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犬球状细胞脑白质营养不良脊髓病变的定量评估

Quantitative evaluation of spinal cord lesions in canine globoid leukodystrophy.

作者信息

Fletcher T F, Jessen C R, Bender A P

出版信息

J Neuropathol Exp Neurol. 1977 Jan;36(1):84-99. doi: 10.1097/00005072-197701000-00009.

DOI:10.1097/00005072-197701000-00009
PMID:833619
Abstract

Lesion distribution in the spinal cord was investigated by sampling an average of 26 spinal cord segments in each of ten dogs severely affected by canine globoid leukodystrophy (GLD). GLD lesions were quantified by subdividing spinal white matter into small unit areas and judging the quartile extent of lesion involvement for each unit area. Along the length of the spinal cord, lesions declined from cranial to caudal, decreasing precipitously in the midlumbar region. The lumbosacral region had the least amount of lesion, and, based on lesion per unit area, the thoracic region was most severely involved. The dorsal funiculus had the greatest concentration and the least dispersion of lesion generally amoung the three funiculi. White matter at the periphery of the spinal cord was involved earlier and more severely than white matter adjacent to gray matter. Amount of lesion per spinal cord could be estimated adequately by sampling one segment from each of five regions. Dogs could be divided into two groups based on rate of lesion development. Clinical signs did not correlate closely with total amount of lesion per spinal cord. Of the total amount of variability among dogs in amount of spinal pathologic involvement, 78% could be accounted for by a mathematical model expressing spinal cord pathologic involvement as a quadratic function of age at onset and duration of the clinical syndrome.

摘要

通过对10只患有严重犬球状细胞脑白质营养不良(GLD)的犬,平均每只犬取26个脊髓节段进行采样,研究脊髓中的病变分布。通过将脊髓白质细分为小单位区域,并判断每个单位区域病变累及的四分位数范围,对GLD病变进行量化。沿着脊髓长度,病变从颅端向尾端逐渐减少,在腰中部区域急剧下降。腰骶部区域的病变最少,基于单位面积的病变情况,胸部区域受累最严重。在三个脊髓索中,背侧脊髓索的病变通常集中程度最高且分散程度最小。脊髓外周的白质比灰质附近的白质更早且更严重地受累。通过从五个区域各取一个节段进行采样,可以充分估计每条脊髓的病变数量。根据病变发展速度,犬可分为两组。临床症状与每条脊髓的病变总量没有密切相关性。在犬脊髓病理受累程度的总变异性中,78%可以用一个数学模型来解释,该模型将脊髓病理受累表示为发病年龄和临床综合征持续时间的二次函数。

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