Alice in Wonderland syndrome

Osman Farooq and Edward J. Fine. Alice in Wonderland Syndrome: a Historical and Medical Review. Pediatric Neurology. In press.

Abstract

Alice in Wonderland syndrome is a disorienting neurological condition that affects human perception to the senses of vision, hearing, touch, sensation and the phenomenon of time. Individuals affected with Alice in Wonderland syndrome can experience alterations in their perception to the size of objects or their own body parts, known as metamorphopsias. It is known to occur in conditions including migraine, epilepsy, as well as certain intoxicants and infectious diseases. The name refers to Lewis Carrol's well-known children's book Alice's Adventures in Wonderland , in which the title character experiences alterations of sensation in which she felt that her body had grown too tall or too small, or parts of her body were changing shape, size or relationship to the rest of her body. The syndrome was described in 1952 by Caro Lippman, and given its name in 1955 by John Todd. The metamorphopsias characteristic of this condition are also sometimes referred to as Lilliputian hallucinations , as a reference to the fictional island of Lilliput in the novel Gulliver's Travels , written by Jonathan Swift in 1726. As such, many literary and medical publications have roots in the description of this syndrome. The purpose of this review is to summarize the literary and historical significance of Alice in Wonderland syndrome as well as to provide the reader with a medical overview of the condition.
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From the article

Alice in Wonderland syndrome and the Brain

Nuclear medicine techniques using technetium during episodes of Alice in Wonderland syndrome have demonstrated decreased cerebral perfusion in various regions of the brain; the frontal, parietal, temporal and occipital lobes, either individually or in combination. It was postulated that any condition that caused a decrease in perfusion in the visual pathways or visual centers of the brain could be responsible for the syndrome. Kuo et al examined four patients with Alice in Wonderland syndrome using SPECT brain scans. For all four patients, areas of reduced cerebral perfusion in the temporal lobe were observed.   Hiemer, et al . believed that the syndrome was due to an unspecific cortical dysfunction resulting from several possible causes: decreased cerebral perfusion, epileptic activities or encephalitis.   Another theory suggests that the body image disorders originate in the parietal lobe. Studies have illustrated that electrical stimulation of the posterior parietal cortex can produce disturbances of body image, including the sensation of body length and size distortion.  Other authors have postulated that decreased blood flow to the non-dominant posterior parietal lobe during a migraine attack may cause metamorphopsias. This is also likely the reasoning of why Alice in Wonderland syndrome can occur in space occupying lesions in the brain, e.g. brain tumors. 

Migraine Headaches

Although the exact mechanism of why migraines can cause Alice in Wonderland syndrome is not known, there are theories that involve both electrical as well as vascular phenomenon. One theory suggests that transient, localized ischemia in areas of the visual pathway may be related to the visual distortions.  36  In addition, during migraines, a spreading wave of depolarization of cells in the cerebral cortex occurs. Depolarization of the glial cells causes extracellular release of potassium and calcium ions, nitric oxide, and arachidonic acid, which in turn activate meningeal nociceptor axons. Nocioceptor neurons are part of the trigeminal nerve's regulation of the brain's vascular system which becomes activated during a migraine headache. Because of the trigeminal nerve's connections to the thalamus and thalamic projections onto the sensory cortex, migraine patients feel intense cranial pain during an attack. At times, the symptoms that occur with Alice in Wonderland syndrome can precede, accompany or even replace the typical symptoms associated with migraines.

Epilepsy

Similar to the electrical depolarization theory in migraines, the intense localized electrical changes that occur in the brain during a seizure can induce the symptoms of Alice in Wonderland syndrome . These have been described to occur in association with seizures originating in the frontal, occipital and parietal lobes as well as temporal lobe epilepsy. The symptoms of AIWS can occur as part of the aura that can precede seizures, or as part of the actual seizure…

Prognosis

Alice in Wonderland syndrome can occur at any age, but appears to be more common during childhood and adolescence. The outcome is usually benign, particularly in children. Most often, patients outgrow these episodes. The long term prognosis typically depends on the etiology of the condition and the underlying condition must be evaluated. Treatment must be directed at the underlying condition. When symptoms are transient and not associated with any other pathology, reassurance that the symptoms themselves are not harmful may suffice.   Alice in Wonderland syndrome can be difficult to diagnose, partly because patients may be reluctant in verbalizing their symptoms for fear of being labeled as ‘crazy’.  Without the knowledge that the bizarre hallucinations of the syndrome can occur in conjunction with common conditions such as migraines, epilepsy and infection, clinicians may be quick to treat with psychotropic drugs or refer patients for psychiatric evaluations.  Weidenfeld and Borusiak conducted a long-term follow-up study of patients and concluded that after ruling out acute medical conditions, such as encephalitis and partial epilepsy, Alice in Wonderland syndrome is a benign self-limiting condition.  Indeed the challenge can lie in making the diagnosis. Fortunately, once identified, the outcome is generally favorable.