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.

An Adult Case of Fisher Syndrome Subsequent to Mycoplasma pneumoniae Infection

Reported herein is an adult case of Fisher syndrome (FS) that occurred as a complication during the course of community-acquired pneumonia caused by Mycoplasma pneumoniae. A 38-yr-old man who had been treated with antibiotics for serologically proven M. pneumoniae pneumonia presented with a sudden onset of diplopia, ataxic gait, and areflexia. A thorough evaluation including brain imaging, cerebrospinal fluid examination, a nerve conduction study, and detection of serum anti-ganglioside GQ1b antibody titers led to the diagnosis of FS. Antibiotic treatment of the underlying M. pneumoniae pneumonia was maintained without additional immunomodulatory agents. A complete and spontaneous resolution of neurologic abnormalities was observed within 1 month, accompanied by resolution of lung lesions.

Oftalmoplejia aguda sin ataxia de carácter recurrente / Recurrent acute ophthalmoplegia without ataxia

Pueden mostrar aumento de anticuerpos anti-GQ1b los síndromes de Miller Fisher, Guillain-Barré con Oftalmoplegia, Rombencefalitis de Bickerstaff y Oftalmoplejia Aguda sin Ataxia, llamadas síndromes anti-GQ1b. Presentamos hombre de 72 años que ingresa por diplopía, oftalmoplejia de instalación aguda y dolor retro-ocular. Tuvo un episodio semejante hace cinco años, recuperado. Al ingreso mostraba oftalmoplejia completa bilateral sin ptosis, miosis y leve enoftalmo del ojo derecho. Potencia muscular conservada, arreflexia osteotendinea, sin compromiso cerebeloso ni sensitivo. LCR y electromiografía normales. RM de cerebro mostraba captación e hiperintensidad (T2) de los pares tercero y sexto. RM de medula espinal no mostró cambio de las raíces espinales. Aumento de GQ1b de 46.2/ 25 en el suero. Mejoró sin tratamiento. Treinta días después, quedaba solo paresia de los sextos pares. El anti-GQ1b es un marcador que identifica las neuropatías con compromiso oculomotor. Las oftalmoplejias agudas sin ataxia tienen reflejos conservados, el 30 por ciento tiene arreflexia. Sólo existen reportes de Síndrome de Guillain-Barré y Miller-Fisher recurrentes con anti-GQ1b. Sería el primer caso descrito de Oftalmoplejia aguda sin ataxia anti-GQ1b, recurrente.

They may exhibit increased anti-GQ1b antibodies in Miller Fisher syndrome, Guillain-Barre syndrome with ophthalmoplegia, Bickerstaff Rhombencephalitis, and Acute Ophthalmoplegia without ataxia , the so called anti-GQ1b syndromes. We report a 72 years old man who was admitted because of diplopia, acute onset ophthalmoplegia and retro-ocular pain. He had a similar episode five years ago, fully recovered. At admission he showed complete bilateral ophthalmoplegia without ptosis, miosis and slight enophthalmos of the right eye. Preserved muscle strength, deep tendon areflexia, without sensory or cerebellar commitment. CSF and electromyography were normal. Brain MRI showed uptake and T2 hyperintensity of the third and sixth cranial nerves. Spinal cord MRI showed no change in the spinal roots. Serum anti-GQ1b increase of 46.2 / 25. He improved without treatment. Thirty days later, paresis was only the sixth pair. The anti-GQ1b is a marker that identifies neuropathies with oculomotor commitment. The acute ophthalmoplegia without ataxia have normal reflex, 30 percent had areflexia. There are only Guillain-Barré and Miller-Fisher syndromes recurrent case reports with anti-GQ1b. It would be the first case of recurrent anti-GQ1b-positive acute ophthalmoplegia without ataxia.

