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      Transient Homonymous Superior Quadrantanopsia in Nonketotic Hyperglycemia: A Case Report and Systematic Review

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          Abstract

          Background and Purpose

          Nonketotic hyperglycemia often causes transient visual field defects, but only scattered anecdotes are available in the literature.

          Methods

          We report a patient with homonymous superior quadrantanopsia due to nonketotic hyperglycemia and provide a systematic literature review of the clinical features of 40 previously reported patients (41 in total, including our case) with homonymous visual field defects in association with nonketotic hyperglycemia.

          Results

          The typical visual field defect was congruous (84.6%), homonymous hemianopsia (87.8%) with macular splitting (61.5%) or sparing (38.5%). It was transient and repetitive in 54.5% of the patients, but it developed as a persistent form in the remainder. Positive visual symptoms such as hallucinations and phosphenes developed in 73.2% of patients. Brain MRI revealed corresponding abnormalities in most patients (84.8%), characterized by a low-intensity white-matter signal or a high-intensity gray-matter signal on T2-weighted or fluid-attenuated inversion recovery images with diffusion restriction or gadolinium enhancement. Most (97.0%) patients recovered completely, with 48.5% treated by glycemic control alone and the remainder also receiving antiepileptic agents.

          Conclusions

          Nonketotic hyperglycemia should be considered a possible cause of transient visual field defects, especially when it is associated with repetitive positive visual symptoms and typical MRI findings in hyperglycemic patients.

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          Most cited references24

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          Chorea associated with non-ketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI study: a meta-analysis of 53 cases including four present cases.

          Chorea associated with non-ketotic hyperglycemia and high signal intensity lesions on T1-weighted brain magnetic resonance images (C-H-BG) is recognized as a unique syndrome that affects elderly women exclusively. However, its overall clinical features are unclear. The literature describing patients with C-H-BG from 1985 to 2001 was reviewed using MEDLINE. Their clinical features and those of four patients with C-H-BG at this hospital were analyzed. This study included 49 patients from the literature and four patients at this hospital. Their mean age at the onset was 71.1 years (range=22-92 years). Women were affected more frequently than men (men/women=17:30). The mean serum glucose level measured after the onset of chorea was 481.5 mg/dl (ranging from 169 to 1264), HbA1c level was 14.4% (ranging from 9.9 to 19.2), and the serum osmolarity was 305.9 mmol/kg (ranging from 291 to 335). Forty-seven patients developed hemichorea. Six patients developed bilateral chorea, and magnetic resonance imaging (MRI) showed bilateral basal ganglia lesions. MRI showed that putamen was involved in all cases (isolated putamen=31 patients, additional basal ganglia lesions=22 patients). None had lesions confined to the caudate nucleus or the globus pallidus. In all, except one, the anterior limb of the internal capsule was spared. Follow-up MRI studies were performed in 22 patients. In most, hemichorea improved along with the disappearance of the lesions. In 39 patients, chorea had ameliorated completely. The remaining 14 cases showed some improvement during the follow-up period. The chorea recurred in seven patients. C-H-BG is a benign disorder affecting the elderly. It affects men much more frequently than has been reported. The high signal intensity basal ganglia lesion on the T1-weighted brain MRI study was reversible, and correlated with the clinical improvement in chorea.
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            Homonymous hemianopias: clinical-anatomic correlations in 904 cases.

            To describe the clinical characteristics and clinical-anatomic correlations of homonymous hemianopia (HH). Homonymous hemianopia impairs visual function and frequently precludes driving. Most knowledge of HH is based on relatively few cases with clinical-anatomic correlations. The authors reviewed medical records of all patients with HH seen in their service between 1989 and 2004. Demographic characteristics, characteristics of visual field defects, causes of visual field defects, neuroradiologic definition of lesion location, and associated neurologic deficits were recorded. A total of 904 HH were found in 852 patients. A total of 340 HH (37.6%) were complete and 564 HH (62.4%) were incomplete. Homonymous quadrantanopia (264 HH, 29%) was the most common type of incomplete HH, followed by homonymous scotomatous defects (116 HH, 13.5%), partial HH (114 HH, 13%), and HH with macular sparing (66 HH, 7%). A total of 407 HH (45.0%) were isolated. Causes of HH included stroke (629 HH, 69.6%), trauma (123, 13.6%), tumor (102, 11.3%), brain surgery (22, 2.4%), demyelination (13, 1.4%), other rare causes (13, 1.4%), and unknown etiology (2, 0.2%). The lesions were most commonly located in the occipital lobes (45%) and the optic radiations (32.2%). Every type of HH, except for unilateral loss of temporal crescent and homonymous sectoranopia, was found in all lesion locations along the retrochiasmal visual pathways. Homonymous hemianopia is usually secondary to stroke, head trauma, and tumors. Although the characteristics of visual field defects can be helpful in lesion location, specific visual field defects do not always indicate specific brain locations.
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              Valproate-Induced Insulin Resistance and Obesity in Children

