Alice in Wonderland Syndrome in a Young Woman with past TBI

doi:10.62055/20010262Lh

ABSTRACT

BACKGROUND

When patients experience a distorted sense of object recognition, they might be experiencing a phenomenon called “Alice-in-Wonderland Syndrome” (AIWS). It can result in macropsia, micropsia, pelopsia, telopsia or a combination of factors.  Patients with a history of head injury often have a slew of neurological complications and eye care providers should understand how to differentiate this condition from hallucinations, understand appropriate work-ups and educate patients accordingly. This article will summarize a patient with multiple functional difficulties following head trauma, with focus placed on her Alice-in-Wonderland Syndrome and a breakdown of the condition as a whole.

CASE REPORT

A 24-year-old Caucasian female presented to the polytrauma optometry clinic due to complaints of light sensitivity, migraines, visual discomfort at near, and instances in which she noticed shimmering in her visual field, along with distortion of the floor and a distorted perception of the size of her feet appearing “the size of a credit card.” In relation to her last complaint, she stated this occurred every 14 days or so, and had been ongoing since a traumatic head injury sustained three years prior.

She was a college student and reported that she struggled with studying due to onsets of migraines within one hour of studying. She reported difficulties in class when focusing at near for too long or when exposed to bright lights from the projector.

Her clinical examination showed refractive error and a trend towards convergence insufficiency and accommodative infacility. Ocular health was generally unremarkable. Part of the management was reassurance and understanding of the etiology of her symptoms, while her binocular vision conditions and photosensitivity were managed with vision therapy and tinted spectacles, respectively. Her AIWS symptoms were determined to be related to her migraines, which were ameliorated as management improved her migraine symptoms.

CONCLUSION

Even the most confusing visual phenomena can be broken down with a systematic work-up and understanding of past literature. Alice in Wonderland Syndrome is relatively rare, but a good understanding of the condition can allow for better management and education given to patients. Furthermore, head trauma can bring a multitude of functional deficits at once, and careful management of each complication is crucial.

Keywords: Traumatic Brain injury (TBI), Alice-in-Wonderland-Syndrome (AIWS), migraine, macropsia, metamorphopsia, convergence insufficiency, accommodative insufficiency, Vision Therapy

CASE REPORT

A 24-year-old Caucasian woman presented to the polytrauma optometry clinic. She had a past history of migraines, which began four years prior following a traumatic brain injury. She noted these migraines would last for about 45 minutes and often had an accompanying “shimmering” aura. Along with this, she had complaints of photosensitivity and binocular diplopia at near. About 8 months following her migraine onset, she began to experience instances in which she would have distortions of size. She described this as parts of her body (often her feet) would “shrink to the size of credit cards.” She was a college student and reported that studying would be a struggle due to migraines from about one hour of nearwork at a time. She would also struggle in class when focusing at near for too long or when exposed to bright lights from the projector. Her medical history also included tachycardia, post-traumatic stress disorder, attention deficit hyperactivity disorder and postural orthostatic tachycardia syndrome.

She had a pertinent history of multiple head injuries before and during her military service. During her time in service, she was strapped to a medical litter (stretcher) when an individual fell and landed on her head, causing her to lose consciousness for about 1-2 hours. Very soon after recovering from this incident, she also contracted COVID-19. After overcoming her illness, she developed subsequent migraines with photophobia, phonophobia, visual, somatic and sensory changes. Prior to her major TBI, she also had multiple concussions from horseback riding and martial arts. A brain MRI taken soon after the TBI revealed a small venous anomaly of the left cerebellar hemisphere, which was deemed to be a normal physiologic variation. There was also mild narrowing of the left transverse sinus, which was also determined to be a normal variation. MRI angiography showed no evidence of mass lesion, hydrocephalus or intracranial hemorrhage. MRI of the brain showed normal findings as well. She underwent an electroencephalogram (EEG) due to her history of migraine headaches with visual disturbances in the absence of seizures. She yielded normal awake and sleep findings, with no clinical or electrographic seizures recorded. Transthoracic echo was normal, and so was her electrocardiogram (EKG).

