Spinocerebellar degeneration secondary to chronic intestinal malabsorption: A vitamin E deficiency syndrome

Two adults are described who developed a progressive neurological disorder more than 20 years after the onset of chronic fat malabsorption. The clinical features included dysarthria, cerebellar ataxia, and prominent proprioceptive loss with depressed or absent tendon reflexes. Serum vitamin E was undetectable in both cases. One patient improved clinically and electrophysiologically after oral therapy with vitamin E. The findings in these patients were similar to those in others recently reported with vitamin E deficiency associated with biliary atresia. Electrophysiological observations suggested that the human deficiency state parallels that found neuropathologically in vitamin E-deficient animals.     

Supernormal excitability of human sensory fibers after ischemia

Weak electrical stimuli were applied to sensory fibers of the median nerve at the wrist, and the ascending action potentials were recorded at the elbow. This was done with or without a preceding conditioning stimulus to test for supernormal excitability to the second stimulus. In the resting nerve, supernormal excitability was present from 3 to 20 msec after a conditioning shock. After a period of ischemia, excitability was both exaggerated and prolonged. this observation confirms the presence in sensory fibers of a phenomenon previously described in motor fibers.     

Transcatheter embolization in the management of pulmonary hemorrhage

A group of 306 patients with acute pulmonary hemorrhage were evaluated by means of bronchial arteriography and treated with transcatheter embolization. Specific causes for bleeding included lung abscess, chronic pneumonia, tuberculosis, lung cancer, and bronchiectasis. In 120 patients the hemoptysis was massive, with volumes exceeding 500 ml/day. The majority (n = 225) were treated during peak hemorrhage. Embolization was performed with one of three methods: particulate embolization with polyurethane or velour, obturation with the angiographic catheter combined with peripheral embolization by means of infused albumin macroaggregates, and regional infusion of sclerosing agents. Effective hemostasis was obtained initially in 278 patients (90.8%), including 87.5% of those treated during peak hemorrhage. In 26 of 28 cases without initial response, the pulmonary artery was the source of bleeding. Recurrent bleeding, within 1-4 days, requiring surgery was observed in 39 patients with initially successful hemostasis. Of 158 patients who were treated without surgery, subsequent episodes of hemoptysis occurred in 36. Combined methods of embolization may improve the efficacy of treatment of operable and inoperable patients with lung disease complicated by hemorrhage.     

Peripheral neuropathy associated with anti-GM2 ganglioside antibodies: clinical and immunopathological studies

GM2 ganglioside is a potential peripheral nerve antigen for neuropathy-associated autoantibodies. However little data are available on their pathogenic effects, if any. In this study we have screened both neuropathy-associated and control sera for anti-GM2 antibodies and subsequently used high titre sera for immunohistological and complement mediated cytotoxicity studies. We identified abnormally elevated anti-GM2 antisera in the normal population, as well as in patients with peripheral neuropathies and other neurological diseases. GM2 antibodies were either mono-reactive, cross-reactive with GM1a, or cross-reactive with GalNAc-GM1b and/or GalNAc-GD1a. All GM2 antisera from neuropathy subjects and normal controls bound to, and were capable of complement-mediated lysis of the NSC-34 cell line which expresses high levels of membrane-associated GM2. However, in immunohistological studies on human and rodent peripheral nervous system tissues, no specific binding was seen with GM2 antisera, either cross-reactive with GalNAc-GM1b and GalNAc-GDla, or with GM1a. These data indicate that although GM2 antisera can lyse neural membranes containing GM2, this antigen(s) is not detectable by standard immunohistological techniques in human or rodent peripheral nerve. This raises doubts about their pathophysiological significance in human autoimmune neuropathy.     

Glycosphingolipid depletion in PC12 cells using iminosugars protects neuronal membranes from anti-ganglioside antibody mediated injury

Autoimmune neuropathies are frequently associated with pathogenic anti-ganglioside antibodies targeting ganglioside-rich neuronal and glial membranes. The extent of injury is determined by the concentration of membrane ganglioside and thus reduction might be expected to attenuate disease. In this study, we suppressed ganglioside biosynthesis in PC12 cells with the glucosylceramide synthase inhibitor, N-butyldeoxynojirimycin and observed reduced plasma membrane antibody binding and a major neuroprotective effect in complement-mediated lysis assays. These data demonstrate that iminosugar inhibitors, currently used to treat type 1 Gaucher disease, are also of potential value for depleting antigen and thereby suppressing tissue injury in anti-ganglioside antibody-associated neuropathy.     

Ganglioside antibodies and neuropathies

PURPOSE OF REVIEW: Continuing progress in the field of antiganglioside antibodies is expanding our comprehension of the pathogenesis of the inflammatory neuropathies. Recent studies have helped unravel all aspects of this process, addressing a range of subjects from the bacterial and host factors involved in antibody induction through to their pathogenic effects. RECENT FINDINGS: This review focuses on developments in the field over the past 18 months. Advances in our understanding of antibody induction, host risk factors, and pathogenic end effects are described systematically, and particular attention is given to the novel concept of ganglioside complexes. SUMMARY: The wealth of scientific data is now driving the design of novel, targeted therapies. This preclinical research is outlined and suggestions made as to how this might be translated into clinical practice.     

结肠冗长症手术治疗及自拟爽府汤预防复发的疗效观察

目的探讨结肠冗长症手术治疗方法以及自拟爽府汤在其术后的应用效果。方法将我院结肠冗长症患者40例随机分为观察组和对照组,对照组给予常规外科手术治疗,在此基础上观察组术后联合自拟爽府汤联合西药治疗,随访评估两者远期治疗效果。结果术后6个月观察组总有效率为100%,对照组为100%,两组比较差异无统计学意义（P〉0.05）。术后随访2年,观察组总有效率仍为100%,复发率为0%;对照组总有效率为68.42%,复发率为31.58%;两组比较差异具有统计学意义（P〈0.05）。结论单纯外科治疗结肠冗长症远期复发率高,联合自拟爽府汤可有效降低复发率。