RESUMO
Guillain-Barrè syndrome (GBS) is usually associated with symmetrical weakness, and therefore asymmetrical weakness may confuse diagnosis. We report on a patient with GBS subsequent to Campylobacter jejuni enteritis who had asymmetrical weakness with CNS involvement. The patient tested positive for anti-ganglioside antibodies, including anti-GM1 IgM, anti-GD1b IgG, and anti-GT1a IgG. Patients with GBS can manifest asymmetrical signs and symptoms attributable to CNS involvement. Prompt, accurate diagnosis and treatment of post-C. jejuni GBS is especially important because its prognosis is relatively poor.
Assuntos
Doença de Alzheimer/genética , Mutação de Sentido Incorreto , Presenilina-1/genética , Adulto , Idade de Início , Substituição de Aminoácidos , Análise Mutacional de DNA , Etilsuccinato de Eritromicina , Feminino , Genes Dominantes , Genótipo , Humanos , Japão , Masculino , Análise de Sequência com Séries de Oligonucleotídeos , FenótipoRESUMO
A patient with acute oropharyngeal palsy associated with internal ophthalmoplegia was reported. A 13-year-old boy had fever and diarrhea for two days. Ten days after resolution of these symptoms, he noticed difficulty in speaking (day 1). Neurological findings on day 4 included bilateral mydriasis, right abducens nerve palsy, nasal voice with absent pharyngeal reflex. Although superficial sensation was preserved, vibratory sensation was reduced in distal limbs. Tendon reflexes were generally absent. Neither ataxia nor dysautonomia was seen. Serum anti-glycolipid antibody assay on day 4 disclosed elevated IgG antibodies to GQ1b and GT1a. His cerebrospinal fluid on day 21 contained 6 mononuclear cells/microl with 137 mg/dl of total protein. Nerve conduction study on day 5 showed minimal sensory nerve involvement. Quantitative sudomotor axon reflex test was normal in the lower extremities. Low dose pilocarpine eyedrops dilated his pupils. Although mild cerebellar-like ataxia appeared on day 5, intravenous immunoglobulin (0.4 g/kg/day for four days) rapidly improved his neurological abnormalities. IgG anti-GQ1b antibody might contribute not only oropharyngeal weakness but also internal ophthalmoplegia in this patient.
Assuntos
Autoanticorpos/sangue , Gangliosídeos/imunologia , Imunoglobulina G/imunologia , Síndrome de Miller Fisher/imunologia , Oftalmoplegia/complicações , Paralisia/imunologia , Doença Aguda , Adolescente , Humanos , Masculino , Síndrome de Miller Fisher/diagnóstico , Orofaringe/imunologiaRESUMO
Serum antibody activities to mixtures of a ganglioside and various phospholipids were compared with those to a ganglioside alone in 30 anti-GM1 IgG-positive GBS patients and 30 anti-GQ1b IgG-positive Miller Fisher syndrome (MFS) patients. Anti-GM1-positive sera had higher antibody reactivities against a mixture of GM1 and several phospholipids including PA, PI and PS, than against GM1 alone. In contrast, in case of anti-GQ1b antibody, no phospholipid provided significant enhancement. Sphingomyelin provided decrease of the activity for both anti-GM1 and anti-GQ1b IgG. The effects of phospholipids must be considered to determine the pathogenetic role of antiganglioside antibodies in GBS and MFS.
Assuntos
Sítios de Ligação de Anticorpos , Gangliosídeo G(M1)/imunologia , Gangliosídeo G(M1)/metabolismo , Gangliosídeos/imunologia , Gangliosídeos/metabolismo , Síndrome de Guillain-Barré/imunologia , Imunoglobulina G/metabolismo , Fosfolipídeos/farmacologia , Adjuvantes Imunológicos/farmacologia , Sítios de Ligação de Anticorpos/efeitos dos fármacos , Cardiolipinas/farmacologia , Síndrome de Guillain-Barré/sangue , Humanos , Imunoglobulina M/metabolismo , Lisofosfatidilcolinas/farmacologia , Lisofosfolipídeos/farmacologia , Ácidos Fosfatídicos/farmacologia , Fosfatidilcolinas/farmacologia , Fosfatidilinositóis/farmacologia , Fosfatidilserinas/farmacologia , Esfingomielinas/imunologia , Esfingomielinas/metabolismoRESUMO
Antibodies specific for a complex of gangliosides GD1a and GD1b (GD1a/GD1b) were found in sera from eight of 100 patients with Guillain-Barre syndrome (GBS) by the use of enzyme-linked immunosorbent assay and thin-layer chromatogram immunostaining. Those sera also had antibody activities to such ganglioside complexes as GD1a/GM1, GD1b/GT1b, and GM1/GT1b but had little or no reactivity to the each isolated antigen. Clustered epitopes of the ganglioside complex in the plasma membrane may be targeted by such an antibody, and interaction between the antibody and ganglioside complex may induce the neuropathy.
Assuntos
Anticorpos/sangue , Gangliosidoses/imunologia , Síndrome de Guillain-Barré/sangue , Síndrome de Guillain-Barré/imunologia , Adulto , Idoso , Cromatografia em Camada Fina/métodos , Ensaio de Imunoadsorção Enzimática/métodos , Feminino , Gangliosídeos/sangue , Gangliosídeos/imunologia , Gangliosidoses/sangue , Humanos , Immunoblotting/métodos , Masculino , Pessoa de Meia-IdadeRESUMO
Anti-GM1 immunoglobulin G (IgG) antibodies are frequently present in sera from patients with Guillain-Barré syndrome (GBS). A previous report on a patient who had a neuropathy with immunoglobulin M (IgM) M-protein binding to a conformational epitope formed by phosphatidic acid (PA) and gangliosides prompted us to investigate the binding of IgG antibodies in GBS sera to a mixture of GM1 and PA (GM1/PA). Of 121 GBS patients, 32 had anti-GM1 IgG antibodies. All 32 also had antibody activity against GM1/PA. Twenty-five (78%) of 32 patients had greater activity against GM1/PA than against GM1 alone. Twelve patients who had no anti-GM1 IgG antibodies had IgG antibody activity against GM1/PA. No GBS patient had IgG antibody against PA alone. In contrast, two rabbit anti-GM1 antisera had greater activity against GM1 alone than against GM1/PA. IgG antibody with greater binding activity against a mixture of GM1 and a phospholipid than against GM1 alone may have an important role in the pathogenesis of GBS and has implications for diagnosis.