Dr. Ron’s Research Review – April 20, 2016

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This week’s research review focuses on Alice in Wonderland syndrome.

Alice in Wonderland syndrome (AIWS), named for Lewis Carroll's titular character, is a disorder characterized by transient episodes of visual hallucinations and perceptual distortions, during which objects or body parts are perceived as altered in various ways (metamorphopsia), including enlargement (macropsia) or reduction (micropsia) in the perceived size of a form. (Todd, 1955)

A total of 48 patients (children average age 8.1 years) diagnosed with "Alice in Wonderland" syndrome or "Alice in Wonderland"-like syndrome were identified. Common visual symptoms were micropsia (69%), teleopsia (50%), macropsia (25%), metamorphopsia (15%), and pelopsia (10%). The etiology was infection in 33% of patients and migraine and head trauma in 6% each. No associated conditions were found in 52%. The symptoms usually resolve, but in more than one third of the cases, they continue. One quarter of patients without a history of migraine may subsequently develop migraine. (Liu et al., 2014)

Several case studies describe Alice in Wonderland syndrome as an initial manifestation of a viral infection, including Epstein-Barr virus. (Tselis, 2014)

 

Dr. Ron


 

Articles

Alice in wonderland" syndrome: presenting and follow-up characteristics.
            (Liu et al., 2014) Download
BACKGROUND:  We investigated the distribution of symptoms and etiologies of patients with "Alice in Wonderland" syndrome (visual perception of change in one's body size) and "Alice in Wonderland"-like syndrome (extrapersonal illusions) at presentation and to determine their prognosis. DESIGN:  Retrospective chart review and telephone interview. METHODS:  Charts of children diagnosed with "Alice in Wonderland" syndrome by a pediatric neuro-ophthalmologist between July 1993 and July 2013 were reviewed. Patients seen before 2012, or their parents, were contacted for follow-up information. RESULTS:  A total of 48 patients (average age 8.1 years) diagnosed with "Alice in Wonderland" syndrome or "Alice in Wonderland"-like syndrome were identified. Common visual symptoms were micropsia (69%), teleopsia (50%), macropsia (25%), metamorphopsia (15%), and pelopsia (10%). Magnetic resonance imaging and electroencephalography were unrevealing in 21 of 21 and 23 of 23 cases, respectively. The etiology was infection in 33% of patients and migraine and head trauma in 6% each. No associated conditions were found in 52%. Of the 15 patients with follow-up, 20% had a few more events of "Alice in Wonderland" syndrome or "Alice in Wonderland"-like syndrome, which eventually stopped after the initial diagnosis; 40% had no more events, and 40% were still having "Alice in Wonderland" syndrome or "Alice in Wonderland"-like syndrome symptoms at the time of the interview, while four patients (27%) developed migraines and one patient (7%) seizures since the diagnosis. CONCLUSION:  "Alice in Wonderland" syndrome and "Alice in Wonderland"-like syndrome typically affect young children, and the most common visual complaints are micropsia and teleopsia. The most common associated condition is infection, but half of these individuals have no obvious trigger. Magnetic resonance imaging and electroencephalography are not helpful. The symptoms of "Alice in Wonderland" syndrome and "Alice in Wonderland"-like syndrome usually resolve, but in more than one third of the cases, they continue. One quarter of patients without a history of migraine may subsequently develop migraine.


 

The syndrome of Alice in Wonderland.
            (Todd, 1955) Download
The purpose of this paper is to draw attention to a singular group of symptoms intimately associated with migraine and epilepsy, although not confined to these disorders. While there is wide appreciation of the fact that epileptic subjects, and their blood relatives, are prone to experience bizarre disturbances of the body image, few realize that essentially similar disorders affect migraine subjects and their families. As a result, many of these patients are unjustifiably dubbed "neurotic" and referred to a psychiatrist, while others torture themselves with secret misgivings concerning their sanity.

Epstein-Barr virus infections of the nervous system.
            (Tselis, 2014) Download
The Epstein–Barr virus (EBV) is a human DNA virus, ubiquitously present in humans since ancient times, which can cause a number of different diseases or no disease at all. It is like other human herpesviruses in that, once the acute infection resolves, the virus enters a latent state, with periodic “reactivations” which can be asymptomatic or fatal. The pathogenesis of EBV disease is somewhat unconventional, as will be discussed below.

 

 

References

Liu, AM, et al. (2014), ‘Alice in wonderland” syndrome: presenting and follow-up characteristics.’, Pediatr Neurol, 51 (3), 317-20. PubMed: 25160537
Todd, J (1955), ‘The syndrome of Alice in Wonderland.’, Can Med Assoc J, 73 (9), 701-4. PubMed: 13304769
Tselis, AC (2014), ‘Epstein-Barr virus infections of the nervous system.’, Handb Clin Neurol, 123 285-305. PubMed: 25015491