Jacqueline M.S. Winterkorn, PhD, MD Hallucinations - Definition
The patient claims he sees or behaves as if he can see something that the clinician cannot see. Hallucinations - Definition
Sensory perception without external stimulation of the relevant sensory organ - Simmons Lessell Conditions Associated with Hallucinations
• Psychiatric • Ocular Conditions – Vitreoretinal – Visual Loss: cataract, cornea, ARMD – Emboli – Optic Neuropathy • Drugs • Migraine • Focal Hemispheric • Midbrain [Peduncular] Hallucinosis Visual Hallucinations Traditional Categories
• UNFORMED – Photopsias, Phosphenes – Retinal Stimulation – Deafferentation • FORMED – Animals, People, Scenes – Cortical Visual Hallucinations Traditional Categories
• IRRITATIVE – Abnormal neuronal activity – Seizure-like activity around lesion • RELEASE – Sensory Deprivation – Loss of external stimulation – Injury resulting in severe visual loss Case History
• 28 y/o man on Haldol referred for difficulty reading owing to accommodative insufficiency • Otherwise normal exam 28 year old man on Haldol Hallucinations in Psych Patient
“...Last Christmas when I was in the hospital, I was seeing stuff -- all kinds of stuff…” Characteristics of Psychiatric Hallucinations
• Formed > Unformed • Imprecise Localization • Not lateralized • Present with eyes open or closed • Schizophrenia - complex visual hallucinations • Auditory esp. voices • Delusional Thinking • Lack insight • Controlled by antipsychotics Case History
• 58 y/o nurse in good health • History of migraine with aura • Now c/o seeing brief vertical flashes in the far temporal periphery of the right eye 58 yo nurse in good health. History of migraine. 57 year old woman with history of migraine
“I would see a flash “I would turn my eye a quick look out the side and it was gone “I never see a light if I don’t move my eye “Very brief -- milliseconds -- that’s how quickly it is “When I had the migraines, it was different.” Moore’s Lightning Streaks
• Brief vertical comma-shaped flash • Usually in temporal periphery • Related to vitreoretinal traction as vitreous shrinks with age • Often seen upon entering dark room • Occurs with eye movements • Hallucination: seen by patient in absence of true visual stimulus Process of Vitreous Detachment 1/3 patients over 50 100% over 80 Photopsias, Photisms and Phosphenes from Vitreo-Retinal Traction • Moore’s Lightning Streaks • Nebel’s Saccadic Flick phosphenes – light flashes elicited by eye movements – deformation of posterior vitreous face • Czermak’s accommodation phosphenes – ciliary muscle pull on peripheral retina • Pressure phosphenes Mechanical stimulation of retina in blind children “If you shut your eyes and are a lucky one you may see… a shapeless pool of lovely pale colors suspended in the darkness; then if you squeeze your eyes tighter, the pool begins to take shape and the colors become so vivid that with another squeeze they must go on fire…” - Peter Pan by J.M. Barrie Other Entoptic Phenomena [Visual images produced by eye structures]
• Purkinje figures - blood vessels of the eye in front of the retina near the macula
• Scheerer’s phenomenon - bright “stars” or “dots” moving in squiggly fashion through vision especially when looking at blue sky (white blood cells in retinal capillaries don’t absorb blue light)
• Media opacities (floaters, emboli, etc)
Scheerer’s Phenomenon
• Bright dots moving in squiggly paths, especially when looking into blue light • Normal phenomenon due to white blood cells moving in the capillaries in front of the retina near the macula • White blood cells appear as bright dots because blue light (430 nm is best) is well- absorbed by red blood cells, but not by white. Microemboli: Sparks appear temporally, track outward, hesitate, vanish in periphery
65 year old man s/p BRAO preceded by photopsias
• Emboli • Jagged motion • Tiny • Bright • Occasionally block
Case History
• 23 y/o woman on BCP c/o photopsias after viral illness • Sudden onset flashing lights and bitemporal scotomata • Flickering lines persisting for months 23 yo woman sees flickering lines in bitemporal scotomas “It started April 12th… We were on my boyfriend’s truck… and I started seeing dots
“Just in the light with both eyes open...