              Background: Valproic acid (VPA), a widely used antiepileptic drug, has broad-spectrum activity against both generalized and partial epilepsy. Among the side effects of VPA, weight gain is frequently reported, although the real incidence and magnitude of this problem is unknown. Its pathogenesis is most likely multifactorial, and is controversial. Methods: In order to evaluate the role of hyperinsulinemia and related hormonal abnormalities in VPA-induced obesity, data from the existing literature have been analyzed and discussed critically. Results: Patients suffering from weight gain show various metabolic and endocrinologic abnormalities. The most frequent are hyperinsulinemia and insulin resistance, hyperleptinemia and leptin resistance, and an increase in the availability of long-chain free fatty acids. Significant weight gain is associated with increased levels of insulin and leptin, suggesting a close relationship between obesity-induced hyperinsulinemia and hyperleptinemia. VPA can directly stimulate pancreatic β-cells and indirectly enhance insulin resistance by suppressing insulin-mediated peripheral glucose uptake. Leptin activation seems to be similar in obese VPA-treated subjects to that seen in otherwise obese subjects. Conclusions: The mechanisms of hyperinsulinemia in VPA-induced weight gain remain unclear, although it is likely that obesity is the cause of hyperinsulinemia and all related metabolic changes. However, this heterogeneous metabolic disorder requires further research.
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                Author and article information

                Journal
                J Clin Neurol
                J Clin Neurol
                JCN
                Journal of Clinical Neurology (Seoul, Korea)
                Korean Neurological Association
                1738-6586
                2005-5013
                October 2020
                09 September 2020
                : 16
                : 4
                : 599-604
                Affiliations
                [a ]Department of Neurology, Korea University Medical Center, Seoul, Korea.
                [b ]Department of Neurology, Seoul National University College of Medicine, Seoul, Korea.
                [c ]Department of Neurology, Seoul National University Bundang Hospital, Seongnam, Korea.
                [d ]Research Administration Team, Seoul National University Bundang Hospital, Seongnam, Korea.
                Author notes
                Correspondence: Jeong-Yoon Choi, MD, PhD. Department of Neurology, Seoul National University Bundang Hospital, 82 Gumi-ro 173beon-gil, Bundang-gu, Seongnam 13620, Korea. Tel +82-31-787-7463, Fax +82-31-719-6828, saideiju@ 123456gmail.com
                Author information
                https://orcid.org/0000-0001-8660-7759
                https://orcid.org/0000-0002-3544-1098
                https://orcid.org/0000-0003-2203-2698
                https://orcid.org/0000-0002-2027-6341
                https://orcid.org/0000-0003-2159-9967
                https://orcid.org/0000-0003-2204-8067
                https://orcid.org/0000-0002-1508-2024
                Article
                10.3988/jcn.2020.16.4.599
                7541982
                33029966
                6658fb76-66bb-49af-afae-f0e67bcc41f3
                Copyright © 2020 Korean Neurological Association

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 10 March 2020
                : 28 May 2020
                : 29 May 2020
                Funding
                Funded by: National Research Foundation of Korea, CrossRef https://doi.org/10.13039/501100003725;
                Award ID: 2020R1A2C4002281
                Categories
                Original Article

                Neurology
                visual fields,quadrantanopsia,hyperglycemia,diabetes mellitus
                Neurology
                visual fields, quadrantanopsia, hyperglycemia, diabetes mellitus

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