Cases That Test Your Skills

The woman who saw the light R. Andrew Sewell, MD, Megan S. Moore, MD, Bruce H. Price, MD, Evan Murray, MD, Alan Schiller, PhD, and Miles G. Cunningham, MD, PhD

How would you Since her first psychotic break 7 years ago, Ms. G sees dancing handle this case? lights and splotches continuously. What could be causing her Visit CurrentPsychiatry.com to input your answers visual ? and see how your colleagues responded

HISTORY Visual disturbances history of . Ms. G has a history of ex- Ms. G, age 30, has schizoaffective disorder and periencing well-formed visual hallucinations, presents with worsening auditory hallucina- such as the devil, but has not had them for tions, paranoid delusions, and thought broad- several years. casting and insertion, which is suspected to be She had 5 to 10 episodes over the last 2 related to a change from olanzapine to aripip- years that she describes as seizures—“an razole. She complains of visual disturbances— earthquake, a huge whooshing noise,” accom- a of flashing lights—that she panied by heart palpitations that occur with describes as occurring “all the time, like salt- stress, in public, in bright lights, or whenever and-pepper dancing, and sometimes splotch- she feels vulnerable. These episodes are not es.” Her visual symptoms are worse when she associated with loss of consciousness, incon- closes her and in dimly lit environments, tinence, amnesia, or post-ictal confusion and but occur in daylight as well. Her vision is oth- she can stop them with relaxation techniques erwise unimpaired. such as deep breathing and reminding herself The visual disturbances started with Ms. G’s to calm down. The last episode was 2 months first psychotic break at age 23 and have per- ago and seemed unrelated to her visual phe- sisted continuously. She reports that at first, nomena. She also complains of occasional the spots were more intense, like an incessant paresthesias in her extremities but denies diz- strobe light. These symptoms are aggravated ziness, presyncope, or blurry or double vision. by sleep deprivation and anger and improve Ms. G is obese and has no history of head only with topiramate, 100 mg/d, which was injury or birth defects. Her family history is prescribed a year earlier after a neurologic negative for ; however, her mother consultation but discontinued because of cog- has bipolar disorder and father has schizo- nitive side effects. phrenia. Her medications are phentermine, Ms. G also complains of dull, aching, contin- 37.5 mg/d for short-term management of uous in the center of her forehead, which do not wake her, are not worse in the Dr. Sewell is clinical instructor in psychiatry, Yale University School morning, and do not vary with the intensity of Medicine, New Haven, CT. Dr. Moore is an instructor in psychiatry, of her visual symptoms. The headaches are in- Dr. Price is chief of neurology, Dr. Murray is a staff neurologist and instructor in neurology, Dr. Schiller is a clinical neuropsychologist corporated into her system of delusions; she and assistant director of the consultation-liaison service, believes they “drain the electricity from her neuropsychology department, and Dr. Cunningham is director Current Psychiatry of the Laboratory for Neural Reconstruction, McLean Hospital/ 44 July 2010 head” and make her feel better. She denies a Harvard Medical School, Boston, MA. Cases That Test Your Skills

