Narita T, Yamano T, Ohno M, Takano T, Ito R, Shimada M
Department of Pediatrics, Shiga University of Medical Science, Japan.
Neuropediatrics. 1998 Oct;29(5):265-7. doi: 10.1055/s-2007-973572.
A female patient, who was diagnosed with Leigh syndrome at 15 months of age, developed fulminating severe hypertension and died at 8 years of age. Hypertension has not been reported as an important clinical symptom in Leigh syndrome. Laboratory findings indicated that it was not associated with endocrinopathic diseases such as pheochromocytoma and aldosteronism, or renal diseases. Brain MRI scan showed symmetrical lesions in the basal ganglia and medulla oblongata including the nucleus tractus solitarius. This nucleus is known to play an important role in maintaining blood pressure. Since the medulla oblongata is a vulnerable site, potential development of hypertension should be taken into consideration when managing Leigh syndrome.
一名女性患者在15个月大时被诊断为 Leigh 综合征,8岁时出现暴发性严重高血压并死亡。高血压在Leigh综合征中尚未被报道为重要的临床症状。实验室检查结果表明,它与嗜铬细胞瘤和醛固酮增多症等内分泌疾病或肾脏疾病无关。脑部MRI扫描显示基底神经节和延髓包括孤束核有对称性病变。已知该核在维持血压方面起重要作用。由于延髓是一个易损部位,在管理Leigh综合征时应考虑高血压的潜在发展。