The patient was established with neurology before presenting for ocular evaluation, and had been diagnosed with migraines, postural orthostatic tachycardia syndrome (POTS), syncopal falls and Alice-in-Wonderland syndrome (AIWS). Neurology deduced that her migraines and POTS developed following the traumatic brain injury, with the subsequent COVID-19 infection possibly contributing to these conditions.

Ocular examination showed visual acuities of 20/20 in each eye, with full confrontation visual fields, round and reactive pupils, full extraocular motilities, full color vision in each eye (tested with Ishihara plates), and equal red cap desaturation. Anterior segment and posterior segment and OCT findings were unremarkable. A 30-2 Humphrey visual field showed no visual field defects.

She described her diplopia as a “mirrored” image, horizontal in nature at distance and near, which worsened with fatigue. Cover test showed ortho posture at distance and 3 exophoric posture at near. Her step ranges at distance were as follows: BI: x/6/4 and BO: x/14/10. Her step ranges at near were as follows: BI: x/10/8 and BO: x/20/18. These are within normal range. Her near point of convergence was reduced at 12 cm break and 16 cm recovery. Accommodative amplitudes were normal for her age, however, accommodative facilities were 5 cycles per minute – lower than the average and she showed difficulty with plus power. Vergence facility was also reduced at 6 cycles per minute, reduced from the norm of 10. She showed 250” global stereo and 70” on randot circles.

She showed a positive response to 1^ BI at near to help with comfort/alleviated diplopia. She was ultimately diagnosed with a trend towards convergence insufficiency, mild accommodative infacility, photosensitivity, vergence infacility, and visual disturbances most likely explained as Alice-in-Wonderland syndrome.

She was prescribed glasses with 1^ BI split prism and +0.50 ADD to use while studying, along with non-prismatic specs with her distance prescription both clear and rose-tinted for light control. She was willing to start vision therapy to improve her vergence and accommodative skills.

For her migraine management, Neurology prescribed Topiramate 200 mg, along with Botox injections and Rimegepant. She was a college student, and with her concurrent vergence dysfunction, was consistently under the stress of migraines when studying during exam season. This improved with vision therapy. Her symptoms of AIWS were correlated with her headaches, and ultimately lessened with control of her migraines.

She was able to complete nine total sessions of vision therapy, showing large strides in her accommodative, oculomotor, and vergence capabilities. She had her final exams around the time of the seventh session. She stated she was feeling the best she ever felt. She was able to read a third of a book in one sitting without issue, and her studying times expanded to about 3 hours before needing a break. She also no longer relied on her prismatic specs with a small add power, she used her everyday clear distance glasses instead. Her migraines had minimized and were no longer triggered by her schoolwork. Her rose-tinted glasses helped to manage her photosensitivity and further alleviate her onset of headaches. Ultimately, with this improvement in migraine reduction, she also noticed a large decrease in the frequency of episodes of seeing the visual phenomena of AIWS.

DISCUSSION

AIWS is a neurological disorder that alters one’s perception of sensory information, including size, space and time. Despite its relative obscurity, there are well-established factors that define this condition. The most common symptomology includes a significant magnification or minification of perceived objects.¹ To use an example from this case, a patient may look at their size 12 shoes and perceive them as the size of a credit card.

Visually, AIWS changes the perception of objects around the individual or the individual themselves. Objects or people may appear larger (macropsia) or smaller (micropsia) than they really are. They could also appear closer (pelopsia) or farther (teleopsia). A combination is possible as well. For example, objects may appear smaller while also perceived as moving farther away (porropsia). Outside of the skewed perception of their own body, individuals may have a distorted connection to their emotions or the passage of time.² Over the last 60+ years, sensory changes that fall under this condition have expanded to 42 visual symptoms and 16 somesthetic and other nonvisual symptoms.² This includes altered perceptions of color, a specific color overlaying the visual field, wrinkling of the visual field, difficulty perceiving motion, illusory splitting of objects, distorted perception of shapes or faces, inverted vision, rotating visual field and loss of stereoscopic vision. ³ Palinopsia is the illusory recurrence of an object in the field of vision even after it’s no longer there, and is present in many cases of AIWS.³ Although this phenomenon is possible at any age, it is most common during adolescence.⁴

