Villegas-Castrejón Hilda, Hernández-Pérez Alma Delia, Peralta Sergio, Vázquez-Escamilla Jesús, Reyes-Marín Baltasar
Departomento de Morfología Celular y Molecular, Torre de Investigación, Instituto Nacional de Rehabilitación, México, DF.
Cir Cir. 2006 Nov-Dec;74(6):477-81.
Krabbe's leukodystrophy is a rare hereditary disease in Mexico. For that reason we report the case of an 11-year-old child. Ultrastructural studies of sural nerve biopsy specimen are described. Myelin sheaths were uniformly thin for the fiber diameters. Cytoplasm of Schwann cells exhibited a moderate dilatation with non-membrane masses with partly curvilinear, needle-shaped structures of variable length. The inclusions often had electron-dense or electron-lucent halos. These inclusions ultrastructurally represented Krabbe's leukodystrophy, and this method aids in the diagnosis in cases that are not available for genetic studies or special laboratory techniques. In this patient, diagnosis of Krabbe's disease was delayed and established several years after the initial symptoms. Electron microscopic examination of a sural nerve provided evidence for a diagnosis of Krabbe's leukodystrophy.
克拉伯病在墨西哥是一种罕见的遗传性疾病。因此,我们报告一例11岁儿童病例。描述了腓肠神经活检标本的超微结构研究。髓鞘对于纤维直径而言均一变薄。施万细胞的细胞质呈现中度扩张,伴有无膜团块,具有部分曲线形、不同长度的针状结构。这些内含物常有电子致密或电子透亮晕环。这些内含物在超微结构上代表克拉伯病,并且这种方法有助于在无法进行基因研究或特殊实验室技术的病例中进行诊断。在该患者中,克拉伯病的诊断被延迟,在最初症状出现几年后才得以确诊。腓肠神经的电子显微镜检查为克拉伯病的诊断提供了证据。