Approximately 39 million people in the US have migraine attacks annually.1 Migraine attacks are headaches that include any part of the head or face and are defined as
- last 4 to 72 hours (treated or untreated);
- with phonophobia, photophobia, or nausea;
- and at least 2 of the following
- unilateral pain,
- pulsating pain,
- moderate to severe pain intensity, or
- aggravation from physical activity.2
Approximately one-third of people who have migraine also experience aura (see Case) as part of their prodrome. A migraine aura can present as multiple different features ranging from fortification spectra of jagged lines that move and leaves transient visual loss behind, colorful or black and white flashes similar to a kaleidoscope in a semicircle shape surrounding an area of transient visual loss, or sensory synesthesia that can spread to the face. Transient dysphasia, which can be mistaken for a symptom of stroke, occurs in 20% of people who experience migraine with aura. When more than one type of aura occurs it usually progresses from visual to sensory symptoms, followed by dysphasia.3 Sometimes, as in the Case presented here, a stranger aura can present that blurs the lines between fiction and reality.
Alice in Wonderland Syndrome (AIWS) is an episode of visual or perceptual distortions of surrounding objects or parts of the body. This aura can last from 10 minutes to a full month. It is named after Lewis Carroll’s fictional character Alice first becomes 10 inches high after drinking from a bottle labeled “DRINK ME.” Later, after eating a piece of cake labeled “EAT ME,” Alice found “when she looked down at her feet, they seemed to be almost out of sight. They were getting so far off.”4 This sounds quite like reminiscent of the experience of Ms. T in the case presented here.
The symptom is called teleopsia, describing objects appearing further away than they actually are. Other manifestations of AIWS are termed micropsia when objects appear smaller than in reality, macropsia when objects appear larger than in reality, and pelopsia, in which objects appear closer than they are. Some reports include a sense of time speeding up or slowing down. The eye is not affected, but rather, perception and visual processing are altered.5,6
These neurologic illusions need to be differentiated from psychiatric psychosis or hallucination. Although schizophrenia, psychotic disorders, and psychoactive drug intoxications (mescaline and other hallucinogens) can lead to visual or perceptual distortions, in AIWS, the person is aware that what they perceive isn’t real. The AIWS has been observed in the context of complex partial seizures, migraine, Epstein-Barr viral infections (mononucleosis), cerebral lesions, and medication side effects (topiramate).6,7
Imaging studies used as a looking glass into the cranium offer a glimpse into the mechanism of AIWS. MRI case reports show visual and auditory hallucinations (both of which are hallmarks of AIWS) can occur in vascular malformations in the right temporoparietal region. Using single-photon emission CT (SPECT) to observe oxygenation perfusion in people with acute AIWS, abnormal blood flow localized to the temporal, occipital, and adjacent perisylvian fissure, all of which are involved in the visual pathway and the visual cortices, was seen.8 In another study, functional MRI (fMRI) was used to observe a boy, age 12 years, experiencing micropsia; increased activation in parietal lobe cortical regions was observed, with reduced activation in primary visual and extrastriatal cortical regions the latter of which are known to be used in the perception of human body parts.9 In most imaging studies of AIWS, symptoms are localized to the temporoparietal junction and occipital visual pathways.
No further workup is required when a person with migraine has AIWS aura and treatment remains the same. For other instances of AIWS, seizures, epilepsy, infections, cerebral lesions, and neurotoxicity should be in the differential diagnosis. Therapy for AIWS that is a migraine aura is the same as for other forms of migraines, which is acute and/or prophylactic medications tailored to the individual.
1. Burch RC, Buse DC, Lipton RB. Migraine: epidemiology, burden, and comorbidity. Neurol Clin. 2019 Nov;37(4):631-649.
2. Headache Classification Committee of the International Headache Society. The international classification of headache disorders, 3rd edition. Cephalalgia. 2013; 33(9);629-808.
3. Ilik F, Ilik K. Alice in Wonderland syndrome as aura of migraine. Neurocase. 2014;20(4):474-475.
4. Carroll L. Alice’s Adventures in Wonderland. London, UK: Chadwick and Sons; 1865.
5. Evans RW. The Alice in Wonderland syndrome. Headache. 2004;44(6):624-625.
6. O’Toole P, Modestino EJ. Alice in Wonderland syndrome: a real life version of Lewis Carroll’s novel. Brain Dev. 2017;39(6):470-474.
7. Kuo YT, Chiu NC, Shen EY, Ho CS, Wu MC. Cerebral perfusion in children with Alice in Wonderland syndrome. Pediatr Neurol. 1998;19(2):105-108.
8. Philip M, Kornitzer J, Marks D, Lee HJ, and Souayah N. Alice in Wonderland syndrome associated with a temporo-parietal cavernoma. Brain Imaging Behav. 2015;9(4):910-912.
9. Brumm K, Walenski M, Haist F, Robbins SL, Granet DB, Love T. Functional MRI of a child with Alice in Wonderland syndrome during an episode of micropsia. J AAPOS. 2010 Aug;14(4):317-322.
DM reports no disclosures.Acknowledgment
This article was previously published in the Texas Neurologic Society newsletter Broca’s Area, and is reproduced here with permission.