Transient hypoglycorrhachia with paroxysmal abnormal eye movement in early infancy.

Paroxysmal abnormal eye movement in early infancy is one of the initial symptoms of glucose transporter 1 deficiency syndrome (GLUT1DS). We describe four early infants with transient hypoglycorrhachia presenting with abnormal eye movements. Their symptoms disappeared after the introduction of a ketogenic diet (KD), and their development was normal. Since no variants in SLC2A1 were detected, the CSF-to-blood glucose ratios (C/B) were re-examined, and within normal range. None of the four patients displayed recurrent symptoms after withdrawal from the KD. Because long-term KD has potential adverse effects and could affect the quality of life of patients and their families, re-examination of CSF glucose during late infancy should be considered in the case of absence of the SLC2A1 pathogenic variant.

[A Suspected Case of Hemorrhagic Hereditary Telangiectasia Presented with Cerebral Hemorrhage in Infancy].

Hereditary hemorrhagic telangiectasia (HHT) is characterized by telangiectasias in multiple organs. We experienced an infant with cerebral hemorrhaging and suspected HHT based on his family history of HHT. Computed tomography angiography confirmed a cerebral arteriovenous fistula. The onset of cerebral arteriovenous fistulas associated with HHT is relatively early, and the incidence of bleeding is relatively common. When HHT is suspected based on a family history, early imaging screening is recommended to improve the neurological prognosis, even in asymptomatic cases. (Received April 7, 2020; Accepted May 7, 2020; Published August 1, 2020).

Clinical evaluation of the arched blade for cataract surgery.

PURPOSE: To evaluate the efficacy of the arched blade for making clear corneal incisions in cataract surgery. METHODS: This prospective study comprised 112 eyes of 74 patients scheduled for cataract surgery. Temporal clear corneal incisions were made with either a 3.2-mm conventional flat blade or the arched blade. The choice of knife was randomly assigned. Two surgeons, one with substantial cataract surgery experience and the other with less experience, performed the surgery. Corneal topography and aberration were examined pre- and postoperatively. The degree of surgically induced astigmatism (SIA) and high order aberration was analysed. The self-sealing ability of the wound was also compared between both blades. RESULTS: For the less experienced surgeon, the degree of SIA was significantly higher with the 3.2-mm flat blade than with the 3.2-mm arched blade as measured at any time during postoperative follow-up. For the more experienced surgeon, the degree of high order aberration increased significantly with the 3.2-mm flat blade. The incision's self-sealing ability was significantly better when the wound was made with the arched blade rather than with the flat blade. CONCLUSION: The arched blade proved to be effective in reducing surgically induced astigmatism and high order aberration in cataract surgery, particularly when used by the less experienced surgeon. Using the arched blade should lead to better wound self-sealing and, therefore, safer surgical results.