Assuntos
Encéfalo/fisiopatologia , Eletroencefalografia/métodos , Demência Frontotemporal/diagnóstico por imagem , Encefalite Límbica/diagnóstico por imagem , Transtornos da Memória/etiologia , Pólipos Adenomatosos/diagnóstico por imagem , Idoso , Diagnóstico Diferencial , Humanos , Encefalite Límbica/diagnóstico , Encefalite Límbica/patologia , Imageamento por Ressonância Magnética , Masculino , Doença de Pick , Tomografia por Emissão de PósitronsRESUMO
Rebuilding brain structure and neural circuitries by transplantation of fetal tissue is a strategy to repair the damaged nervous system and is currently being investigated using striatal primordium in Huntington's disease (HD) patients. Four HD patients underwent bilateral transplantation with human fetal striatal tissues (9-12 week gestation). Small blocks of whole ganglionic eminencies were processed to obtain cell suspension and then stereotactically grafted in the caudate head and in the putamen. Follow-up period ranged between 18 and 34 months (mean, 24.7 months). Surgery was uneventful. Starting from the fourth month after grafting, neo-generation of metabolically active tissue with striatal-like MRI features was observed in 6 out of 8 grafts. The increase in D2 receptor binding suggested striatal differentiation of the neo-generated tissue in 3 patients. New tissue, connecting the developing grafts with the frontal cortex and, in one case, with the ventral striatum, was also observed. The new tissue growth halted after the ninth month post transplantation. All patients showed stabilization or improvement in some neurological indices. No clinical and imaging signs, suggestive of graft uncontrolled growth, were seen. This study provides the first evidence in humans that neuroblasts of a striatal primordium can develop and move into the brain after neurotransplantation. Primordium development resulted in the building of a new structure with the same imaging features as the corresponding mature structure, combined with short- and long-distance targeted migration of neuroblasts. The results of this study support both the reconstructive potential of fetal tissue and the remarkably retained plasticity of adult brain. Further studies are necessary to assess the clinical efficacy of the human fetal striatal transplantation.
Assuntos
Transplante de Tecido Encefálico/métodos , Corpo Estriado/transplante , Doença de Huntington/fisiopatologia , Doença de Huntington/cirurgia , Adulto , Movimento Celular/fisiologia , Corpo Estriado/citologia , Corpo Estriado/diagnóstico por imagem , Ensaio de Imunoadsorção Enzimática/métodos , Feminino , Feto , Fluordesoxiglucose F18 , Seguimentos , Regulação da Expressão Gênica/fisiologia , Antígenos HLA/metabolismo , Humanos , Doença de Huntington/diagnóstico por imagem , Imageamento Tridimensional/métodos , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Proteínas do Tecido Nervoso/genética , Proteínas do Tecido Nervoso/metabolismo , Exame Neurológico/métodos , Ligação Proteica/fisiologia , RNA Mensageiro/metabolismo , Receptores de Dopamina D2/metabolismo , Índice de Gravidade de Doença , Tomografia Computadorizada de Emissão de Fóton Único/métodosRESUMO
Replacement of damaged neuronal population by fetal tissue transplantation represents a potential treatment for neurodegenerative diseases. Consistent success has been achieved with fetal striatal transplantation in Huntington's disease animal models and patients. We report the neo-generation of metabolically active tissue with striatum-like imaging features after transplantation of striatal primordia in a patient with Huntington's disease. This study represents the first "in vivo" demonstration that a human striatal anlagen, transplanted into the adult human brain, is able to progress in its development and to generate a new anatomical structure in the host, without evidence of neoplasia or teratoma.
Assuntos
Transplante de Tecido Encefálico/métodos , Corpo Estriado/embriologia , Corpo Estriado/transplante , Transplante de Tecido Fetal/métodos , Sobrevivência de Enxerto/fisiologia , Doença de Huntington/terapia , Adulto , Células Cultivadas , Corpo Estriado/metabolismo , Metabolismo Energético/fisiologia , Feminino , Lateralidade Funcional/fisiologia , Humanos , Doença de Huntington/patologia , Doença de Huntington/fisiopatologia , Imageamento por Ressonância Magnética , Plasticidade Neuronal/fisiologia , Neuronavegação , Tomografia por Emissão de Pósitrons , Técnicas Estereotáxicas , Resultado do TratamentoRESUMO
We describe and present a videotape of a 57-year-old woman admitted to our Neurological Clinic at 46 years of age due to extrapyramidal manifestations suggesting Parkinson's disease (PD) and with a brain magnetic resonance imaging scan showing multi-infarctual leukoencephalopathy. Various investigations led to the diagnosis of Anderson Fabry's disease (AFD). We discuss the possibility of correlation between the patient's parkinsonism and AFD.