An Elderly Woman With Difficulty Reading and Abnormal Eye Movements Virginie Desestret, MD, PhD, Nathalie Streichenberger, MD, PhD, Muriel Panouillères, Denis Pélisson, PhD, B. Plus, MD, Charles Duyckaerts, MD, PhD, Dennis K. Burns, MD, Christian Scheiber, MD, PhD, Alain Vighetto, MD, Caroline Tilikete, MD, PhD Dr Tilikete A 73-year-old woman was evaluated in our neuro- ophthalmology clinic with a 1-year history of progressive difficulty reading. The patient’s visual acuity, pupillary reactions to light and near stimulation, visual fields, and fundi were normal. Examination of her eye movements revealed a supranuclear vertical gaze abnormality, charac- terized by lack of upward saccades but intact downward saccades. The patient also had had difficulty initiating voluntary, especially leftward horizontal saccades on command, but reactive horizontal saccades were relatively well preserved. She was able to follow a pencil light moved by the examiner using small saccades (saccadic smooth pursuit) and her vestibulo-ocular reflex (VOR) was intact. She had apraxia of lid closure. The patient had no cognitive deficit, behavioral or social disturbance, aphasia, alexia, limb apraxia, postural ataxia, pyramidal signs or parkinsonism. Neuropsychological testing was hindered by reading difficulties but disclosed mild attentional and executive deficits, with verbal memory and language conserved (Table 1). Sac- cades were recorded (500 Hz; EyeLink II eye-tracker; SR Research, Mississauga, Canada) during a paradigm of reactive saccades toward 8 degrees right or left and a paradigm of voluntary scanning saccades during simultaneous presentation of three targets (28, 0, and +8 degrees). VOR during pendular chair stimulation (maximum velocity: 40 degrees per second, frequency: 0.25 Hz) and smooth pursuit (target amplitude: 30 degrees, frequency: 0.15 Hz) were recorded using 25-Hz infra- red video-oculography (VNG Ulmer; Synapsys, Marseille, France). The VOR was normal with preservation of the rightward and leftward quick phases (reflexive saccades) (Fig. 1A). Smooth pursuit showed saccadic following of the target (Fig. 1B). Reactive saccades to the right (Fig. 1C; Table 2) and left (Fig. 1D; Table 2) had normal latency and ampli- tude. Voluntary scanning saccades presented abnormal latency, specifically to the left (Fig. 1E, F; Table 2). Neuro- imaging included magnetic resonance imaging (MRI) of the brain, a SPECT scan, and a DaTscan. This last study is a SPECT scan for striatal dopamine transporter visualization. Dr Scheiber The brain MRI shows only frontal cortical atrophy without brainstem atrophy (Fig. 2A, B), and the standard SPECT study demonstrates bilateral frontal and left parietal hypo- perfusion (Fig. 2C, D). The DaTscan shows bilateral ni- grostriatal dopamine transporter loss (Fig. 2E). Dr Tilikete The patient was diagnosed with an acquired ocular motor apraxia and supranuclear vertical ophthalmoplegia, presumably from some type of degenerative process. There were no other signs of Parkinson disease or progressive supranuclear palsy (PSP). The differential diagnosis included fronto-temporal lobar degeneration (FTLD) and cortico-basal degeneration. Hospices Civils de Lyon, Service d’Anatomo-pathologie (VD, NS), Groupement Hospitalier Est, Bron, France; Hospices Civils de Lyon, Unité de Neuro-ophtalmologie and Service de Neurologie D (VD, AV, CT), Hôpital Neurologique, Bron, France; Université Claude Bernard Lyon I (NS, MP, DP, CS, AV, CT), Lyon, France; INSERM U1028 and CNRS UMR5292 (MP, DP, AV, CT), Lyon Neuroscience Research Center, IMPACT team, Lyon, France; Hospices Civils de Lyon, Service d’Explorations Fonctionnelles Neurologiques (BP), Hôpital de la Croix-Rousse, Lyon, France; Laboratoire de Neuro- pathologie R. Escourolle (VD, CD), La Salpêtrière Hospital, Paris, France; Department of Pathology (DKB), University of Texas Southwestern Medical Center, Dallas, Texas; Hospices Civils de Lyon, Service de Médecine Nucléaire (CS), Groupement Hospitalier Est, Bron France; and Centre de Neurosciences Cognitives (CS), UMR 5229—UCB Lyon 1 Prise de décision et Neuroéconomie Team, Lyon, France. Supported by the Hospices Civils de Lyon (HCL/P 2002.303). The authors report no conflicts of interest. Address correspondence to Caroline Tilikete, MD, PhD, Hospices Civils de Lyon, Unité de Neuro-ophtalmologie, Hopital Neuro- logique, 59 Boulevard Pinel, 69677 Bron Cedex, France; E-mail: caroline.tilikete@inserm.fr 296 Desestret et al: J Neuro-Ophthalmol 2013; 33: 296-301 Clinical-Pathological Case Study Section Editor: Neil R. Miller, MD Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.