Three-Dimensional Printed Brain Model of a Patient With Alexia Without Agraphia Syndrome

A 64-year-old woman presented with acute painless bilateral vision loss. She had well-controlled psoriatic arthritis, but the remainder of her past ocular, surgical, and family histories were noncontributory.

Photo and Video Essay Section Editors: Kimberly M. Winges, MD Michael J. Gilhooley, MA, MB, BChir, DPhil Three-Dimensional Printed Brain Model of a Patient With Alexia Without Agraphia Syndrome Alexis R. Pascoe, MD, Subahari Raviskanthan, MBBS, Peter W. Mortensen, MD, Andrew G. Lee, MD FIG. 1. A. Automated perimetry (visual fields 24-2) showing a complete right homonymous macular splitting hemianopsia. B. Optical coherence tomography of the retinal nerve fiber layer shows hemiband atrophy (primarily temporal fiber loss) of the right eye and hourglass atrophy (both superior and inferior fiber loss) of the left eye. Department of Ophthalmology (ARP, AGL), University of Texas Medical Branch, Galveston, Texas; Department of Ophthalmology (SR, PWM, AGL), Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas; Departments of Ophthalmology, Neurology, and Neurosurgery (AGL), Weill Cornell Medicine, New York, New York; University of Texas MD Anderson Cancer Center (AGL), Houston, Texas; Texas A and M College of Medicine (AGL), Bryan, Texas; and Department of Ophthalmology (AGL), The University of Iowa Hospitals and Clinics, Iowa City, Iowa. The authors report no conflicts of interest. Address correspondence to Andrew G. Lee, MD, Department of Ophthalmology, Blanton Eye Institute, Houston Methodist Hospital, 6560 Fannin Street, Suite 450, Houston, TX 77030; E-mail: aglee@ houstonmethodist.org Pascoe et al: J Neuro-Ophthalmol 2023; 43: e55-e57 CASE REPORT A 64-year-old woman presented with acute painless bilateral vision loss. She had well-controlled psoriatic arthritis, but the remainder of her past ocular, surgical, and family histories were noncontributory. Her medications included citalopram, etanercept, and apremilast. She had previously been on adalimumab for her psoriatic arthritis. Her symptoms started 2 days after commencing apremilast injections as disease modifying antirheumatic drug (DMARD). She initially presented with acute severe headache and altered mental status, with subsequent loss of consciousness. e55 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay A computerized tomography (CT) of the head showed a large left temporoparieto-occipital hematoma with mass effect. A CT venogram showed a left transverse sinus thrombosis. She underwent an emergent decompressive hemicraniectomy for the hematoma. Two months into her recovery, a deep venous thrombosis occurred and was started on the appropriate anticoagulation. A hypercoagulable workup showed positive serum antiphospholipid antibodies. After her stroke, the patient complained that she could write but she could not read despite preserved central visual acuity in both eyes. On neuro-ophthalmic examination, the visual acuity was 20/30 in the right eye and 20/25 in the left eye. Her pupils were isocoric. The extraocular motility, intraocular pressure measurements, and slit-lamp biomicroscopy were unremarkable. Her National Institute of Health stroke scale score was 3 with a right homonymous hemianopsia and mild aphasia. Her speech was fluent without dysarthria, and naming and repetition were intact. She was unable to read, but she could recognize the individual letters on the Snellen chart. Automated perimetry (visual fields 24-2) revealed a right, complete, macular splitting, homonymous hemianopsia. Ophthalmoscopy and optical coherence tomography of the retinal nerve fiber layer showed hemiband-like atrophy in the right eye and an “hourglass” pattern of atrophy in the left eye (Fig. 1) consistent with a homonymous hemianopsia on perimetry. MRI of the brain revealed left temporal, parietal, and occipital chronic encephalomalacia and gliosis (Fig. 2A). The patient worked in a nearby research facility and created a 3dimensional (3D)-printed figure of her brain (Fig. 2B), which demonstrated the encephalomalacia in the left occipital cortex. DISCUSSION This patient had the classic occipital lobe disconnection syndrome, alexia without agraphia (AWA). AWA is due to the FIG. 2. A. MRI of the brain showing temporo-occipital encephalomalacia. B. Three-dimensional