AIWS is a distortion of sensory perception. Contrariwise, a hallucination involves specific aspects of outside stimuli not actually there. Therefore, there should first be a differentiation between hallucinations and AIWS. If the patient is seeing objects not within reality, they are experiencing a hallucination, not AIWS. AIWS causes specific changes to real present stimuli, so it cannot be labeled as a hallucination or illusion.⁵ Conditions like isolated migraines, temporal lobe epilepsy, infections, non-specific hyperpyrexia, schizophrenia and other disorders of perception have been interpreted as AIWS.⁶ The condition itself can be a consequence of many of these conditions, but is not always causally connected.⁷  Retinal examination should rule out retinal pathology, including macular degeneration or central serous chorioretinopathy, responsible for metamorphopsias. Furthermore, if there is no recurrence, it may be a consequence of an isolated event, like an isolated migraine, and not AIWS. If symptoms exist in the absence of other neurological or ocular pathology, a diagnosis of AIWS can be determined.

Disorders of visuo-spatial coordination include optic ataxia – defined by the inaccuracy of visually guided movements. It reflects a disintegration of the spatial coordinate frames of the parietal lobe (involved in spatial visual attention) and motor movement.³ A trailing phenomenon, perceived as a persistent recurrence of an image (palinopsia), is commonly seen as well. This can be seen in AIWS, typically because of parietal lobe dysfunction. This disorder contains four distinct subtypes. Polyopia is the perception of multiple copies of the same image, typically arranged in rows and columns. Visual preservation is the perception of an object appearing to stay within its retinal loci, despite the eyes or head moving. This causes an object to stay within a person’s vision despite movement away from the object.  Delayed palinopsia occurs as an object returns into the field of view, almost as an afterimage not seen immediately preceding the real-life image. Illusory spread is the continuation of a pattern from an object to the surrounding area. For example, a design of a checkerboard may perceptually continue onto the nearby desk or floor.^8,3

The temporal lobe is crucial for recognition of familiar objects. Dysfunction of object constancy from a temporal lobe insult may cause macropsia and micropsia on a neurological level in the absence of retinal pathology.¹⁰ This causes a disintegration of an object’s relative distance compared to their visual size. Pelopsia or telopsia may arise as well. One study observing electrical stimulation in the posterior part of the left middle temporal gyrus resulted in the inability to verbalize size judgements (ex: “Is a tree bigger than an ant?”). This was suggested to be via damage to the superior temporal language area and inferior temporal object recognition area.¹⁰

The temporo-parieto-occipital junction of the brain is the site of visual and somatosensory information integration, needed for proper interpretation of self and the external world. The complexity of visual difficulties is theorized to worsen with more anterior lesions. Location near the occipital lobe may carry simple visual sequelae, while lesions near the parietal and temporal lobes may incorporate somatosensory or cognitive dysfunctions.⁸ The visual cortex within the occipital lobe carries its own subdivisions that vary in complexity. The primary visual cortex, referred to as V1, projects to more complex connections, which are referred to as V2-V5. Lesions of V1 will cause a scotoma of a single hemifield. Lesions to the ventral aspect of V2 and V3 are associated with upper quadratic field defects, while lesions to the dorsal aspect of V2 and V3 are associated with inferior field defects. Deficits in V4 along the occipitotemporal pathway show poor size discrimination along a single hemifield.¹⁰ In migraine-related brain insult, resulting scotomas can be pinpointed to these locations.³

Literature most commonly associates AIWS with migraines.¹¹ The moniker itself came from John Todd, an English psychiatrist who experienced sensory distortions of his own body and alluded it to imagery portrayed in the book of the same name.¹² It’s suggested that Lewis Carroll, the author of the book Alice in Wonderland, had experienced some of these body schema illusions, in turn inspiring events in the book. Lewis Carroll had a well-known history of classic migraines, which he stated were preceded by ocular disturbances.¹³

