Recurrent Ophthalmoplegia Presenting Different Clinical Features in a Patient with Anti-GQ1b Antibody Syndrome

No abstract available.

Síndrome anti-GQ1b: Descripción de cuatro pacientes y revisión de la literatura / Anti-GQ1b syndrome: Report of four cases

Anti-GQ1b syndrome includes Miller Fisher Syndrome (MFS), Guillain Barré Syndrome (GBS), Bickerstaff`s brain stem encephalitis (BBE) and Acute Ophtamoplegia (AO). We report four patients aged 16 to 76 years, with anti-GQ1b syndrome. All presented with MFS, one of them evolved to GBS pharyngeal-cervical-brachial variant and other to GBS with BBE. All had a previous history of diarrhea or upper respiratory tract infection. All had positive anti-GQ1b serum antibodies. Both brain magnetic resonance imaging and cerebrospinal fluid analysis were normal. Electrophysiology studies were compatible with a demyelinating disease. Two patients needed airway protection with an orotracheal tube and developed dysautonomia. All four patients were treated with immunomodulation. On the sixth month follow-up, patients had only minimal alterations in the neurological examination.

Comparison of immunoblotting and ELISA for detection of anti-ganglioside antibodies in children with Guillain-Barre Syndrome

Anti-ganglioside antibody assays are widely used for diagnosis of auto-immune peripheral neuropathies. This study aimed to determine serum levels of anti-ganglioside antibodies in children with Guillain-Barre syndrome by immunoblotting technique and compare the results with those obtained by ELISA method. In this investigation, 50 children with Guillain-Barre syndrome [GBS] who were admitted from July 2006 to July 2008, to Tabriz Children's hospital in the north-west of Iran were studied. 30 children admitted for various other reasons than GBS were randomly selected as a control group. The levels of anti-ganglioside antibodies in serum were measured by ELISA and immunoblotting methods using commercial kits. Anti-ganglioside antibodies [IgG] were detected in 16 [32%] GBS patients and in 1 [3.3%] control using ELISA assay. However, by employing immunoblotting technique, antibodies against seven gangliosides were found positive in 28 [56%] GBS patients and none in the control group. The sensitivities of immunoblotting and ELISA methods were 56% and 32% and their specificities were 100% and 97%, respectively [p<0.001]. According to the clinical criteria of GBS, the specificity and sensitivity of immunoblotting was better than those of ELISA. It is important to notice that the immunoblotting method is able to measure the seven types of antibodies [GM1, GM2, GM3, GD1a, GD1b, GT1b, and Gq1b] simultaneously and it is an easy, routine method with a lower cost

A case of Miller Fisher syndrome with anti GQ1b in Thailand.

Miller-Fisher syndrome (MFS) is a rare variant of Guillain-Barré syndrome (GBS) and is characterized by the clinical triad of ataxia, ophthalmoplegia, and areflexia. The incidence rate in Thailand has not been established but it occurred approximately 1-5% that of GBS. Here, the authors report a Thai patient diagnosed as MFS that had a positive test of antibodies against the ganglioside GQ1b. These antibodies have diagnostic and pathogenic importance to MFS because of high sensitivity and specificity. All other investigations, such as cerebrospinal fluid analysis, electrophysiological studies, and imaging studies had no significant abnormalities. The patient was successfully treated with intravenous immunoglobulin and fully recovered within one month. After eighteen months follow-up, he is still healthy and has had no recurrent symptoms.

Secretion of metastasis related gangliosides by mouse B16-melanoma in circulation in vivo and in culture media in vitro.

Mouse B16LuF1 melanoma cells of lower metastatic potential to lung were treated in vitro with same concentration (50 microM) of gangliosides prepared from plasma of mice bearing lung metastasis of B16LuF5, B16LuF9 or B16LuF10 melanoma cell lines of increasing metastatic potential to lung (LuF1 < LuF5 < LuF9 < LuF10) and injected to normal mice through tail vein. The number of metastatic tumor nodules formed in lung increased gradually in mice receiving B16LuF5, B16LuF9 and B16LuF10-ganglioside-treated B16LuF1 cells compared to mice receiving B16LuF1 cells without any ganglioside treatment. Similarly, mouse B16LuF1 melanoma cells treated in vitro with 50 microM concentration of gangliosides prepared from spent culture media of B16LuF5, B16LuF9 or B16LuF10 cells cultured in vitro were injected to normal mice through tail vein. The number of metastatic tumor nodules formed in lung increased gradually in mice receiving B16LuF5, B16LuF9 and B16LuF10-ganglioside-treated B16LuF1 cells compared to mice receiving B16LuF1 cells without any ganglioside treatment. The results indicated that metastasis-associated gangliosides present in plasma and culture media of B16-melanoma of increasing metastatic potential to lung enhanced metastatic potential of B16LuF1 cells. The increasing concentration of metastasis-associated gangliosides present in plasma and in culture media of B16-melanoma of increasing metastatic potential possibly determined increase in metastatic potential of B16LuF1-melanoma cells.

Circulating angiogenic factors in patients with chronic liver diseases

Platelet-derived endothelial cell growth Factor [PD-ECGF], basic fibroblast growth factor [b-FGF], gangliosides [Gs] and nitric oxide [NO] are angiogenic factors expressed in various cancer tissues as well as non malignant tissues. Little is known about the role of these factors in patients with chronic liver diseases such as chronic hepatitis [CH], cirrhosis, and hepatocellular carcinoma [HCC] with cirrhosis. The levels of these factors were determined in 28 patients with chronic hepatitis, 43 with cirrhosis and 29 patients with HCC. The study also included 18 normal individuals who are comparable to patients in age as a control group. The study revealed that the levels of these angiogenic factors are significantly increased in patients with HCC in comparison with either patients with hepatitis or liver cirrhosis. Moreover, both Gs and NO are also increased in patients with cirrhosis in comparison with controls. In HCC the levels of angiogenic factors reflected tumor burden where they were significantly increased in higher burden. These angiogenic factors are derived from HCC cells as well as inflammatory cells. In cirrhosis the elevation of these factors might be related to the extent of inflammation and angiogenesis in the cirrhotic liver. These factors showed significant positive correlations with liver enzymes in HCC. Assessment of the angiogenic factors in patients with chronic liver diseases would help to follow progression of liver affection. The expression of PD-ECGF in HCC would predict its chemosensitivity

Glycosphingolipids of porcine blood: human blood group A and H antigens with type 1 chain in erythrocytes and plasma.

Glycosphingolipids were purified from porcine erythrocytes and plasma. Two minor glycolipids with human blood group A and H antigenicities were found in both sources as components. The two antigenic glycolipids were identified as a hexaglycosylceramide (IV3 alpha GalNAc,IV2 alpha Fuc-Lc4Cer) for the A antigen and pentaglycosylceramide (IV2 alpha Fuc-Lc4Cer) for the H antigen and belonged to lactoseries (type 1 sugar chain) in contrast to those with neolacto core (type 2 sugar chain) in human erythrocytes, thereby endorsing biochemically the previous serological observations that the A antigen on porcine erythrocytes is uptake from plasma, probably the H antigen being the case. In addition to major glycolipids of globoseries in red cells and plasma, a variety of acidic glycolipids including two classes of sulphatides (sulphated galactosylceramide and sulphated lactosylceramide) and five classes of gangliosides (GM3, GD3, GM1, fucosyl GM1 and GD1a) containing N-acetylneuraminic acid and N-glycolylneuraminic acid were obtained from plasma.