obesity, oxcarbazepine, 600 mg/d for sup- , or in the peripheral posed partial seizures, sertraline, 100 mg/d for that are worse in dim illumina- depression, aripiprazole, 10 mg/d, and nizati- tion and localizable by the patient. Retinal dine, 150 mg/d, for gastroesophageal reflux detachment can cause , floaters, disease. She does not use alcohol, cigarettes, or a “curtain” or “shadow” moving over or illicit drugs. Ms. G obtained a master’s de- the visual field, which may be accompa- gree in health communications and works as nied by central or peripheral vision loss.3 a research assistant at a local medical school. Severe myopia is a risk factor for . Which condition does Ms. G’s presentation New-onset photopsia can suggest cy- suggest? tomegaloviral retinitis in an immune- a) compromised patient, and ocular-vascular b) occipital lobe seizure crisis in a patient with . Clinical Point c) arteriovenous malformation New-onset or recurrent photopsia with bi- Treatment of d) retinal detachment lateral blurred vision, ataxia, vertigo, dys- e) all of the above arthria, or drop attacks may be caused by hallucinations varies vertebrobasilar artery insufficiency (VBAI). from emergent Photopsia has been reported as the present- ophthalmologic exam The authors’ observations 4 ing symptom in carotid artery dissection. and retinal to Visual hallucinations are not distortions Photopsia correlated with movement antipsychotic therapy of reality but original false implicates the or . All of that co-occur with real perceptions.1 They these etiologies except for VBAI should be to simple observation vary from simple, elemental hallucina- referred to an ophthalmologist for indirect tions involving flashes of light or geomet- ophthalmoscopy and evaluation. ric figures to more complex elaborations such as seeing crawling insects or a flock Nonemergent causes of photopsia include: of angels.2 Hallucinations can originate • drug toxicity anywhere in the from the • migraine lens to the visual cortex and can be vas- • thyroid ophthalmopathy cular, toxic, electrophysiologic, structural, • arteriovenous malformations or neurochemical manifestations of neuro- • seizures logic, psychiatric, or ophthalmologic dis- • psychiatric disorders. orders. Depending on the hallucination’s Digoxin, cocaine, paclitaxel, and clo- etiology, treatment varies from emergent miphene may have ocular side effects, ophthalmologic examination and retinal including photopsia. The first symptoms surgery to antipsychotic therapy to simple of digitalis toxicity often are visual and observation. Photopsia is a include photopsia, yellow or green discol- of flashing lights, and its etiology varies; oration of the visual field, halos, and the causes can be categorized as emergent or appearance of frost over objects.5 Signs nonemergent. of cocaine intoxication can include visual hallucinations such as photopsia, shad- Emergent causes of photopsia typi- ows, moving objects, and insects crawling cally have a sudden onset, often can be and co-occurring euphoria, hypervigi- diagnosed from associated symptoms or lance, impaired judgment, and autonomic medical history, and must be recognized changes such as tachycardia, pupillary quickly to prevent permanent vision loss. dilation, , and nausea.6 High- Posterior vitreous detachment and retinal dose paclitaxel infusion has been reported Current Psychiatry tearing can manifest as flashes of light, to cause photopsia.7 Clomiphene can cause Vol. 9, No. 7 45 Cases That Test Your Skills

Table 1 Diagnoses suggested by photopsia phenomena Feature Suggested diagnosis Appearance Twinkling lights with grey spots Emboli Zigzag lines Occipital epilepsy White flashes Vitreous traction on the retina Flickering lights and shimmering Clomiphene use Temporally located flashes of light, floaters, or Posterior vitreous detachment and retinal tearing blurred vision that is worse in dim illumination Location Temporal Migraine or retinal pathology Clinical Point Central Occipital lesion or embolus Seizure activity in the Monocular Retinal pathology occipital cortex and Binocular Cerebral cause adjacent areas can Duration Lightning quick Vitreous detachment produce static lights Lasts up to 30 minutes Migraine or occipital epilepsy and stars

flickering lights and shimmering that per- of 18 patients with occipital epilepsy, visual sists after discontinuing the medication.8 seizures lasted from a few seconds to 3 min- Migraine with , which includes utes—although rarely 20 to 150 minutes— photopsia 39% of the time,9 often involves and occurred in multiple clusters a day or an expanding central visual disturbance or week.10 All but 2 patients had secondary , and usually is confined to 1 visual generalized tonic-clonic convulsions. Oc- field but may involve both. The aura typi- cipital seizures often are misdiagnosed as cally lasts 10 to 20 minutes and often is fol- the visual aura of migraine, particularly lowed by headache. Patients often report a when elementary visual hallucinations are history of episodic stereotyped auras and followed by post- and vom- headache. Ocular examination is normal. iting.11 Lights with a color or a spherical Ophthalmic migraine, also known as shape suggest occipital epilepsy. Consider ocular or , is thought to be ordering an electroencephalogram (EEG) if caused by transient of the cho- the clinical diagnosis is unclear. roidal or retinal arteries and can be precipi- tated by postural changes, exercise, and oral Which approach would you chose to evaluate contraceptives. It manifests as a gradual vi- Ms. G’s photopsia? sual disturbance in a mosaic scotomata pat- a) EEG tern that enlarges, producing total unilateral b) thyroid function tests visual loss lasting from minutes to an hour, c) urine toxicology and—misleadingly—can be associated with d) consultation minimal or no headache. Ocular examina- e) all of the above tion is normal, and a personal or family his- tory of migraine confirms the diagnosis. Seizure activity in the occipital cortex EXAMINATION No obvious cause and adjacent association areas can produce During a mental status exam, Ms. G is pleas- Current Psychiatry 46 July 2010 static light and stars. In a prospective study ant, cooperative, alert, and oriented to person, Cases That Test Your Skills

