Herath H M M T B, Kulatunga Aruna
National Hospital of Sri Lanka, Colombo, Sri Lanka.
Egypt J Neurol Psychiatr Neurosurg. 2020;56(1):15. doi: 10.1186/s41983-020-0149-9. Epub 2020 Jan 29.
Systemic lupus erythematosus is a connective tissue disorder, which causes complex multi organ involvement. Neurological and cardiac manifestations have been well noted but complications such as status epilepticus and acute myocarditis with heart failure at presentation remains uncommon.
A 15-year-old, previously healthy, South Asian, Sri Lankan female presented with status epilepticus and the seizures only responded to intravenous midazolam and thiopentone sodium. On the fourth day, she developed tachycardia and shortness of breath and was found to have cardiomyopathy with heart failure with an ejection fraction 40%. Along with a positive urinary sediment, a positive ANA with a very high level of ds-DNA and low C3 and C4 levels confirmed our suspicion of systemic lupus erythematosus.
Systemic lupus erythematosus presents in a variety of clinical presentations and the spectrum may range from unique to ubiquitous. Clinicians should have a high index of suspicion specially when encountering atypical presentations with multi-organ involvement, especially when patients tend to be young females. Status epilepticus and myocarditis are uncommon manifestations of systemic lupus erythematosus, and should be appreciated early, as if inappropriately managed would have a deleterious impact on mortality and morbidity.
系统性红斑狼疮是一种结缔组织疾病，可导致复杂的多器官受累。神经和心脏表现已广为人知，但癫痫持续状态和急性心肌炎伴心力衰竭等并发症在发病时仍不常见。
一名15岁、既往健康的南亚斯里兰卡女性，以癫痫持续状态就诊，癫痫发作仅对静脉注射咪达唑仑和硫喷妥钠有反应。第四天，她出现心动过速和呼吸急促，被发现患有心肌病伴心力衰竭，射血分数为40%。除了尿沉渣阳性外，抗核抗体阳性、双链DNA水平极高以及C3和C4水平降低证实了我们对系统性红斑狼疮的怀疑。
系统性红斑狼疮有多种临床表现，其范围可能从独特到普遍存在。临床医生应高度怀疑，特别是在遇到多器官受累的非典型表现时，尤其是当患者为年轻女性时。癫痫持续状态和心肌炎是系统性红斑狼疮的罕见表现，应尽早识别，因为处理不当会对死亡率和发病率产生有害影响。
