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Alice in Wonderland syndrome
ARTICLE IN PRESS a r c h s o c e s p o f t a l m o l . 2 0 1 4;x x x(x x):xxx–xxx
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Alice in Wonderland syndrome夽 V.M. Asensio-Sánchez Servicio de Oftalmología, Hospital Clínico Universitario, Valladolid, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
Case report: A case of Alice in Wonderland syndrome is described as the only sign of
Received 2 February 2011
Epstein–Barr virus infection.
Accepted 6 July 2012
Discussion: Epstein–Barr virus infection may include visual symptoms as the first or only
Available online xxx
signs of disease. All patients presenting with a clinical picture consistent with the Alice in Wonderland syndrome should undergo serological testing for Epstein–Barr virus infection.
Keywords:
˜ de Oftalmología. Published by Elsevier España, S.L. All rights © 2011 Sociedad Espanola reserved.
Epstein–Barr virus Infectious mononucleosis Visual illusions Metamorphopsia Alice in Wonderland syndrome
Síndrome de Alicia en el país de las maravillas r e s u m e n Palabras clave:
˜ Caso clínico: Se describe el caso de un varón de 19 anos con un síndrome de Alicia en el país
Virus de Epstein-Barr
de las maravillas como única manifestación de infección del virus de Epstein-Barr.
Mononucleosis infecciosa
Discusión: La infección por el virus de Epstein-Barr puede tener síntomas variados, y los
Ilusiones visuales
visuales pueden ser la primera o la única clínica de la enfermedad. A todos los pacientes
Metamorfopsia
con síndrome de Alicia en el país de las maravillas se les debería realizar una serología del
Síndrome de Alicia en el país de las
virus Epstein Barr.
maravillas
˜ de Oftalmología. Publicado por Elsevier España, S.L. Todos los © 2011 Sociedad Espanola derechos reservados.
Introduction The Epstein–Barr virus (EBV) is a gammaherpesvirus which infects a significant number of the population.1 The primary infection can be asymptomatic. In some cases it can evolve
to infectious mononucleosis (IM), characterized by the typical triad of fever, pharyngitis and lymphatic adenopathies, together with the presence of atypical lymphocytosis. Generally, infectious mononucleosis is a young people’s disease.1 The “Alice in Wonderland syndrome” (AIWS) is characterized by the presence of distortion in the bodily image and
夽 Please cite this article as: Asensio-Sánchez V.M. Síndrome de Alicia en el país de las maravillas. Arch Soc Esp Oftalmol. 2014. http://dx.doi.org/10.1016/j.oftal.2012.07.023 E-mail address: victor [email protected]
˜ 2173-5794/$ – see front matter © 2011 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L. All rights reserved. OFTALE-398; No. of Pages 2
ARTICLE IN PRESS 2
a r c h s o c e s p o f t a l m o l . 2 0 1 4;x x x(x x):xxx–xxx
Fig. 1 – Normal right eye optic coherence tomography. Fovea linear. Note the pigmentary-choriocapillary complex and the photoreceptor integrity line.
the perception of size, distance, form or spatial relationships of objects (metamorphopsia).2 Copperman described three patients with AIWS as a first expression of IM.3 This paper describes a young patient with AIWS without clinical signs or symptoms suggesting active infectious mononucleosis.
Clinic case A University student, 19, without relevant personal or familiar history, was referred to our practice by the primary health care doctor to discard possible central serous maculopathy. The patient referred that since 2 weeks he began to see “things smaller than usual and difficulty for reading because the lines appeared crooked” with both eyes, particularly with the right eye, during 5 min several times a day, “although now it lasts longer”. The patient was not on medical treatment and denied taking drugs or psychotropic preparations. Best corrected visual acuity in each eye was 1. Anterior and posterior pole examination, performed with Zeiss SL 115 and threemirror Goldmann lens, was normal. Visual field and color tests were normal in both eyes. Optic coherence tomography and macular scan centered on the fovea were performed with normal results (Fig. 1). Physical and neurological studies were also normal, as well as EEG. Cerebral and orbitary nuclear magnetic resonance did not identify pathologies. The physical exploration was normal, without megalia or adenopathy. Serological studies were performed, with positive IgM antibodies against the EBV viral capsid at 1:890, 2 weeks later at 1:1450 and 16 weeks later at 1:250. With this information, AIWS associated to EBV infection was diagnosed. Three months after the first visit the patient was asymptomatic.
