RESIDENT & FELLOW SECTION Clinical Reasoning: — Section Editor Two see or not two see Is it really double John J. Millichap, MD vision?

Richard Ronan Murphy, SECTION 1 predominately in the horizontal plane, in all direc- MBChB A 57-year-old right-handed woman presented to the tions of gaze. The diplopia persisted with monocular Abdullah Al Sawaf, MD emergency department with complaints of double vision in each eye, and did not improve with a pinhole Danny R. Rose Jr., MD vision and intractable nausea that began abruptly 2 test. The degree of diplopia waxed and waned during Larry B. Goldstein, MD days earlier. Her visual symptoms were characterized the examination, with visual field extinction tests Charles D. Smith, MD as seeing overlapping or separate horizontally or diag- being difficult to perform reliably. Her pupils were onally displaced objects. She had no history of head- equal with bilateral hippus. Visual fields were full to aches or stroke. Her cerebrovascular risk factors confrontation. Direct funduscopy revealed normal Correspondence to included hypertension, type II diabetes, coronary optic discs. She had a mild right hemiparesis with R.R. Murphy: artery disease, and cigarette smoking. Her medica- [email protected] mild right arm and leg drift, but no facial asymmetry. tions included clopidogrel, lisinopril, paroxetine, There was mild hypesthesia over her right arm and leg and oxycodone. Her family history was notable for and appendicular ataxia in her right arm that was late-onset ischemic heart disease in her parents with worse with eyes open. She did not have extinction no first-degree relatives with early vascular disease. to double simultaneous sensory stimuli. Gait evalua- On examination, her blood pressure was 158/ tion was deferred during her initial examination. 101 mm Hg, pulse rate was 87 bpm, and she was afe- Questions for consideration: brile. She was alert and fully oriented. Her attention, recall of recent events, and general fund of knowledge 1. What is the significance of the presence of diplopia were normal. Her speech was fluent and nondysarth- in both eyes, with either eye closed? ric. Cranial nerve examination was notable for no dys- 2. What more could be elicited from the history and conjugacy or nystagmus, but double vision examination to help characterize the problem?

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From the Department of Neurology, University of Kentucky, Lexington. Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article. e56 © 2017 American Academy of Neurology ª 2017 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. SECTION 2 Additional examination findings. The patient was unable The patient had the abrupt onset of bilateral monoc- to describe events in the Cookie Thief image, used in ular diplopia. Monocular diplopia affecting one eye the NIH Stroke Scale, or identify complex overlapping is commonly related to an intraocular process such figures. She was unable to recognize pictures of famous as a refractive error, cataract, or a macular disorder. faces or well-known landmarks; however, she was able to Bilateral monocular diplopia due to a refractive recognize family members and hospital staff. The cause is unusual because simultaneous acute ocular patient could read text and identify solid colors pre- pathology in both eyes is unlikely. Perception of sented on a tablet computer, but had difficulty reading sight, including the subjective experience of seeing numbers on Ishihara color plates. double, involves multifaceted higher-order cognitive The patient later elaborated that the double vision processing. Perplexing visual symptoms such as the involved persisting images or trails left by objects, overlapping images that this patient reported, com- more so in her left visual field. Most of these symp- bined with her motor and sensory abnormalities, toms improved over hours and largely resolved by raised concern for a central process. The abrupt the following day. onset of her symptoms and her risk factor profile Question for consideration: suggests a cerebrovascular cause. A more detailed examination of cortical visual perception was then 1. How may the patient’s visual deficits be concisely performed. defined or labeled?

