Department of Neurology, Mayo Clinic, Rochester, MN, USA.
Department of Radiology, Mayo Clinic, Rochester, MN, USA.
Mult Scler. 2019 Apr;25(5):709-714. doi: 10.1177/1352458518767045. Epub 2018 Mar 23.
Episodic hypothermia (EH) can occur in multiple sclerosis (MS). The putative mechanism is impairment of thermoregulation due to a presumed demyelinating hypothalamic lesion.
To describe a cohort of patients with MS, who developed EH.
Patients were identified through review of the Mayo Clinic electronic medical record (1996 to July 2015). Search terms were [multiple sclerosis] or [MS] within the diagnoses field and [hypothermia] within any field. We reviewed records for accuracy of diagnoses and abstracted relevant data. Magnetic resonance imaging (MRI) was reviewed for presence of hypothalamic lesions.
Of 156 patients, 34 had concurrent MS and hypothermia. Thirty-two (94%) had progressive disease at EH onset. Median MS duration was 19.9 years, and median expanded disability status scale (EDSS) was 8.0. Most patients presented with alterations in consciousness. Infection was suspected as the precipitating factor in 19 (56%), but clinically/laboratory supported in only 9 (28%). MRI lesions were evident within the hypothalamus in only 4 (14%).
EH occurs predominantly in patients with advanced secondary progressive MS. The major manifestation is altered consciousness. Infection is often suspected as causal, but infrequently confirmed. Although commonly implicated, hypothalamic lesions were rarely evident on MRI and were absent in two post-mortem evaluations.
发作性低体温(EH)可发生在多发性硬化症(MS)中。推测的机制是由于假定的下丘脑脱髓鞘病变导致体温调节受损。
描述一组发生 EH 的 MS 患者。
通过对梅奥诊所电子病历(1996 年至 2015 年 7 月)的审查来确定患者。搜索词为诊断字段中的 [多发性硬化症] 或 [MS] 和任何字段中的 [低体温]。我们检查了记录以确保诊断的准确性,并提取了相关数据。对磁共振成像(MRI)进行了下丘脑病变的检查。
在 156 名患者中,有 34 名同时患有 MS 和低体温。32 名(94%)在 EH 发作时患有进行性疾病。MS 病程的中位数为 19.9 年,扩展残疾状态量表(EDSS)的中位数为 8.0。大多数患者表现为意识改变。19 例(56%)怀疑感染是诱发因素,但仅 9 例(28%)有临床/实验室支持。MRI 病变仅在 4 例(14%)中显示在下丘脑内。
EH 主要发生在进展性继发性 MS 患者中。主要表现为意识改变。感染常被怀疑是病因,但很少得到证实。尽管经常被牵涉在内,但下丘脑病变很少在 MRI 上显现,并且在两次尸检评估中都不存在。