Discussion EBV is broadly extended. It is estimated that 95% of adults between 35 and 40 years of age have been infected.1 EBV clinic
is broad and highly varied, including infectious mononucleosis, personality changes, depression, meningoencephalitis, mononeuritis, ataxia, convulsions, retinochoroiditis. In 1955, Todd described AIWS as a visual illusion (metamorphopsia) characterized by distortion in forms, movements, sizes and even colors.2 AIWS is a perceptual alteration syndrome which occurs in patients with epilepsy or migraines and in drug abusers. However, it has rarely been described in association with EBV.2,3 A recently published article related also with the H1N1 influenza.4 The patient described in this paper had no personal or familial history of epilepsy, migraine or drug abuse and the initial examination was not relevant as it had no signs or symptoms of active EBV infection, which was subsequently demonstrated serologically. The titles of 1:320 or higher indicate active infection. The virus capsid antigen abundantly expresses in the infection and induces a response of the IgM isotype in the primary disease which lasts 2–3 months. The duration of the visual illusion ranges between 2 weeks and 7 months but with complete recovery and all other described cases.2,3 Metamorphopsia has been described in retinal pathology and in occipital, occipito-temporal or occipito-parietales injuries, migraines, influenza processes, epilepsy, schizophrenia and psychotropic drugs.2 By means of functional magnetic resonance, Brumm et al.5 found in a 12-year-old patient a reduction in the activation of the primary visual cortex and increased activation in the parietal cortex during a AIWS episode. The case presented herein is another example of the variation in the onset symptoms of EBV infection. Accordingly, ophthalmologists must bear in mind the association of AIWS and EBV in patients with visual distortions as the only symptom because they could be considered as psychiatric patients and referred for inadequate treatment.
Conflict of interest No conflict of interest has been declared by the author.
references
1. Odumade OA, Hogquist KA, Balfour Jr HH. Progress and problems in understanding and managing primary Epstein–Barr virus infections. Clin Microbiol Rev. 2011;24:193–209. 2. Todd J. The syndrome of “Alice in Wonderland”. Can Med Assoc J. 1955;73:701–4. 3. Copperman SM. Alice in Wonderland syndrome as a presenting symptom of infectious mononucleosis in children: a description of three affected young people. Clin Pediatr. 1977;16:143–6. 4. Augarten A, Aderka D. Alice in Wonderland syndrome in H1N1 influenza: case report. Pediatr Emerg Care. 2011; 27:120. 5. Brumm K, Walenski M, Haist F, Robbins SL, Granet DB, Love T. Functional magnetic resonance imaging of a child with Alice in Wonderland syndrome during an episode of micropsia. J AAPOS. 2010;14:317–22.
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Alice in Wonderland syndrome夽 V.M. Asensio-Sánchez Servicio de Oftalmología, Hospital Clínico Universitario, Valladolid, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
Case report: A case of Alice in Wonderland syndrome is described as the only sign of
Received 2 February 2011
Epstein–Barr virus infection.
Accepted 6 July 2012
Discussion: Epstein–Barr virus infection may include visual symptoms as the first or only
Available online xxx
signs of disease. All patients presenting with a clinical picture consistent with the Alice in Wonderland syndrome should undergo serological testing for Epstein–Barr virus infection.
Keywords:
˜ de Oftalmología. Published by Elsevier España, S.L. All rights © 2011 Sociedad Espanola reserved.
Epstein–Barr virus Infectious mononucleosis Visual illusions Metamorphopsia Alice in Wonderland syndrome
Síndrome de Alicia en el país de las maravillas r e s u m e n Palabras clave:
˜ Caso clínico: Se describe el caso de un varón de 19 anos con un síndrome de Alicia en el país
Virus de Epstein-Barr
de las maravillas como única manifestación de infección del virus de Epstein-Barr.
Mononucleosis infecciosa
Discusión: La infección por el virus de Epstein-Barr puede tener síntomas variados, y los
Ilusiones visuales
visuales pueden ser la primera o la única clínica de la enfermedad. A todos los pacientes
Metamorfopsia
con síndrome de Alicia en el país de las maravillas se les debería realizar una serología del
Síndrome de Alicia en el país de las
virus Epstein Barr.
maravillas
˜ de Oftalmología. Publicado por Elsevier España, S.L. Todos los © 2011 Sociedad Espanola derechos reservados.