Table 2 Photopsia differential diagnosis: Look for co-occurring symptoms

Co-occurring symptoms Suggests Seizure activity associated with flashing lights Occipital epilepsy Yellow or green discoloration to the visual field, halos, and Digitalis toxicity appearance of frost over objects Shadows, moving objects, and insects crawling with co- Cocaine intoxication occurring euphoria, hypervigilance, impaired judgment, and autonomic changes such as tachycardia, pupillary dilation, hypertension, and nausea Floaters, or a ‘curtain’ or ‘shadow’ moving over the visual field, Retinal detachment which may be accompanied by central or peripheral vision loss Bilateral blurred vision, ataxia, vertigo, dysarthria, or drop attacks Vertebrobasilar artery insufficiency Clinical Point The appearance, place, and time. Her speech is fluent, her af- ity testing, visual field testing, and fun- location, and duration fect is full, and she describes her mood as a duscopic examination. The appearance, of photopsia may help “sensitive, overwhelmed state.” Her thoughts location, and duration of photopsia may narrow the differential occasionally are tangential, and she persever- help narrow the differential diagnosis diagnosis and identify ates on guilty, embarrassed, and paranoid Table 1 and and identify emergent cases ( emergent causes thoughts. She has auditory hallucinations and Table 2). Seizure activity associated with experiences photopsia during the interview, flashing lights suggests occipital epilepsy. but denies other visual hallucinations. She A history of hypertension, diabetes, or shows no deficits in attention, concentration, polymyalgia rheumatica points to vascu- memory, language, calculations, visuospatial lar causes, and hyperthyroidism suggests abilities, or general fund of knowledge. that flashing lights may be Her physical exam shows pupils that are of Graves’ disease. Myopia or eye trauma equal, round, and reactive to light with normal could indicate vitreous traction. Prescrip- extraocular movements, intact visual fields, tion medications and illicit drug use may 20/20 visual acuity in both eyes with glasses, point to a toxic etiology.12 and sharp optic disc margins with no retinal abnormalities apparent on funduscopic exam. The rest of her physical and neurologic exam OUTCOME Diagnosis of exclusion is normal. Her complete blood count, electro- Ms. G’s visual disturbance resembles vitreous lytes, kidney, liver function tests, urinalysis, detachment or classic migraine; however, and serum toxicology screens are normal. She the onset is not sudden, her funduscopic has no history of immunodeficiency. exam is normal, and she has no discrete epi- An MRI performed a year earlier showed sodes with concomitant headache. Ms. G’s mild diffuse congestion of a left maxillary si- improvement with topiramate is inconsis- nus but no other intracranial abnormalities, tent with an ophthalmic origin because such and a waking EEG with sphenoidal electrodes photopsia should not improve with medica- was normal. tion. It is consistent with recurrent seizures or status epilepticus of the occipital cortex; however, she did not experience multiple The authors’ observations discrete episodes a day or generalized sei- Evaluation of patients with photopsia in- zures experienced by most occipital seizure cludes taking a history and performing patients, and her EEG and clinical history Current Psychiatry an eye examination including visual acu- are not consistent with seizures. Because Vol. 9, No. 7 47 Cases That Test Your Skills

