Valente Luca G, Antwi Kwadwo, Nicolas Guillaume P, Wild Damian, Christ Emanuel
University of Bern, Switzerland.
Division of Nuclear Medicine, University Hospital of Basel, Switzerland.
Swiss Med Wkly. 2018 Nov 18;148:w14682. doi: 10.4414/smw.2018.14682. eCollection 2018 Nov 5.
BACKGROUND Important causes of endogenous hyperinsulinaemic hypoglycaemia (EHH) in adult patients are insulinoma and adult nesidioblastosis. Data on main symptoms in EHH are scarce and controversial. We analysed main symptoms of patients with EHH in the framework of two prospective studies investigating glucagon-like peptide-1 receptor imaging. METHODS Patients were referred from secondary European endocrine centres and endocrinologists. Inclusion criteria were biochemically proven EHH (glucose <2.5 mmol/l in the presence of inadequate insulin and C-peptide levels) with neurological hypoglycaemic symptoms. Demographic characteristics and aetiologies of the patients with EHH were retrieved. Main symptoms were categorised into neurological, sympathicoadrenal (sweating, tremor, palpitation, hunger, shivering and pallor) and nonspecific other symptoms (nonspecific asthenia, weight gain, gastrointestinal symptoms and headaches). Neurological symptoms were subdivided into moderately impaired consciousness (confusion, dizziness, somnolence and delirium), visual, speech and sensorimotor impairment, severely impaired consciousness (loss of consciousness and apathy), attention deficit, seizures and personality changes. Biochemical assessment and duration of EHH at the end of a fasting test were recorded. RESULTS Fifty-four patients with full documentation were included in the analysis (74% female; mean age 54 years, range 22–84). Median duration from onset of symptoms to diagnosis of EHH was 12 months (range 0–120). Fifty (92.6%) patients had neurological symptoms, including moderately impaired consciousness (46.3%), visual, speech and sensorimotor function impairment (44.4%), severely impaired consciousness (37%), attention deficit (31.5%), seizures (16.7%) and personality change (13%). Sympathicoadrenal symptoms were present in 33 (61.1%) patients. Nonspecific other symptoms occurred in 36 (66.7%) patients. 43 patients (79.6%) suffered from symptoms of at least two different categories. CONCLUSIONS Clinical symptoms of EHH are characterised by a wide variety of mainly different neurological symptoms (“neurological chameleon”). EHH should be considered as a differential diagnosis in many neurological disorders. Trial registration numbers NCT00937079 & NCT02127541
背景 成年患者内源性高胰岛素血症性低血糖症（EHH）的重要病因是胰岛素瘤和成人胰岛细胞增殖症。关于EHH主要症状的数据稀少且存在争议。我们在两项研究胰高血糖素样肽-1受体成像的前瞻性研究框架内分析了EHH患者的主要症状。
方法 患者来自欧洲二级内分泌中心和内分泌科医生。纳入标准为经生化证实的EHH（血糖<2.5 mmol/L，同时胰岛素和C肽水平不足）且伴有神经性低血糖症状。收集EHH患者的人口统计学特征和病因。主要症状分为神经症状、交感肾上腺症状（出汗、震颤、心悸、饥饿、寒战和面色苍白）和非特异性其他症状（非特异性乏力、体重增加、胃肠道症状和头痛）。神经症状又细分为意识中度受损（意识模糊、头晕、嗜睡和谵妄）、视觉、言语和感觉运动功能受损、意识严重受损（意识丧失和淡漠）、注意力缺陷、癫痫发作和人格改变。记录空腹试验结束时的生化评估结果和EHH病程。
结果 54例有完整记录的患者纳入分析（74%为女性；平均年龄54岁，范围22 - 84岁）。从症状出现到诊断为EHH的中位病程为12个月（范围0 - 120个月）。50例（92.6%）患者有神经症状，包括意识中度受损（46.3%）、视觉、言语和感觉运动功能受损（44.4%）、意识严重受损（37%）、注意力缺陷（31.5%）、癫痫发作（16.7%）和人格改变（13%）。33例（61.1%）患者有交感肾上腺症状。36例（66.7%）患者有非特异性其他症状。43例（79.6%）患者至少有两种不同类型的症状。
结论 EHH的临床症状以多种主要不同的神经症状为特征（“神经变色龙”）。在许多神经系统疾病中应考虑将EHH作为鉴别诊断。试验注册号NCT00937079和NCT02127541
