Spinocerebellar ataxia (Machado-Joseph’s disease: three case reports

Introduction: Spinocerebellar ataxias (SCA) are genetic neuro-degenerative diseases that are characterized by a progressive loss of cerebellar neurons. In Brazil, the most common form of SCA is the SCA3, also known as Machado-Joseph Disease (MJD). Objective: To describe the feelings and expectations...

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Detalles Bibliográficos
Autores: Cyrne, Dayane Alves Brandão, Malagutti, William, Barnabé, Anderson Sena, Fornari, João Victor, Rodrigues, Francisco Sandro Menezes, Ferraz, Renato Ribeiro Nogueira
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2011
País:Brasil
Institución:Universidade Nove de Julho (UNINOVE)
Repositorio:Revista Conscientiae Saúde (Online)
Idioma:portugués
OAI Identifier:oai:ojs.periodicos.uninove.br:article/2374
Acceso en línea:https://periodicos.uninove.br/saude/article/view/2374
Access Level:acceso abierto
Palabra clave:Ataxia
Genetics
Machado-Joseph disease
Neurology.
Doença de Machado-Joseph
Genética
Neurologia.
Descripción
Sumario:Introduction: Spinocerebellar ataxias (SCA) are genetic neuro-degenerative diseases that are characterized by a progressive loss of cerebellar neurons. In Brazil, the most common form of SCA is the SCA3, also known as Machado-Joseph Disease (MJD). Objective: To describe the feelings and expectations of the MJD patients about their own illness. Method: We conducted interview with targeted three patients with MJD from the same family. Results and conclusion: The respondents know that the disease is hereditary, have ataxia, dysmetria, motor coordination difficulties and depression. The severity of the disease is perceived through the deaths experienced between family members. The MJD brings major changes in activities of daily living, leading to need help from others. The participants’ feelings toward the disease are expressed ambiguously, appearing rebellion and conformity.