Ghay Richa, Thaman Dushyant, Kahlon Nikita
Department of Physiology, Sri Guru Ram Das Institute of Medical Sciences and Research, Amritsar, Punjab, India.
J Basic Clin Physiol Pharmacol. 2013;24(2):159-61. doi: 10.1515/jbcpp-2012-0067.
Bilateral temporal lobe agenesis/hypogenesis along with middle cranial fossa arachnoid cysts (ACs) is extremely rare, and very few cases have been reported in the literature.
We present the case of 2-year-old female presenting with chief complaints of headache, vomiting, and fever. There was a history of premature delivery in the seventh month and up-rolling of eyes since birth.
Magnetic resonance imaging findings revealed bilateral temporal lobe agenesis with bilateral ACs, along with encephalomalacia in both parietal lobes. Ultrasound abdomen findings and biochemical tests revealed renal parenchymal disease. The lipid profile analysis revealed highly elevated triglyceride levels to 457.4 mg% and also enhanced very-low-density lipoprotein levels to 91.5 mg%. Presently, the patient is being treated on conservative lines.
To our knowledge, this is the first report of an unusual association between bilateral temporal lobe agenesis associated with renal parenchymal disease and dyslipidaemia.
双侧颞叶发育不全/发育不良伴中颅窝蛛网膜囊肿（ACs）极为罕见，文献报道的病例极少。
我们报告了一名2岁女性患者，主要症状为头痛、呕吐和发热。该患者有7个月早产史，自出生起就有眼球上翻症状。
磁共振成像结果显示双侧颞叶发育不全伴双侧ACs，同时双侧顶叶存在脑软化。腹部超声检查结果和生化检查显示存在肾实质疾病。血脂分析显示甘油三酯水平大幅升高至457.4mg%，极低密度脂蛋白水平也升高至91.5mg%。目前，该患者正在接受保守治疗。
据我们所知，这是首例关于双侧颞叶发育不全与肾实质疾病及血脂异常之间罕见关联的报告。