“With both eyes open it’s right off to the center of everything…” 23 year old on BCP after virus c/o visual hallucinations
• No prior neuro or endocrine history • Sudden onset dots and lines • Just lateral to center in each eye • Persistent for months • VA 20/20 • No RAPD • VF bitemporal defect
Acute Macular Neuroretinopathy
• Sudden onset paracentral scotoma • Clover-leaf, wedge-shaped dark macular lesions OU • Scotoma consistent with retinal lesions • normal FA, normal ICG • Retinal Lesion seen with red-free light • Usually young women • Hormonal? Immunologic?
Bos and Deutman AJO 1975 Retinal Causes of Visual Loss
• Photopsias in chorioretinal Inflammations – AZOOR acute zonal occult outer retinopathy – AIBSE acute idiopathic blind spot enlargement – MEWDS multiple evanescent white dot syndrome – MFC multifocal choroiditis – PIC punctate inner choroidopathy • Continuous photopsias in or adjacent to a scotoma Case History
67 y/o woman c/o dimness in both eyes, first the left 4 months ago, and then the right a few weeks later. During this time she had frontal HA and continuous “sparkles” in both eyes, most noticeable when reading. She has been in good health despite a 50-pack- year smoking history. Exam
Acuity: 20/25 OU HVF: severely constricted OU Fundus: narrowed arterioles, disc pallor OU
MRI nl ERG prolonged and reduced rod and cone responses confirms global photoreceptor dysfunction Anti recoverin Abs present Cancer Associated Retinopathy
• Episodic Blurring/ Dimming of Vision • Bright photopsias (“sparkles”), mostly continuous • Photophobia/Nyctalopia • Progressive loss of acuity, color and VF • Narrowing of retinal arterioles • Vitreous cells • Affects cones and rods - ERG extinguished • May precede discovery of small cell lung Ca Case History
• 76 year old woman, bilaterally pseudophakic. Complaining of flickering lights in both eyes for the past year. • Like sun flickering through Venetian blinds • Notices upon opening eyes in the morning • Continuous throughout the day • Seen in periphery • Gone in total darkness WHAT IS THE SIGNIFICANCE OF THE PHOTOPSIAS GOING AWAY IN DARKNESS? EXAM
• VA 20/25 ou • AOHRR 5/6 ou • VF full • Pupils No RAPD • Motility full • Retina attached, normal discs MEDICAL HISTORY
• Medical History – angina – atrial fibrillation – pulmonary embolus • Medications – Inderal – Lanoxin – Persantine DIGOXIN LEVEL
1.8 NG/ML [NL .5 - 2.0 NG/ML] Digoxin Toxicity • ERG diagnosis: Prolonged cone b-wave implicit time
• Can occur at “normal” levels of Digoxin • Reversible Symptoms of Digoxin Toxicity
• Flickering - light through venetian blinds • Flashing lights • Mist, haze, dark clouds or snowy vision • Decreased visual acuity/central scotoma • Decreased color vision • Yellow or green tint [Xanthopsia] • Diplopia • Pain on eye movements Digitalis [Foxglove] Characteristics of Drug-Induced Hallucinations
• Formed or unformed • Not lateralized • Environmental Influences - ambient light • Frequently overlooked and ascribed to migraine or entoptic phenomena Some Drugs that Cause Visual Hallucinations
• Amphetamines • Anticonvulsants • Bromocriptine • Clomid • Cocaine • Cycloplegics - atropine, cyclogel, scopolamine • Digoxin • Elavil • Inderal • LSD, Mescalin • NSAIDs • Viagra
Dopamine Agonist Therapy
• Treat hyperprolactinemia in Pituitary Prolactinomas • DA agonists used for chronic therapy – Bromocriptine and Cabergoline • Side effect: hallucinations • Sinemet [dopamine] for PD associated with hallucinations in 1/4 of patients Visual Toxicity of Sildenafil (Viagra) [Don’t it turn his brown eyes blue]
• Patients c/o seeing blue [cyanopsia] • Also reported: HA, AION • Pathophysiology of blue vision – Viagra main action is strong blockade of phosphodiesterase type 5 in penis – Also weakly blocks phosphodiesterase 6 in retina Visual Toxicity of clomiphene citrate (CLOMID) • Photopsias – Shimmering, Pulsing, vibrating • Binocular Phenomena – ‘trailing’ of objects in periphery – palinoptic long-lasting images • Blurred Vision but normal VA, VF • mfERG nl - suggests cortical dysfunction • Not reversible - lasts for years Phosphenes in Optic Neuropathy
• Visual-auditory synesthesia • Auditory - induced visual phosphenes • Seen with optic neuritis - 30% ONTT • Seen after AION 44 year old artist referred for Optic Neuritis