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Neurology 89 August 8, 2017 e57 ª 2017 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. SECTION 3 recognizing people in person when richer perceptual Palinopsia is defined as an abnormal perception of cues were present, suggests an associative visual visual stimuli in the visual field after stimulus agnosia. removal, but sometimes experienced as multiple im- Simultanagnosia is a type of apperceptive visual ages or trails as an object is moved in the visual field. agnosia, commonly defined as the inability to per- These phenomena are termed visual trailing or as ceive multiple objects at the same time, typically asso- cerebral diplopia or polyopia when a patient reports ciated with parieto-occipital lesions. It may be more seeing 2 or more duplicated images arranged in accurately defined as the inability to perceive and ordered rows or columns after fixation on an object.1 decode the scene as a whole whereas the ability to This can be due to ocular disease, but tends to localize identify discrete elements is not impaired, i.e., “seeing to lesions involving the primary visual cortex, optic the forest but not the trees,”6 or also perhaps a tree radiations (more commonly right-sided), or optic but not the forest. tract. It also can be caused by a range of conditions Simultanagnosia was identified when the patient such as toxicity from medications or illicit drugs, seiz- could not identify overlapping objects in pictures or ures, migraine, or traumatic brain injury.1–4 In this decode complex visual scenes, whereas she had no dif- patient, additional history and examination con- ficulty naming objects presented in simple pictures. firmed the visual complaint was consistent with Simultanagnosia that occurs with impaired reach- palinopsia. ing or grasping under visual guidance (optic ataxia) Visual agnosia encompasses impairment in per- and impaired gaze fixation (oculomotor apraxia) ception (apperceptive agnosia) and recognition (asso- define Bálint syndrome. This syndrome tends to ciative agnosia) of visually presented objects, not due localize to the occipito-parietal junction, involving to a deficit in vision. Prosopagnosia is a subtype of dorsal, spatial visual associative pathways, sparing associative visual agnosia with impaired recognition the ventral, semantic connections.7 Bálint syndrome of familiar faces that tends to localize to lesions of may occur with bilateral middle cerebral artery/pos- the right fusiform gyrus.5 This patient had difficulty terior cerebral artery watershed infarcts. Although this recognizing pictures of famous landmarks and faces. patient had transient right upper limb ataxia that was She had no apparent visual field deficit or extinction worse with her eyes open, there was neither convinc- to double simultaneous stimuli. She had no trouble ing optic ataxia nor oculomotor apraxia. naming common objects or recognizing family or Question for consideration: staff. Her difficulty recognizing pictures of faces and famous places, but not with other visual stimuli, or 1. To what areas do the patient’s symptoms localize?

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e58 Neurology 89 August 8, 2017 ª 2017 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. SECTION 4 This is a paradigm shift in the behavioral examination, A CT scan of the brain revealed only an area of hypo- elements of which may now be rapidly performed at intensity in the right cerebellar hemisphere. CT the point of care. angiogram showed marked atherosclerotic disease in In this case, an MRI brain scan confirmed an etio- the vertebrobasilar system, but no arterial occlusion. logic diagnosis of stroke with ischemic lesions involving A diffusion-weighted MRI was obtained because of portions of the inferior temporal and occipital lobes concern for a parietal or parieto-occipital cortical bilaterally, including small infarcts adjacent to the pri- stroke (figure). This showed scattered lesions, consis- mary visual cortex (lesions 2 and 4), as well as infarcts tent with multiple emboli to the bilateral posterior in the brainstem and cerebellum. Palinopsia without cerebral artery (PCA) territories, involving both pri- visual field defects can occur with lesions in the posterior mary and visual associative areas. Small pontine per- visual pathways.4 Interestingly, visual hallucinations forator and right superior cerebellar artery (SCA) have been described with brainstem lesions, and this distribution strokes were also present. patient also had a small pontine infarction. The phe- nomena of Lhermitte peduncular hallucinosis are typi- DISCUSSION This case demonstrates the clinical chal- cally described as well-formed, bright, colorful shapes, lenge of localizing a subjective complaint of double patterns, or images.8 Palinopsia, as occurred in this vision, which may take many forms and can localize patient, does not fit well with the previous descriptions to the eyes, oculomotor systems, visual pathways, and of peduncular hallucinosis, nor would her visual agnosia as in this patient, central structures involved in conscious or simultanagnosia. The brainstem and cerebellar lesions visual perceptual processes. A careful history and exam- can, however, account for her mild right-sided sensory ination was key. Bilateral monocular diplopia or any abnormalities, right hemiparesis, and right arm ataxia other unusual visual symptoms should lead to testing (lesions 10–12). of higher order visual function, which may be quickly Although the patient’s symptoms were present for and easily performed during the initial evaluation. Por- several days at the time of her initial presentation and table tablet computers have removed the inconvenience she was not a candidate for acute intervention, a high of performing such examinations in clinical settings. index of suspicion, including querying for visual or

Figure Diffusion-weighted MRI 2 days after onset of symptoms

(A–F) Locations established by coregistration with the ICBM atlas: 1, right cerebellar vermis; 2, left inferior occipital gyrus; 3, right cerebellar vermis; 4, left (and right) inferior occipital gyrus; 5, junction left inferior temporal gyrus/fusiform gyrus; 6, right dorsomedial nucleus thalamus; 7, right precuneus; 8, left cuneus; 9, right cerebellum lobule VIII; 10, paramedian anterior pons; 11, right cerebellar crus I; 12, right cerebellar lobule VI.

