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1.
Transfus Apher Sci ; 39(3): 229-33, 2008 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-18955013

RESUMO

Limbic encephalitis (LE) is a neurological syndrome usually presenting in a paraneoplastic form. Recently many cases were reported with no concurring neoplasia, presenting with specific antibodies for voltage-gated potassium channel or for neuronal membrane antigens. Anti-glutamic acid decarboxylase (GAD) antibodies act against GABAergic receptors of the central nervous system. These antibodies were found in coeliac disease serum and in neurologic patients. We are reporting a case of a 21-year-old coeliac woman manifesting complex multiple-daily partial drug-resistant seizures for 7 years. The diagnosis was of a non paraneoplastic limbic encephalitis, unresponsive to high dose cortisone and IGIV infusion. The use of therapeutic plasma exchange (TPE) has resulted in an improvement in symptoms with quite a long disease-free period of time. When the frequency of the procedures was decreased, seizures appeared again and, after suspension of TPE, the clinical status worsened. The role of TPE in the treatment of LE still has to be defined.


Assuntos
Autoanticorpos/sangue , Glutamato Descarboxilase/imunologia , Encefalite Límbica/terapia , Troca Plasmática , Adulto , Anti-Inflamatórios/administração & dosagem , Autoanticorpos/imunologia , Doença Celíaca/sangue , Doença Celíaca/imunologia , Doença Celíaca/terapia , Cortisona/administração & dosagem , Feminino , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Fatores Imunológicos/administração & dosagem , Encefalite Límbica/sangue , Encefalite Límbica/imunologia
2.
J Neuroimmunol ; 199(1-2): 155-9, 2008 Aug 13.
Artigo em Inglês | MEDLINE | ID: mdl-18586329

RESUMO

Limbic encephalitis (LE) is a neurological syndrome that may present in association with cancer, infection, or as an isolate clinical condition often accompanying autoimmune disorders. Here we have characterized the clinical and laboratory features of two patients presenting with subacute onset, and chronic evolution, of anterograde amnesia and drug-resistant epilepsy associated with thyroid autoimmunity and in absence of tumoral pathology despite long follow-up. Antibodies against onconeural antigens, voltage gated potassium channel and glutamate receptors, which may accompany paraneoplastic as well as non-paraneoplastic LE, were negative. However, biochemical studies showed high titers, and sustained intrathecal synthesis, of antibodies directed against neuronal glutamic acid decarboxylase (GAD). In one patient, plasma exchange determined a dramatic improvement of the neurological deficits along with the decrease of autoantibodies.


Assuntos
Autoanticorpos/imunologia , Autoantígenos/imunologia , Encéfalo/patologia , Glutamato Descarboxilase/imunologia , Encefalite Límbica/imunologia , Encefalite Límbica/patologia , Adulto , Anticonvulsivantes/uso terapêutico , Autoanticorpos/líquido cefalorraquidiano , DNA Viral/líquido cefalorraquidiano , Diabetes Mellitus Tipo 1 , Feminino , Herpesvirus Humano 6 , Humanos , Encefalite Límbica/terapia , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Plasmaferese , Infecções por Roseolovirus/líquido cefalorraquidiano , Infecções por Roseolovirus/complicações , Convulsões/etiologia
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