBCs in an attempt to understand the mechanism by which C4 is bound to normal RBCs in the absence of RBC antibodies (Abs). Because RBCs from C1q-deficient patients express Rg/Ch, it seems that C1q is not essential for C4 binding. Treatment of normal RBCs with proteolytic enzymes, including trypsin, eliminated positive reactions with anti-Rg/Ch even though the C4d fragment is considered to be resistant to cleavage by trypsin. By correlating agglutination reactions with numbers of bound C4d and C3d molecules, it is evident that both C4d and C3d were affected by trypsin treatment and that anti-Rg/Ch were not capable of agglutinating RBCs with less than 50 molecules of bound C4d. It is concluded that trypsin-sensitive and -insensitive RBC membrane structures may both act as acceptors for C4. RBCs with null phenotypes of the major blood group systems all expressed Rg/Ch antigens, so none of the structures that carry these antigens act preferentially as acceptors for C4.     

应激干预对抑郁症患者皮电和心率的影响

目的：了解放松训练和静坐两种干预方式对抗心算对抑郁症患者皮电和心率的影响，以及正常人、单纯抑郁症患者和伴焦虑症状的抑郁症患者在皮电和心率上的差异。方法：选择2006—06／09在河北医科大学第一附属医院精神卫生科门诊及住院的单纯抑郁症和伴焦虑症状的抑郁症患者各24例作为单纯抑郁症组和伴焦虑抑郁症组，均符合中国精神障碍分类与诊断标准，每组男女各12例，年龄17—50岁。选择同期院内医护人员及患者家属24人作为对照组，男女各12人，年龄19-47岁。所有对象对实验均知情同意。以心算为应激源，放松训练和静坐为干预手段，记录被试在基线期、干预期、应激期及恢复期的皮电和心率。结果：抑郁症患者48例及健康对照者24人全部进人结果分析。①在基线期，伴焦虑抑郁组心率明显高于单纯抑郁症组和对照组，皮电明显低于另外两组（P〈0．01），单纯抑郁症组和对照组皮电和心率比较，差异均不明显（P〉0．05）。②在干预期，被试的皮电升高，心率下降，放松训练的效果明显好于静坐，尤其对伴焦虑症状的抑郁症患者效果最好，静坐会加重伴焦虑症状的抑郁症患者的紧张状态。③在应激期，放松训练有效的对抗心算引起的皮电下降以及心率的升高，效果明显好于静坐组。④恢复期放松训练和静坐时的皮电、心率指标基本都恢复到静息状态。结论：①静息状态下伴焦虑症状的抑郁症患者的交感神经功能亢进，兴奋性高于单纯抑郁症患者和正常人。②放松训练可以缓解交感神经的紧张程度，并能够较静坐更好的对抗应激引起的交感神经活动增强。③静坐对单纯抑郁症患者和正常人有放松作用，但可引起伴焦虑症状的抑郁症患者更多的紧张情绪。     

Creutzfeldt--Jakob Disease in Recipients of Human Growth Hormone in the United Kingdom: A Clinical and Radiographic Study

In the past 3 years there have been five further cases, in additionto one case reported in 1985, of Creutzfeldt-Jakob disease inrecipients of human growth hormone in the United Kingdom. Theclinical findings of two of these cases are described, demonstratinga typical presentation with a predominantly cerebellar syndromeat onset which is not commonly a presenting feature of sporadicCreutzfeldt-Jakob disease. In one case a ^99mTc hexamethylpropylenaminesingle photon emission tomographic scan showed marked impairmentof tracer uptake in the basal ganglia and cerebral cortex ata time when the clinical picture was predominantly cerebellar.This technique may be useful in early diagnosis. In the othercase post mortem examination of the brain showed prominent amyloiddeposition in the cerebellum, which has not been described previouslyin pituitary-hormone related Creutzfeldt-Jakob disease. Thepreviously published cases of growth hormone-related Creutzfeldt-Jakobdisease are reviewed and reasons for the particular clinicalpattern seen are discussed.