BACKGROUND: 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.METHODS: 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.RESULTS: 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.CONCLUSIONS: To our knowledge, this is the first report of an unusual association between bilateral temporal lobe agenesis associated with renal parenchymal disease and dyslipidaemia.
Abdomen Amino Acid Metabolism, Inborn Errors