Bazille Céline, Megarbane Bruno, Bensimhon Dan, Lavergne-Slove Anne, Baglin Anne Catherine, Loirat Philippe, Woimant France, Mikol Jacqueline, Gray Françoise
Service Central d'Anatomie et de Cytologie Pathologiques, Hôpital Lariboisière, Paris, France.
J Neuropathol Exp Neurol. 2005 Nov;64(11):970-5. doi: 10.1097/01.jnen.0000186924.88333.0d.
Cerebellar syndromes and radiologic cerebellar atrophy after hyperpyrexia have occasionally been reported, mostly in neuroleptic malignant syndromes, but neuropathologic studies are extremely rare. We studied 3 patients (a 74-year-old woman, a 63-year-old man, and an 80-year-old man) who had heat stroke during heat waves in France. One patient had generalized seizures and died 28 hours after admission. The other patients survived one month and 2 months after admission; both had palatal myoclonus, and in one case, magnetic resonance imaging showed high signal intensity in the cerebral peduncles. The main neuropathology in the 3 cases was severe diffuse loss of Purkinje cells associated with heat shock protein 70 expression by Bergmann glia. In situ end labeling was negative in surviving Purkinje cells, suggesting that the mechanism of neuronal death was not apoptosis. Degeneration of Purkinje cells axons resulted in myelin pallor of the white matter of the folia and of the hilum of the dentate nuclei. DNA internucleosomal breakages were identified by in situ end labeling in the dentate nuclei and centromedian nuclei of the thalamus and were associated with degeneration of the cerebellar efferent pathways: superior cerebellar peduncles, decussation of the superior cerebellar peduncles (Wernekinck commissure), and dentatothalamic tract. These findings suggest that the mechanisms of neuronal death in the dentate nuclei and centromedian nuclei of the thalamus was different from that in Purkinje cells and more likely resulted from deafferentation. Ammon's horn and other areas susceptible to hypoxia were spared. These observations confirm the selective vulnerability of Purkinje cells to heat-induced injury and involvement of the cerebellar efferent pathways in palatal myoclonus.
高热后出现的小脑综合征及放射学小脑萎缩偶尔有报道，大多见于神经精神性恶性综合征，但神经病理学研究极为罕见。我们研究了3例在法国热浪期间发生中暑的患者（1名74岁女性、1名63岁男性和1名80岁男性）。1例患者出现全身性惊厥，入院28小时后死亡。另外两名患者分别在入院后1个月和2个月存活；两人均有腭肌阵挛，其中1例磁共振成像显示脑桥有高信号强度。3例患者的主要神经病理学表现为浦肯野细胞严重弥漫性丢失，同时伯格曼胶质细胞表达热休克蛋白70。存活的浦肯野细胞原位末端标记呈阴性，提示神经元死亡机制并非凋亡。浦肯野细胞轴突的退变导致小叶白质和齿状核门的髓鞘苍白。通过原位末端标记在齿状核以及丘脑中央中核中发现了DNA核小体间断裂，这与小脑传出通路的退变有关：小脑上脚、小脑上脚交叉（韦内肯克连合）和齿状丘脑束。这些发现提示，丘脑齿状核和中央中核的神经元死亡机制与浦肯野细胞不同，更可能是由传入神经阻滞所致。海马及其他易受缺氧影响的区域未受影响。这些观察结果证实了浦肯野细胞对热诱导损伤的选择性易损性以及小脑传出通路在腭肌阵挛中的作用。