Introduction The Epstein–Barr virus (EBV) is a gammaherpesvirus which infects a significant number of the population.1 The primary infection can be asymptomatic. In some cases it can evolve
to infectious mononucleosis (IM), characterized by the typical triad of fever, pharyngitis and lymphatic adenopathies, together with the presence of atypical lymphocytosis. Generally, infectious mononucleosis is a young people’s disease.1 The “Alice in Wonderland syndrome” (AIWS) is characterized by the presence of distortion in the bodily image and
夽 Please cite this article as: Asensio-Sánchez V.M. Síndrome de Alicia en el país de las maravillas. Arch Soc Esp Oftalmol. 2014. http://dx.doi.org/10.1016/j.oftal.2012.07.023 E-mail address: victor [email protected]
˜ 2173-5794/$ – see front matter © 2011 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L. All rights reserved. OFTALE-398; No. of Pages 2
ARTICLE IN PRESS 2
a r c h s o c e s p o f t a l m o l . 2 0 1 4;x x x(x x):xxx–xxx
Fig. 1 – Normal right eye optic coherence tomography. Fovea linear. Note the pigmentary-choriocapillary complex and the photoreceptor integrity line.
the perception of size, distance, form or spatial relationships of objects (metamorphopsia).2 Copperman described three patients with AIWS as a first expression of IM.3 This paper describes a young patient with AIWS without clinical signs or symptoms suggesting active infectious mononucleosis.
Clinic case A University student, 19, without relevant personal or familiar history, was referred to our practice by the primary health care doctor to discard possible central serous maculopathy. The patient referred that since 2 weeks he began to see “things smaller than usual and difficulty for reading because the lines appeared crooked” with both eyes, particularly with the right eye, during 5 min several times a day, “although now it lasts longer”. The patient was not on medical treatment and denied taking drugs or psychotropic preparations. Best corrected visual acuity in each eye was 1. Anterior and posterior pole examination, performed with Zeiss SL 115 and threemirror Goldmann lens, was normal. Visual field and color tests were normal in both eyes. Optic coherence tomography and macular scan centered on the fovea were performed with normal results (Fig. 1). Physical and neurological studies were also normal, as well as EEG. Cerebral and orbitary nuclear magnetic resonance did not identify pathologies. The physical exploration was normal, without megalia or adenopathy. Serological studies were performed, with positive IgM antibodies against the EBV viral capsid at 1:890, 2 weeks later at 1:1450 and 16 weeks later at 1:250. With this information, AIWS associated to EBV infection was diagnosed. Three months after the first visit the patient was asymptomatic.
Discussion EBV is broadly extended. It is estimated that 95% of adults between 35 and 40 years of age have been infected.1 EBV clinic
is broad and highly varied, including infectious mononucleosis, personality changes, depression, meningoencephalitis, mononeuritis, ataxia, convulsions, retinochoroiditis. In 1955, Todd described AIWS as a visual illusion (metamorphopsia) characterized by distortion in forms, movements, sizes and even colors.2 AIWS is a perceptual alteration syndrome which occurs in patients with epilepsy or migraines and in drug abusers. However, it has rarely been described in association with EBV.2,3 A recently published article related also with the H1N1 influenza.4 The patient described in this paper had no personal or familial history of epilepsy, migraine or drug abuse and the initial examination was not relevant as it had no signs or symptoms of active EBV infection, which was subsequently demonstrated serologically. The titles of 1:320 or higher indicate active infection. The virus capsid antigen abundantly expresses in the infection and induces a response of the IgM isotype in the primary disease which lasts 2–3 months. The duration of the visual illusion ranges between 2 weeks and 7 months but with complete recovery and all other described cases.2,3 Metamorphopsia has been described in retinal pathology and in occipital, occipito-temporal or occipito-parietales injuries, migraines, influenza processes, epilepsy, schizophrenia and psychotropic drugs.2 By means of functional magnetic resonance, Brumm et al.5 found in a 12-year-old patient a reduction in the activation of the primary visual cortex and increased activation in the parietal cortex during a AIWS episode. The case presented herein is another example of the variation in the onset symptoms of EBV infection. Accordingly, ophthalmologists must bear in mind the association of AIWS and EBV in patients with visual distortions as the only symptom because they could be considered as psychiatric patients and referred for inadequate treatment.
Conflict of interest No conflict of interest has been declared by the author.
references
1. Odumade OA, Hogquist KA, Balfour Jr HH. Progress and problems in understanding and managing primary Epstein–Barr virus infections. Clin Microbiol Rev. 2011;24:193–209. 2. Todd J. The syndrome of “Alice in Wonderland”. Can Med Assoc J. 1955;73:701–4. 3. Copperman SM. Alice in Wonderland syndrome as a presenting symptom of infectious mononucleosis in children: a description of three affected young people. Clin Pediatr. 1977;16:143–6. 4. Augarten A, Aderka D. Alice in Wonderland syndrome in H1N1 influenza: case report. Pediatr Emerg Care. 2011; 27:120. 5. Brumm K, Walenski M, Haist F, Robbins SL, Granet DB, Love T. Functional magnetic resonance imaging of a child with Alice in Wonderland syndrome during an episode of micropsia. J AAPOS. 2010;14:317–22.