Cruz-Sanchez F F, Martos J A, Rives A, Ribalta T, Ferrer I, Cardesa A
Department of Pathology, Hostpiral Clinico y Provincial, Barcelona, Spain.
Clin Neurol Neurosurg. 1991;93(3):217-22. doi: 10.1016/s0303-8467(05)80006-x.
A 24-year-old man developed progressive dementia in seven years. The patient suffered a severe bronchopneumonia and eventually died few days later. Brain coronal sections showed a soft gray-brownish discoloration of white matter of centrum ovale but the subcortical arcuate fibers and the interne capsule were preserved. Microscopically, the white matter showed marked loss of myelin and oligodendrocytes, abundant hypertrophic astrocytes and numerous "globoid cells". The latter showed strong positivity in immunostains for a mouse monoclonal antigalactocerebroside antibody. The presence of these cells in the brain white matter might be the morphological basis to classify the present case as one of Krabbe's Leukodystrophy.
一名24岁男性在7年内出现进行性痴呆。该患者患严重支气管肺炎，数日后最终死亡。脑冠状切片显示卵圆中心白质呈软的灰棕色变色，但皮质下弓形纤维和内囊保存完好。显微镜下，白质显示髓鞘和少突胶质细胞明显丢失，大量肥大星形胶质细胞和众多“球状细胞”。后者在小鼠单克隆抗半乳糖脑苷脂抗体免疫染色中呈强阳性。脑白质中这些细胞的存在可能是将本病例归类为克拉伯病（异染性脑白质营养不良）的形态学基础。
