Alice in wonderland syndrome

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Disease Entity

Alice in wonderland syndrome is a disorder of visual perception. It was inculcated into medical literature by Lipmann in 1952, where he described it as an impairment of time,sense and body image[1]. Symptoms are similar to those perceived by the character in the book (Alice in wonderland) by Lewis Caroll.

Etiology[2]

It is a rare neuro-ophthalmological entity with varied causes. No large epidemiological data has been published yet. The possible and common causes are listed below. Migraine is the most common cause followed by Infections principally by Epstein Barr virus. AIWS primarily involves the paediatric population.

  1. Migraine
  2. Epilepsy
  3. Hypnotic states
  4. Drug intoxication with lysergic acid diethylamide (LSD) or marijuana,
  5. Fever
  6. Cerebral lesions
  7. Schizophrenia
  8. Infectious mononucleosis
  9. Encephalopathy by coxsackievirus , H1N1 influenza, borrelia burgdorferi, scarlet fever, typhoid fever,
  10. Toxic encephalopathy
Type Disease
Nervous system pathologies Migraine, Temporal lobe epilepsy, trauma associated encephalopathy, brain tumors, intracranial aneurysms, acute disseminated encephalomyelitis
Psychiatric pathologies schizophrenia,depressive syndrome,derealization/depersonalization disorder
Infectious diseases lymes disease, shigellosis,Epstein Barr virus infection, coxsackievirus infection,H1N1 influenza virus, CMV and varicella
Drugs Montelukast,Dextromethorphan, Topiramate,risperidone
Psychoactive substances Marijuana,LSD,cocaine,amantia muscaria

Pathophysiology

The TPO-C which connects the temporo-occipital, parieto-occipital, and temporo-parietal junctions is where visual and somatosensory information is integrated to generate the inner and external representation of self. Other complex perception can be perceived by the patient if other areas of the brain are involved, evolving into complex somatosensory disorder.[3]Alice in Wonderland syndrome has been attributed to the migrainous cortical dysfunction of the non-dominant parietal lobe.[4]

Studies have shown that electrical stimualtion of the parietal lobe leads to distortion in the size and length of the image perceived.Decreased perfusion to the non-dominant parietal lobe during an attack leads to perception of symptoms.[5]Usually symptoms of AIWS can precede or accompany a migrane attack.

Alice 2.jpg

Symptoms

  1. Metamorphopsia - visual distortions
  2. Macropsia - seeing images larger than normal
  3. Micropsia – seeing images smaller than normal
  4. Achromatopsia – inability to perceive color
  5. Teleopsia – seeing farther than normal
  6. Pelopsia – seeing closer than normal
  7. Visual hallucinations
  8. Partial/total body macro/micro-matognosia
  9. Quick-motion phenomenon
  10. Dysmorphopsia – lines and countours appearing wavy
  11. Feeling of derealisation, depersonalization, somatopsychic duality
  12. Alteration in judgement of time
Distortion of self-image as shown in the book "Alice in wonderland"

Micropsia and Telopsia are the most common symptoms.

Proposed classification of symptoms in AIWS[3]

Types Obligatory symptoms Facultative symptoms
A Aschematia:partial or total macrosomatognosia or microsomatognosia; paraschematia Derealization, depersonalization, somatopsychic

duality, aberration in judgement of time

B Macro- and micropsia and/or tele- and pelopsia.When micropsia and telopsia appear at the same time and for the same object:porropsia

Lilliputianism (people appearing smaller)

C Type A + type B symptoms

Clinical diagnosis

Diagnostic criteria for migraine associated with AIWS[6]

  1. One or more episodes of self-experienced body schema illusion or metamorphopsia
  2. Duration < 30 min
  3. Accompanied by headache or a history of migraine
  4. RMI, CSF, and EEG all normal (visual evoked potentials may be abnormal)

Lanska and Lanska proposed that Alice in wonderland syndrome can be divided into Pure Alice in wonderland(Inter-personal) and Alice in wonderland like syndrome(Extra-personal), latter being more common.[7]

Diagnostic procedures

In diagnostics,[8] MRI and EEG are usually not that helpful

Treatment

The condition as such is self-limiting[9]. There is no active intervention protocol as of now. Treating migraines can alleviate AIWS symptoms. Antipsychotics have also been tried.

Prognosis

AIWS has no age predilection,but is most commonly seen in children and adolescents.Prognosis usually depends upon the underlying mechanism and should be treated appropriately. Though being self limiting,long term follow-up is required.

References

  1. Losada-Del Pozo R, Cantarín-Extremera V, García-Peñas JJ, et al. [Characteristics and evolution of patients with Alice in Wonderland syndrome]. Rev Neurol. 2011;53(11):641-648.
  2. Brodsky MC. Pediatric Neuro-Ophthalmology. 2nd ed. New York: Springer-Verlag; 2010. https://www.springer.com/gp/book/9780387690698. Accessed September 22, 2019.
  3. 3.0 3.1 Mastria G, Mancini V, Viganò A, Di Piero V. Alice in Wonderland Syndrome: A Clinical and Pathophysiological Review. BioMed Research International. doi:10.1155/2016/8243145
  4. Evans RW, Rolak LA. The Alice in Wonderland Syndrome. Headache. 2004;44(6):624-625. doi:10.1111/j.1526-4610.2004.446013.x
  5. Hiemer S, Schoettler A, Kluger G, et al (1998): Gibt es das Alice-im Wunderland-Syndrom. In: Kohlschuetter A (ed.) Bentele KHP (1922): Aktuelle Neuropaediatrie. Novartis Pharma Verlag, Nuernberg, pp. 425–42.
  6. Valença MM, de Oliveira DA, Martins HA de L. Alice in Wonderland Syndrome, Burning Mouth Syndrome, Cold Stimulus Headache, and HaNDL: Narrative Review. Headache. 2015;55(9):1233-1248. doi:10.1111/head.12688
  7. DJ LJ and L. Alice in Wonderland Syndrome: somesthetic vs visual perceptual disturbance. - PubMed - NCBI. https://www.ncbi.nlm.nih.gov/pubmed/23446681. Accessed September 22, 2019.
  8. Liu AM, Liu JG, Liu GW, Liu GT. “Alice in wonderland” syndrome: presenting and follow-up characteristics. Pediatr Neurol. 2014;51(3):317-320. doi:10.1016/j.pediatrneurol.2014.04.007
  9. Beh SC, Masrour S, Smith SV, Friedman DI. Clinical characteristics of Alice in Wonderland syndrome in a cohort with vestibular migraine. Neurology: Clinical Practice. 2018;8(5):389-396. doi:10.1212/CPJ.0000000000000518