Right Homonymous Hemianopia: A Clinical Case Report of Schizencephaly

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Abstract

Purpose: To report a case of a 56-year-old male with right homonymous hemianopia. Methods: Retrospective descriptive study of a case report based on information from clinical records, patient observation and analysis of complementary diagnostic tests. Results: An asymptomatic 56-year-old male presented to our hospital for a routine ophthalmic examination. The best-corrected visual acuity was 20/20 in the right eye (RE) and in the left eye (LE). Pupillary function, intraocular pressure, external segment examinations and slit-lamp biomicroscopy were normal, bilaterally. Fundoscopy showed a cup-to-disc (C/D) ratio in the RE of 0.3 and of 0.4 in the LE. Retinal nerve fiber layer (RNFL) thickness measured by spectral domain optical coherence tomography revealed thinning of the superior, temporal and nasal RNFL in the RE and thinning of the superior, inferior and temporal RNFL in the LE. Automated static perimetry showed right homonymous hemianopia. Brain computed tomography (CT) showed an open-lip schizencephaly with a significant reduction of the left brain parenchyma. Conclusions: Despite the large visual defect, the patient was unaware of it and had an active professional life. This is an interesting case because despite the extensive morphological abnormalities seen on brain CT there is a relatively small functional repercussion.

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Most cited references 9

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Schizencephalies; a study of the congenital clefts in the cerebral mantle; clefts with fused lips.

R Wadsworth, P I Yakovlev (1946)

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Schizencephaly—diagnostics and clinical dilemmas

Agata Halabuda, Lukasz Klasa, Stanisław Kwiatkowski … (2015)

Background Schizencephaly is an uncommon congenital disorder of cerebral cortical development. The defect is characterized by the presence of a cleft in the brain extending from the surface of the pia mater to the cerebral ventricles. The margins of the cleft are lined with heterotropic, dysplastic gray matter. The causes of schizencephaly are heterogeneous and can include teratogens, prenatal infection, maternal trauma, or EMX2 mutations. Method In the present paper, the authors described difficulties in employing diagnostic imaging in differentiating between type II (open-lip) schizencephaly and much more common intracranial fluid spaces of a different origin (arachnoid cysts and hydrocephalus). Result In all the three cases, the treatment consisted in implantation of a shunt system; nevertheless, it should be emphasized that a surgical intervention in the third presented case (type II schizencephaly) aimed at relieving the symptoms of intracranial hypertension—a directly life-threatening condition—since shunting is not a method of treating schizencephaly itself. Conclusions Although proper interpretation of the character of intracranial fluid spaces is of significance for further therapeutic management, yet, the key decision as to the surgical intervention is made based on clinical presentation, predominantly on symptoms of intracranial hypertension.

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Schizencephaly: clinical and imaging features in 30 infantile cases.

D Lacombe, D Fontan, V Flurin … (2000)

Schizencephaly is an uncommon structural disorder of cerebral cortical development, characterized by congenital clefts spanning the cerebral hemispheres from the pial surface to the lateral ventricles and lined by cortical gray matter. Either an antenatal environmental incident or a genetic origin could be responsible for this lesion which occurs between the third and fourth month of gestation. We report the clinical and cranial imaging features of 30 children, of whom 15 had unilateral and 15 had bilateral lesions. Their ages at the time of the first presentation ranged from 1 month to 10 years. They were thoroughly studied from clinical, epileptical, imaging and electroencephalographic (EEG) viewpoints. Five patients were investigated by cranial computed tomography (CT), eight by cranial magnetic resonance (MR) imaging, and 17 by both methods. The clinical features consisted of mild hemiparesis in 17 cases (57%), 12/17 were related to a unilateral phenotype (80% of all unilateral forms) and 5/17 to a bilateral phenotype. A tetraparesis was present in nine cases, all of which were due to a bilateral cleft. Bilateral forms were significantly associated with tetraparesis, whereas unilateral forms were associated with hemiparesis. Mental retardation was observed in 17 cases (57%), and was observed significantly more often in bilateral clefts (80%). When both hemispheres are involved, an absence of reorganization of the brain function between the two hemispheres leads to severe mental deficits, in addition to the cerebral anomaly itself. Eleven patients had seizures (seven from unilateral and three from bilateral forms). The degree of malformation was not related to the severity of epilepsy. Migration disorders, such as dysplasia or heterotopia, were observed in 30% of cases and are also important etiopathogenetic factors. The septum pellucidum was absent in 13 cases (43%), with septo-optical dysplasia in two cases. Corpus callosum dysgenesis was noted in 30% of cases. Four cases of mega cisterna magna were noted. Although familial cases and environmental factors have been previously reported, schizencephaly appears to be, in the majority of cases, sporadic.

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Journal

Journal ID (publisher-id): COP

Journal ID (pmc): COP

Journal ID (doi): 10.1159/issn.1663-2699

Title: Case Reports in Ophthalmology

Publisher: S. Karger AG

ISSN (Electronic): 1663-2699

Publication date Subscription-year: 2016

Publication date Collection: January – April 2016

Publication date (Electronic): 08 January 2016

Volume: 7

Issue: 1

Pages: 16-20

Affiliations

Ophthalmology Department, Leiria Hospital Center, Leiria, Portugal

Author notes

*Arminda Neves, Ophthalmology Department, Leiria Hospital Center, Rua das Olhalvas, Pousos, PT-2410-197 Leiria (Portugal), E-Mail armindaneves@hotmail.com

Article

Publisher ID: 443323 Pmcid ID: PMC4748790 Other ID: Case Rep Ophthalmol 2016;7:16-20

DOI: 10.1159/000443323

PMC ID: PMC4748790

PubMed ID: 26889154

SO-VID: e755b592-8bf9-4ba8-a981-ca8e30924d11

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This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC). Usage and distribution for commercial purposes requires written permission. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

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Right Homonymous Hemianopia: A Clinical Case Report of Schizencephaly

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Abstract

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Karger: Ophthalmology

Most cited references 9

Schizencephalies; a study of the congenital clefts in the cerebral mantle; clefts with fused lips.

Schizencephaly—diagnostics and clinical dilemmas

Schizencephaly: clinical and imaging features in 30 infantile cases.

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