Inmunodetección de gangliósidos en tejido fetal humano con el empleo de anticuerpos monoclonales / Immunodetection of gangliosides in human fetal tissue by using monoclonal antiobodies

Se emplearon anticuerpos monoclonales E1, A3 y P3 de nueva generación para conocer la localización de las moléculas reconocidas por estos anticuerpos en el tejido fetal humano. Se utilizaron además fragmentos de tejidos fetales, con edades gestacionales comprendidas entre 12 y 24 semanas. El anticuerpo monoclonal A3 reconoció células gliales del cerebro, cerebelo y médula espinal. El anticuerpo monoclonal P3 identificó células gliales del cerebelo y células epiteliales en el intestino delgado y en el músculo liso del estómago. El anticuerpo monoclonal E1 reconoció células gliales del cerebro y células del intestino delgado

Síndrome de Guillain-Barré: etiología y patogénesis / Guillain-Barre syndrome: etiology and pathogenesis

Guillain-Barre syndrome (GBS) is a reactive, self-limited, monophasic disease triggered by a preceding bacterial or viral infection. GBS has also been linked to underlying systemic diseases, certain malignancies, surgery, pregnancy, trauma severe infection, and tissue transplantation (bone marrow and organs). Although its pathogenesis is unclear, it is likely to be a consequence of an immune mediated process. Therefore, we believe that GBS results from an aberrant immune response that somehow mistakenly attacks the nerve tissue of its host, most probably by recognizing a molecular similar epitope mechanism (molecular mimicry). Immune reactions against these epitopes result in acute inflammatory demyelinating neuropathy or acute axonal forms. GBS has a worldwide distribution with an annual incidence of approximately 1.2-8.6 cases per 100,000 people. Both genders are at similar risk (but there is a slight male predominance). All ages are affected, although the distribution is bimodal. The supporting measures are critically important to provide optimal treatment. Immunomodulation with plasma exchange and intravenous immunoglobulin treatments shorten the disease course. Outcome is generally good, with virtually full recovery in 70-80 of the patients. In this review physiopathological aspects and clinical implications of GBS are fully discussed.

Significance of serum antibody to GD1b ganglioside in patients with Guillain-Barré syndrome.

BACKGROUND & OBJECTIVES: The precise etiological factors in Guillain-Barré syndrome (GBS) are still unknown. However, humoral and cellular immune factors may have a role in the pathogenesis of GBS. The present study was undertaken to evaluate the clinical significance of circulating serum IgG antibody to GD1b ganglioside in patients with GBS. METHODS: Serial samples of serum were collected from 18 patients with GBS undergoing plasma exchange (PE) during their hospital stay. Serum IgG antibody titers to GD1b, before, during as well as following PE were measured by an indirect enzyme-linked immunosorbent assay (ELISA). RESULTS: In 10 of 18 patients with GBS the antibody to GD1b was present in high titers (1:640-1:5120) prior to PE and the antibody titers in these 10 patients decreased following PE. At the time of completion of the study, the anti GD1b antibody titers declined in relation to clinical recovery in 7 of 10 patients with GBS. INTERPRETATION & CONCLUSION: The findings of the present study show that antibody to GD1b gangliosides may be one of the immunological factors in the pathogenesis of GBS and PE decreases the anti GD1b antibody titers in these patients.

Inmunorreacción de los anticuerpos monoclonales A3, E1 y P3 en tejido normal / Immunoreaction of the monoclonal antibodies A3, E1 and P3 in normal tissue

Conocer el reconocimiento tisular en órganos normales de estos anticuerpos monoclonales (AcMs) dirigidos contra moléculas de gangliósidos es importante como parte de su evaluación para uso diagnóstico. Las muestras fueron procesadas frescas y posteriormente fijadas en paraformaldehído. Los 3 sobrenadantes de cultivo se trabajaron puros y en todos los experimentos se utilizaron controles negativos y positivos. Los 3 AcMs reaccionaron con células del epitelio intestinal y el tejido conectivo de la submucosa. Los antígenos A3 y E1 se localizaron también en el epitelio traqueobronquial. El AcM E1 reaccionó con los epitelios renal y prostático que el A3 lo hizo en la epidermis y los ganglios autónomos mientéricos. La inmunotinción de las secreciones renales e intestinales con estos AcMs junto a las características del marcaje tisular pudieron sugerir la naturaleza de estos antígenos