References 1. Jaspers K. General psychopathology. Manchester, United Related Resources Kingdom: Manchester University Press; 1962. • Ophthalmology Web. www.ophthalmologyweb.com. 2. Manford M, Andermann F. Complex visual hallucinations. • Aleman A, Larøi F. Hallucinations: the science of idiosyncratic Clinical and neurobiological insights. Brain. 1998;121:1819- 1840. perception. Washington, DC: American Psychological Association; 2008. 3. Amos JF. Differential diagnosis of common etiologies of photopsia. J Am Optom Assoc. 1999;70(8):485-504. Drug Brand Names 4. Biousse V, Touboul PJ, D’Anglejan-Chatillon J, et al. Ophthalmologic manifestations of internal carotid artery Aripiprazole • Abilify Oxcarbazepine • Trileptal dissection. Am J Ophthalmol. 1998;126(4):565-577. Clomiphene • Clomid, Paclitaxel • Onxol, Taxol 5. Butler VP Jr, Odel JG, Rath E, et al. Digitalis-induced Serophene Phentermine • Adipex-P, Inoamin visual disturbances with therapeutic serum digitalis Digoxin • Lanoxin Sertraline • Zoloft concentrations. Ann Intern Med. 1995;123(9):676-680. Nizatidine • Axid Topiramate • Topamax 6. Mitchell J, Vierkant AD. Delusions and hallucinations of Olanzapine • Zyprexa Ziprasidone • Geodon cocaine abusers and paranoid schizophrenics: a comparative study. J Psychol. 1991;125(3):301-310. Disclosure 7. Seidman AD, Barrett S, Canezo S. Photopsia during 3-hour The authors report no financial relationship with any paclitaxel administration at doses > or = 250 mg/m2. J Clin company whose products are mentioned in this article or with Oncol. 1994;12(8):1741-1742. Clinical Point manufacturers of competing products. 8. Purvin VA. Visual disturbance secondary to clomiphene citrate. Arch Ophthalmol. 1995;113(4):482-484. A perceptual 9. Queiroz LP, Rapoport AM, Weeks RE, et al. Characteristics of migraine visual aura. Headache. 1997;37(3):137-141. disturbance related 10. Panayiotopoulos CP. Visual phenomena and headache in occipital epilepsy: a review, a systematic study and to schizoaffective we cannot rule out occipital lobe epilepsy, we differentiation from migraine. Epileptic Disord. 1999; 1(4):205-216. disorder is the most refer Ms. G for repeat EEG and routine oph- 11. Walker MC, Smith SJ, Sisodiya SM, et al. Case of simple thalmologic exam, both of which are normal. partial status epilepticus in occipital lobe epilepsy likely etiology misdiagnosed as migraine: clinical, electrophysiological, Perceptual disturbances are common in and magnetic resonance imaging characteristics. Epilepsia. of Ms. G’s photopsia 13-15 1995;36(12):1233-1236. schizoaffective disorder and schizophrenia, 12. Lichter M. Flashing lights—a warning. The Canadian which we consider the most likely etiology of Journal of Diagnosis. 2002;19:31-35. 13. Parnas J, Handest P, Saebye D, et al. Anomalies of subjective Ms. G’s photopsia, given her normal neuro- experience in schizophrenia and psychotic bipolar illness. logic and ophthalmologic examinations. We Acta Psychiatr Scand. 2003;108(2):126-133. 14. Phillipson OT, Harris JP. Perceptual changes in switch her from aripiprazole to ziprasidone, schizophrenia: a questionnaire survey. Psychol Med. 40 mg/d, discontinue phentermine, and taper 1985;15(4):859-866. 15. Cutting J, Dunne F. Subjective experience of schizophrenia. and discontinue oxcarbazepine. Schizophr Bull. 1989;15(2):217-231.

Bottom Line Because psychiatrists often are consulted to evaluate patients experiencing visual hallucinations, it is important to rule out neurologic and ophthalmologic causes before determining that photopsia has a psychiatric origin. A thorough history with attention to onset, duration, frequency, and associated symptoms can help differentiate between emergent and nonemergent causes of photopsia, and prompt appropriate referrals for electroencephalogram or ophthalmologic examination.

Current Psychiatry 48 July 2010