• 6 days ago, acute loss of vision OD • Progressed over 2-3 days • No pain • Visual hallucination elicited by startling sound: auditory induced visual phosphenes
Auditory induced visual phosphenes Photopsias, Photisms, Phosphenes Unformed Visual Hallucinations • Descriptions Suggest Cause – shimmering, pulsing MAR – vibrating in periphery Clomid – snow through headlights retinal ischemia – geometric cortical • Associated Symptoms – Photophobia retinal or migrainous – Scotoma chorioretinal inflammation – Headache migraine Purple Vision
. Pituitary Apoplexy . Giant Cell Arteritis . Carotid Insufficiency . Ischemia Hallucinations from Sensory Deprivation
• After prolonged reduction in environmental stimuli • Normal individuals develop impaired intellectual abilities • Hallucinate – Simple: lines, dots, geometric patterns – Complex: animals, people, wallpaper, architecture, bizarre scenes Charles Bonnet Syndrome Visual Hallucinations in the Elderly
• Hallucinations proportional to visual loss • Hallucinations range from mild to strong • Simple flashes to people, animals, scenes • May be underreported for fear of psych stigma • May resolve when vision improved e.g. CE • Many causes of severe visual loss Causes of Release Hallucinations Injury to the Visual System
• Visual cortex ischemia • Optic neuropathies • Patching or enucleation • Ocular causes Macular Degeneration Corneal Opacity Glaucoma Cataract AIDS/CMV Retinopathy Macular Hole Drugs to control Charles Bonnet Release Hallucinations • ANTI-SEIZURE – Neurontin – Tegretol – Valproate – Valium – Klonopin • ANTI-PSYCHOTIC – Risperdal – Zyprexa – Haldol • ANTI-ISCHEMIA – Gingko Biloba 60 bid and Trental – Propulsid Deafferentation Causes Hallucinations
• Increased spontaneous activity reflects denervation supersensitivity • Increased excitability of neurons may be caused by an increased number of adenylate cyclase sites affecting D1 and D2 receptor function • Hallucinations previously thought to be caused by disinhibition
Burke JNNP 2002 Hallucinations of man with macular hole deafferenting an area of visual cortex
• Fleeting, faint images • Size < 1o - corresponding to size of macular hole • Hallucinations attributable to activation of blobs in V1 and stripes in V2. • Cortex hyperexcitable owing to focal deafferentation
Burke JNNP 2002 Regional Distribution of Hallucinations
• Particular types of hallucinations associated with particular areas of brain – superior temporal sulcus = faces – ventral occipitotemporal ctx = scenes – parietal lobe = palinopsia, perseveration – area V4 - color – visuotopic areas = blobs V1, stripes V2 as defined by fmri and cytochrome stain
Santhouse Brain 2000 Case History
• 16 y/o girl with headaches and episodic visual symptoms followed by loss of consciousness • Told of seizure disorder, but not responding to anticonvulsants Migraine
In the borderland of epilepsy -- near it but not of it. - Gowers, 1907 16 year old girl with headaches 16 year old girl with headaches Told of Seizure Disorder
“As long as I can remember I have had headaches.” “When I have a headache, everything is small and I’m growing…” “I got like a strobe light…when I closed my eyes it was like a heartbeat. “I could not see… 5-10 minutes…the tingling goes through my whole body Basilar Migraine or Bickerstaff’s Syndrome
• Symptoms of posterior circulation insufficiency – VF defects – vertigo – nystagmus – motor and sensory deficits – altered consciousness [RAS] • Young women and children • May be hypersensitive to vasoconstrictors Alice in Wonderland Syndrome
• I’m very small and shrinking • Sign of juvenile migraine – also seen with EBV • Complex hallucinations • Impaired sense of time, body image and visual perception • Pathophysiology speculative – Ischemia Top of Basilar, MCA – Spreading depression Top of the Basilar Syndrome
• Lesions at Midbrain/Diencephalic Junction • Peduncular Hallucinosis – Vivid, colorful, animated scenes – Especially animals and cartoons – Normal or Lillipution dimensions – Lasting seconds to hours – Not frightening • IIIrd nerve palsy and ataxia common
L Caplan Top of the Basilar Syndrome Monocular vs Binocular Hemianopic
• Difficult for patient to discriminate if hallucinations are in the right eye or on the right side of both eyes • May have to demonstrate to patient who lost vision owing to migraine that she didn’t experience blurring of her right eye, but rather of her right visual field.