There are established diagnostic criteria for migraine-associated AIWS. The individual must have: 1) one or more episodes of self-experienced illusion or metamorphopsia, 2) duration under 30 minutes, 3) accompaniment of a headache or history of migraine, and 4) MRI, cerebrospinal fluid analysis and EEG all deemed normal. Visual evoked potentials may still be abnormal to qualify as migraine-associated AIWS.⁸

 While central pathology is considered the most prevalent cause, conditions outside the brain itself can lead to AIWS. Symptoms, including visual tilt, may arise from peripheral vestibular dysfunction, primarily through damage to the otoliths.¹⁴ The perception of balance arises from vision, proprioception, and vestibular feedback, along with their neurological central processing. The otoliths of the inner ear are components of the peripheral vestibular system that detect gravity and linear acceleration, enabling proper orientation and balance. Classic presentation of otolith disorders is feeling “drunk” or “tumbling” in the absence of drug use or other neurological disorders.¹⁵

Other causes of AIWS include infections, seizures (specifically at the temporal lobe), stroke, schizophrenia, depressive disorders, brain tumors, degenerative brain diseases, recreational drugs and medications (substance-induced). Potential infectious etiologies are Lyme disease, shingles, Epstein-Barr virus, cytomegalovirus and varicella zoster. Psychoactive drugs known to cause AIWS include marijuana, LSD and cocaine. Pertinent medications that could cause AIWS symptoms are dextromethorphan, dihydrocodeine, citalopram, montelukast, risperidone and topiramate.^4,16 Miscellaneous causes are an altered state of mind before and after sleep (hypnagogic vs hypnopompic state). Hypnotherapy, hyperpyrexia and sensory deprivation have infrequently been associated as well.³

No definitive treatment has yet been determined for AIWS. It is common that symptoms of AIWS may fully self-resolve with time, however, the underlying etiology (i.e. migraines, epilepsy, inflammatory conditions) must be considered.⁴ For benign presentations with no major underlying ocular or systemic pathology, reassurance on the nature of the condition may be enough.

If an underlying diagnosis is determined, appropriate treatment should be administered. Antiepileptic medications, migraine medications, antivirals, antibiotics, drug cessation and sleep intervention may also be necessary depending on the cause. Valproate (an anti-epileptic drug) has also been shown to reduce symptoms.¹⁷ Medication side effects can complicate systemic treatment. For example, topiramate, usually prescribed for migraines, can also cause perceptual disorders including AIWS.³ The same can be said for risperidone, an anti-psychotic medication.¹⁸ If a central lesion is suspected, brain MRI, blood tests and EEG should be considered. MRI can be the most helpful diagnostic tool, although the likelihood of discovering brain abnormalities is low.

One study observed the effect of repetitive transcranial magnetic stimulation (rTMS) at a frequency of 1 Hz at T3P3, overlying Brodmann’s area 40 for a patient with verbal auditory hallucinations along with visual hallucinations in AIWS. Initially, activation was observed in bilateral basal ganglia, auditory cortex, Broca’s area, primary visual cortex and dorsal visual cortex among other areas. At the second week of treatment, sensory deceptions/distortions and mood symptoms showed complete remission. After 8 months, when many of the original symptoms had returned, a second treatment phase with rTMS showed complete remission once again.⁶

CONCLUSION

The patient in this case report was experiencing symptoms of an already-established diagnosis of AIWS. This care team was able to assess MRI, EKG and EEG results to rule out neurological abnormalities. Her symptoms were closely tied to her migraine onset and alleviated with migraine management. Beyond medical therapy, the goal for management was to minimize the visual triggers and sequelae of her migraines. Tints for light sensitivity, spectacle wear for visual comfort and vision therapy to minimize binocular and accommodative stress improved the patient’s quality of life and correlated with improvement in her episodes of AIWS. Ultimately, the most helpful practice clinicians may have is reassurance that the condition can improve over time.

 

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