RESUMEN
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.
Asunto(s)
Adolescente , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Anticuerpos Antiidiotipos/sangre , Encefalitis/diagnóstico , Gangliósidos/sangre , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Miller Fisher/diagnóstico , Oftalmoplejía/diagnóstico , Tronco Encefálico , Encefalitis/tratamiento farmacológico , Gangliósidos/inmunología , Síndrome de Guillain-Barré/tratamiento farmacológico , Inmunoglobulinas Intravenosas/uso terapéutico , Imagen por Resonancia Magnética , Síndrome de Miller Fisher/tratamiento farmacológico , Oftalmoplejía/tratamiento farmacológicoRESUMEN
Anti-GQ1b syndrome includes Miller Fisher Syndrome (MFS), Guillain Barré Syndrome (GBS), Bicker staff`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.
Asunto(s)
Anticuerpos Antiidiotipos/sangre , Encefalitis/diagnóstico , Gangliósidos/sangre , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Miller Fisher/diagnóstico , Oftalmoplejía/diagnóstico , Adolescente , Adulto , Anciano , Tronco Encefálico , Encefalitis/tratamiento farmacológico , Femenino , Gangliósidos/inmunología , Síndrome de Guillain-Barré/tratamiento farmacológico , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Síndrome de Miller Fisher/tratamiento farmacológico , Oftalmoplejía/tratamiento farmacológicoRESUMEN
Recent studies have shown that antiganglioside antibodies, particularly those associated with the disialosyl group, may be involved in immune-mediated sensory peripheral neuropathies. We report the results of plasma screening for antiganglioside antibodies in two patients with chronic ataxic neuropathy. We found reactivity against gangliosides GD3, GD1b, and GT1b in one of them and against GD1a in the other, even though both had nearly identical clinical pictures. Results suggest that anti-GD1a antibodies, which are usually associated with motor polyneuropathy, may also be involved in the pathogenesis of clinically pure sensory polyneuropathy.
Asunto(s)
Anticuerpos/sangre , Ataxia/inmunología , Gangliósidos/inmunología , Adulto , Ataxia/sangre , Enfermedad Crónica , Extremidades , Gangliósidos/sangre , Humanos , MasculinoRESUMEN
Recent studies have shown that antiganglioside antibodies, particularly those associated with the disialosyl group, may be involved in immune-mediated sensory peripheral neuropathies. We report the results of plasma screening for antiganglioside antibodies in two patients with chronic ataxic neuropathy. We found reactivity against gangliosides GD3, GD1b, and GT1b in one of them and against GD1a in the other, even though both had nearly identical clinical pictures. Results suggest that anti-GD1a antibodies, which are usually associated with motor polyneuropathy, may also be involved in the pathogenesis of clinically pure sensory polyneuropathy (AU)#S#a
Asunto(s)
Humanos , Masculino , Adulto , RESEARCH SUPPORT, NON-U.S. GOVT , Gangliósidos/inmunología , Anticuerpos/inmunología , Ataxia/inmunología , Gangliósidos/sangre , Anticuerpos/sangre , Ataxia/sangre , Extremidades , Enfermedad Crónica , Cromatografía Líquida de Alta Presión/métodos , Cromatografía en Capa Delgada/métodosRESUMEN
An abnormal urinary excretion of sulphated glycosaminoglycans in a patient with GM-2 gangliosidosis (Tay-Sachs disease) is described. Besides the accumulation of GM-2 ganglioside in liver and lack of hexosaminidase A, the patient shows an abnormal urinary excretion of an iduronic acid-rich low molecular weight heparan sulphate. Also, no dermatan sulphate could be detected in the urine, whereas this compound was the main sulphated glycosaminoglycan in the liver of the patient. Heparan sulphate was the main glycosaminoglycan of normal liver. The total amount of sulphated glycosaminoglycans in the urine and liver of the patient did not differ significantly from the amounts found in the liver and urine of normal subjects. Several plasma glycosidases have been assayed and the activities did not differ significantly from the values obtained for the plasma of normal subjects.