Neurology 89 August 8, 2017 e59 ª 2017 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. ataxic symptoms, is needed to improve the chance of STUDY FUNDING rapid clinical diagnosis.9 Seizures, migraine, trauma, No targeted funding reported. and hallucinogenic drugs or toxins need to be consid- DISCLOSURE ered in the differential diagnosis. A primary psychiat- The authors report no disclosures relevant to the manuscript. Go to ric cause should not be diagnosed without a thorough Neurology.org for full disclosures. evaluation for a structural abnormality. Approaches to identifying this problem include having the patient REFERENCES describe the Cookie Thief picture. Overlapping fig- 1. Gersztenkorn D, Lee AG. Palinopsia revamped: a system- – ures is a useful bedside screen for visual perception atic review of the literature. Surv Ophthalmol 2015;60:1 35. problems in a multitude of clinical settings. Figures 2. Pomeranz HD, Lessell S. Palinopsia and polyopia in the can be obtained from the article by Giannakopoulos absence of drugs or cerebral disease. Neurology 2000;54: 10 et al., and are an excellent addition to examination 855–859. aids carried by the neurologist. 3. Abert B, Ilsen PF. Palinopsia. Optometry 2010;81:394– Anecdotally, the famous faces images we have long 404. carried (e.g., George Bush, Princess Diana) were famous 4. Ritsema ME, Murphy MA. Palinopsia from posterior visual pathway lesions without visual field defects. during their era, but can mystify younger patients. To J Neuro-ophthalmol 2007;27:115–117. keep pace with the fading of fame, a clinically useful, 5. Schultz C, Sorger B, Caldara R, et al. Impaired face dis- if not validated, set of currently famous (or infamous) crimination in acquired prosopagnosia is associated with faces and places can be obtained on the Internet. Use abnormal response to individual faces in the right middle of electronic media also allows easy portability and rapid fusiform gyrus. Cereb Cortex 2006;16:574–586. access to specialized assessments such as the overlapping 6. Thomas C, Kveraga K, Huberle E, Karnath HO, Bar M. figures, the NIH Stroke Scale, and other measures that Enabling global processing in simultanagnosia by psycho- physical biasing of visual pathways. Brain 2012;135:1578– can be useful in specific acute settings. 1585. This patient illustrates how commonly available 7. Rizzo M, Vecera SP. Psychoanatomical substrates of Ba- technology (the computer tablet) can aid clinical diag- lint’s syndrome. J Neurol Neurosurg Psychiatry 2002;72: nosis, and together with modern imaging, lead to 162–178. a better understanding of the potential causes of tran- 8. Cummings JL, Miller BL. Visual hallucinations: clinical sient clinical symptoms and findings. occurrence and use in differential diagnosis. West J Med 1987;146:46–51. 9. Huwez F, Casswell EJ, FAST-AV or FAST-AB tool im- AUTHOR CONTRIBUTIONS proves the sensitivity of FAST screening for detection of Richard Ronan Murphy: concept and design, critical revision of manu- script for intellectual content. Abdullah Al Sawaf: acquisition of clinical posterior circulation strokes. Int J Stroke 2013;8:E3. examination findings. Danny R. Rose, Jr.: critical revision of manuscript 10. Giannakopoulos P, Gold G, Duc M, Michel JP, Hof PR, for intellectual content. Larry Goldstein: critical revision of manuscript Bouras C. Neuroanatomic correlates of visual agnosia in for intellectual content. Charles D. Smith: critical revision of manuscript Alzheimer’s disease: a clinicopathologic study. Neurology for intellectual content. 1999;52:71–77.

e60 Neurology 89 August 8, 2017 ª 2017 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. Clinical Reasoning: Two see or not two see−−Is it really double vision? Richard Ronan Murphy, Abdullah Al Sawaf, Danny R. Rose, Jr., et al. Neurology 2017;89;e56-e60 DOI 10.1212/WNL.0000000000004196

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References This article cites 10 articles, 3 of which you can access for free at: http://n.neurology.org/content/89/6/e56.full#ref-list-1 Subspecialty Collections This article, along with others on similar topics, appears in the following collection(s): All Cerebrovascular disease/Stroke http://n.neurology.org/cgi/collection/all_cerebrovascular_disease_strok e Clinical neurology examination http://n.neurology.org/cgi/collection/clinical_neurology_examination Diplopia (double vision) http://n.neurology.org/cgi/collection/diplopia_double_vision Visual fields http://n.neurology.org/cgi/collection/visual_fields Visual processing http://n.neurology.org/cgi/collection/visual_processing Permissions & Licensing Information about reproducing this article in parts (figures,tables) or in its entirety can be found online at: http://www.neurology.org/about/about_the_journal#permissions Reprints Information about ordering reprints can be found online: http://n.neurology.org/subscribers/advertise

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