printed brain model showing the area of encephalomalacia matching the clinical syndrome of right homonymous hemianopsia and alexia without agraphia. e56 disruption of the connections between the different languages areas in the brain organized around the lateral tissue. These areas include the Broca area for expressing language and the Wernicke area for auditory language comprehension. The areas important for naming, reading, writing, and calculation are the angular gyrus and the supramarginal gyrus. Normally, visual information travels from the right occipital lobe via the splenium of the corpus callosum and directly from the left occipital lobe to the left angular gyrus for reading. If there is damage to the splenium, the information cannot be transmitted from the intact right occipital cortex, leading to AWA. The etiology for AWA is typically due to a left posterior cerebral artery (PCA) occlusion, leading to infarction of the left visual cortex and the left splenium. Other etiologies for the syndrome include acute encephalopathy, multiple sclerosis, migraine, surgery for a left occipital lobe vascular malformation, epileptic focus, and occipital tumors (e.g., glioblastoma) and occipital hematomas (1–5). The role of the DMARDs in her presentation is ill defined; however, patients with psoriasis are known to have an increased risk of venous thromboembolism. The hazard ratio was 1.35 for venous thromboembolism in patients with RA on DMARD (6). In addition to the increased risk due to DMARDs, this patient also had antiphospholipid syndrome, which has also been linked to etanercept (Enbrel) use (7). Three-dimensional printing has been increasingly used in medicine, with many roles including individualized preoperative planning, personalized medical treatment devices, and rehabilitation management devices. For example, in stroke management, orthotic devices have been printed to assist in rehabilitation of post stroke spasticity (8). Also 3D printed surgical simulators have been gaining increased prevalence, with the ability to individualize the simulator to each patient’s pathology, although finer microdissection techniques have not been reported, possibly related to the detail currently obtainable in 3D printing (9). One report noted a decrease in operative time of 30 minutes in surgeons who used 3D printing and simulators before performed cerebrovascular and endovascular surgeries on patients (10). Another review with 32 neurosurgeons reported that they would likely change the craniotomy size and location if using a 3D printed model of the patient’s pathology, rather than currently available MRI of the brain—those who were less experienced in either training or the number of cases they had seen were more likely to change their operative plan based on the 3D printed model (11). The role of 3D printing has also been noted to be valuable for patient education in small groups studied, including a population of congenital heart disease patients and their parents, in whom both the parents, and the treating cardiologists noted improved communication with a 3D printed model compared with a standard visit, although subjective/objective evidence of improved understanding of the patient’s disease was not noted (12). Pascoe et al: J Neuro-Ophthalmol 2023; 43: e55-e57 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay This case is interesting and unique and demonstrates the use of a 3D printed brain model of AWA. To our knowledge, this is the first such report in the Englishlanguage ophthalmic literature. STATEMENT OF AUTHORSHIP Conception and design: A. R. Pascoe, S. Raviskanthan, P. W. Mortensen, A. G. Lee; Acquisition of data: A. R. Pascoe, S. Raviskanthan, P. W. Mortensen, A. G. Lee; Analysis and interpretation of data: A. R. Pascoe, S. Raviskanthan, P. W. Mortensen, A. G. Lee; Drafting the manuscript: A. R. Pascoe, S. Raviskanthan, P. W. Mortensen, A. G. Lee; Revising the manuscript for intellectual content: A. R. Pascoe, S. Raviskanthan, P. W. Mortensen, A. G. Lee; Final approval of the completed manuscript: A. R. Pascoe, S. Raviskanthan, P. W. Mortensen, A. G. Lee. REFERENCES 1. Caffarra P. Alexia without agraphia or hemianopia. Eur Neurol. 1987;27:65–71. 2. Marti-Massó J, Astudillo A, Obeso J, Carrera N, de la Puente E. 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