“I had blurred vision in my right eye”
Ocular Occipital How do you Covered eye Both open know? or closed How long? Secs to Mins. 5-40 mins. What did you Black or grey ZigZag lines see? curtain Scintillations
Pain No HA afterwards
Frequency Rare Common Teichopsia or Fortification Spectrum is a scotoma surrounded by a jagged edge, resembling the walls of a fortified city.
Jagged edges shimmer making a scintillating scotoma.
Starts as small ‘c’ centrally and “marches” across field expanding and enlarging until it disappears in periphery Migrainous Aura
• March from center to periphery typically 20 minutes [IHS allows 4 mins to 1 hour] • Negative scotoma: homonymous hemianopia • Photophobia common • May or may not be followed by headache • Pathophysiology – Leão’s spreading depression 3 mm/min – spreading oligemia – platelet activation and decreased 5HT Other positive phenomena in Migraine
• Blotches • Heat waves • Water running down window pane • Lightning bolts • Zig zag lines • TV snow
9 y/o c/o visual distortions • 4 episodes over the last 8 months lasting about 20 minutes • Starting in the “right eye” and spreading to the “left eye” • Followed by slight headache, but no photophobia, sonophobia, or nausea • Normal neuro-ophth exam, MRI, and MRA • Mother, maternal aunt, and maternal grandmother have migraines with aura
When to work up a migraine
• Aura and Headache always on same side/ never switch sides • Aura lasts longer than an hour • Scotoma does not expand and migrate • Persistent Visual Field defect
Case History
• 56 year old woman with migraines onset age 34. • Teichopsia always on right Headache always on left • Headache associated with right homonymous hemianopia and recently with periods of aphasia.
56 year old woman s/p left occipital meningioma “I am having trouble, hallucinating when I start to read” Tell me what you see? “Men chasing me with guns, that was the worst. That really lasted all night. I kept looking outside, trying to see if I could visualize something else…” Next time they come you look right at them! “I keep thinking somebody’s standing there.” Why don’t you look at it -- does it go away? “YES IT DOES -- gives you a sense of control.” Homonymous Hemianopsia & Hallucinations
• Always ASK if patient seeing anything unusual. • Advise patient that Hallucinations are “normal” – 50% pts with HH • Do hallucinations frighten patient? – Usually just annoying. – They will resolve in weeks. • Explain how patient can control hallucinations – look directly at image -- it will flee “Release” or deafferenting hyperexcitability Hallucinations
• Occipital or parieto-occipital lesions • Stroke victims report seeing: – “typical migraines” – phosphenes - colored spots – photopsias - geometric shapes – TV static – objects – animals – people – complex scenes Irritative Hallucinations
• Focal epileptiform • Less common than release hallucinations • Attacks are brief and stereotypic • Confined to an area of visual field • Visual disturbance follows head pain • Reported more often with right brain lesions • Classical dichotomy – unformed - occipital – formed - temporal • Treat with anticonvulsants Allesthesia and Palinopsia
• Allesthesia - Transfer of a visual percept from the seeing field to a non-seeing field
• Palinopsia - Perseveration of a visual percept longer than the stimulus is present
• Both found with [right] parietal lesions Case History
• 34 y/o woman with left-sided headaches x 10 years and aura of flashing lights on the right • Workup for syncope revealed left parietal AVM • Now post-op 34 y/o woman s/p resection of left parietal AVM “I seem to hold that person’s face -- hold the image -- in the blind spot a multiple amount of times.
“...I could tell ‘cause that color is there, in my blind spot...
“It could last one - three, four, five minutes.” Visual Allesthesia
• Visual information from normal homonymous VF is transferred to the other abnormal hemifield.
• Pt sees images in the defective left VF transposed from the good right field. Transfer from right brain to damaged left brain seldom reported.
• Epileptogenic activity implicated. Anticonvulsants decreased occurrence. Case History
• 31 y/o man in good health • Headache workup revealed left parieto-occipital meningioma • Post-op has right inferior quadrantanopsia s/p left parietal meningioma, has R inf quad defect 31 yo s/p L parietal meningioma has R inf quad defect “Some would move fast, some would move slow…and I thought, interesting, what is it?