Neuropatías autoinmunes: parte I: neuropatías y anticuerpos antineuronales / Autoimmune neurophaties: part I: neuropathy and antineural antibodies

Se trata de la primera parte de 2 artículos tendientes a aclarar el valor de exámenes serológicos en el diagnóstico de las neuropatías autoinmunes. Específicamente de los anticuerpos antineurales y de las gamapatías monoclonales. En esta primera revisión se presentan las posibilidades y limitaciones de los anticuerpos antineurales en manejo clínico de estas neuropatías. En primer lugar se describen las características generales de los distintos tipos de anticuerpos. Los síndromes polineuropáticos han sido divididos en motores, sensitivos, sensitivo/motores y Guillain-Barré, discutiéndose para cada caso la utilidad diagnóstica de los distintos anticuerpos antineurales

Ultrastructural localization of gangliosides, calcium and a high-affinity Ca(2+)-ATPase in nerve terminals: a contribution to the possible functional role of gangliosides.

By means of newly developed electron microscopical techniques (electron spectroscopic imaging, ESI; electron energy loss spectroscopy, EELS; immunogold labelling) a specific accumulation of endogenous calcium within the synaptic cleft and a distinct localization of a high-affinity Ca(2+)-ATPase at the inner sides of the pre- and postsynaptic membrane of nerve cells from fish brain have been demonstrated. Additionally, a differentiation-dependent expression of polysialoganglioside epitopes on the outer surface of nerve terminals in clustered arrangements was demonstrated using their ultracytochemical detection by means of the monoclonal antibody Q211. These results which are in agreement with parallel biochemical investigations on modulatory effects of exogenous gangliosides on a high-affinity Ca(2+)-ATPase in the CNS of vertebrates support our hypothesis that Ca(2+)-ganglioside complexes act as modulators for the processes of synaptic transmission and long-term neuronal adaptations.

Detecçäo de anticorpos e anticomponentes lipídicos (cardiolipina, gangliosídios e galactocerebrosídios) em pacientes com doença de Behçet / Detection of antibodies and lipid anticomponents (cardiolipin, gangliosides and galactosilceramidas) in patients with Behçet's disease

Os autores avaliam a presença de anticorpos antifraçöes lipídicas (cardiolipina, gangliosídios e galactocerebrosídios) em 13 pacientes com doença de Behcet. Anticorpos séricos anticardiolipina do isotipo IgG foram detectados em 30// dos casos, 50// do isotipo IgM. Em nenhum dos pacientes foi observada presença de isotipo IgA. Antigalactocerebrosídio foram detectados em apenas dois (15//) dos pacientes. Com relaçäo às manifestaçöes clínicas, observou-se que a presença de anticorpos anticardiolipina configura fator de risco para o desenvolvimento do acometimento neurológico

Neuropsychiatric manifestations of systemic lupus erythematosus: the value of anticardiolipin, antigangliosides and antigalactocerebrosides antibodies

In an attempt to find a serological marker for neuropsychiatric manifestations (NPM) of SLE sera from 66 patients (classified in three groups, according to their NPM - defined, probable and without NPM) were analysed by ELISA for IgG and antigalactocerebrosides antibodies. A strong correlation was found between IgM antigangliosides and antigalactocerebrosides antibodies and NPM, but not with IgG class. IgM and IgG antibodies anticardiolipin were not correlated with BPM in this study. Both IgM antigangliosides and seven patients with definid NPM but clinically inactive. The analysis of these autoantibodies showed an important role predictive for NPM in SLE; the negative test decreases the chance of the NPM