“I noticed one of the nurses had a hairstyle different than others…she had a bun...and one of the figures that walked by on my right side had a bun
“The real stuff that was going on on the left side, I saw in shadow on my right side”
“You’d see it when the curtain was closed.” Brain Mechanisms
• Palinopsia: Hold in blind field what occurred briefly in good field – Subcortical transfer of info without cortical inhibition to limit time • Why seldom reported with Left-sided lesions? – Usually left-sided lesions impair ability to describe phenomena Moral of the Story
We don’t know all the underlying mechanisms, but we can localize affected sites in the brain and make important diagnoses by listening to patients’ descriptions of their hallucinations. Frumious Bandersnatch Hallucinations
Jacqueline M.S. Winterkorn, PhD, MD HALLUCINATIONS
Jacqueline M.S. Winterkorn, PhD, MD That’s All Folks Extra slides Parasomniac Hallucinations
• At bedtime or awakening from sleep • Formed • Drug-Induced • Common in PD Allesthesia and Palinopsia after Left parietal lesion.
• Same phenomenon on left or right, but pts with left sided lesions may not talk about it Signs of Carotid Insufficiency Migraine in Lupus • Visual hallucinations common • Usually scintillating zig zag lines and fortification spectra • Associated with Headache and Homonymous hemianopsia • Vascular dysfunction suspected – Vasospasm – Spreading depression – Serotonin
Homonymous Hemianopsia and Hallucinations • Localize lesion e.g. With HH, if RAPD=OT; Pursuit defect=parietal; Central sparing=occipital • Check for other visual syndromes [alexia, agraphia, prosopagnosia, disorientation] • Always ASK if patient seeing anything unusual. • Advise patient that Hallucinations are normal – 50% with HH • Do hallucinations frighten patient? – Usually just annoying. – They will resolve in weeks. • Explain how patient can control hallucinations – look directly at image -- it will retreat
Photopsias, Photisms, Phosphenes Unformed Visual Hallucinations • Descriptions Suggest Cause – shimmering, pulsing MAR – vibrating in periphery Clomid – snow through headlights retinal ischemia – geometric cortical • Associated Symptoms – Photophobia retinal or migrainous – Scotoma chorioretinal inflammation – Headache migraine Qualities of Photopsias Localizing Value
Continuous photoreceptor Episodic migraine seizure Evoked vitreous traction Spontaneous migraine Colored seizure Achromatic migraine Single vitreous traction Multiple ischemia Diffuse drug toxicity Localized AMN AZOOR Monocular ocular Binocular cortical Qualities of Photopsias Localizing Value
Continuous photoreceptor Episodic migraine seizure Evoked vitreous traction Spontaneous migraine Colored seizure Achromatic migraine Single vitreous traction Multiple ischemia Diffuse drug toxicity Localized AMN AZOOR Monocular ocular Binocular cortical
Suspicious Migraines
• Symptoms and Headache never switch sides • Scotoma does not expand and migrate • Aura lasts longer than an hour • Visual field deficit persists Case History A healthy 9 year old girl awoke in the night and called to her mother. When her mother entered the room, the little girl said she could only see the right half of her mother’s face. A week later she had an episode when she saw a c-shape glistening in her left visual field for about an hour, followed by headache. There was a family h/o migraine.
• VA 20/20 OU • AOHRR color 6/6 OU • No RAPD
56 yo woman with migraine
EXAM VA 20/25 ou AOHRR 5 1/2 of 6 ou Pupils No RAPD Eye Movements normal Fundus nl D,V, M
[Classic] Migraine with Aura
• Teichopsia or Fortification Spectrum is a scotoma surrounded by a jagged edge, resembling the walls of a fortified city. • Jagged edges shimmer making a scintillating scotoma. • Starts as small ‘c’ centrally and “marches” across field expanding and enlarging until it disappears in periphery
85 y/o migraineur c/o attacks of vertigo
• First attack the day after sailing in hot weather • Difficulty looking up with neck extended: ─Judging wind at the top of the spinnaker ─Art class model on a raised platform • History of acephalgic migraines • Normal neuro-ophthalmic exam Beauty Parlor Syndrome
• Vertebrobasilar insufficiency • Avoid extending neck • Daily ASA