“Curiouser and Curiouser!”—Macroacusia and Visual Disturbances in Alice in Wonderland Syndrome
Alice in Wonderland syndrome (AIWS) is a disorder characterized by distortions of visual perception, the body schema, and experience of time, attributed to functional and structural aberrations of the perceptual system. This fascinating syndrome was named after “Alice's Adventures in Wonderland” (1865) by Lewis Carroll (Charles Lutwidge Dodgson [1832–1898]); in the story, the protagonist experiences a distorted perception of her body image. Visual distortions (metamorphopsias) are the commonest symptoms of AIWS, with micropsia and macropsia reported in 58.6 and 45.0% of patients, respectively. However, somesthetic and other nonvisual distortions may also occur. Although generally considered a rare entity, up to 30% of adolescents can experience occasional symptoms of AIWS. The etiology of AIWS is extremely heterogeneous: infectious encephalitis account for the majority of cases (21.6%) in patients aged ≤18 years, with the Epstein–Barr virus (EBV) accounting for 68.4% of cases due to encephalitis. Although full remission of symptoms occurs in 46.7% of cases, the prognosis can be worse in cases of encephalitis. Interestingly, in adults and elderly patients, similar visual disperceptions can occur after a strategic stroke of temporal and/or insular lobes and should be considered in differential diagnosis.
A 9-year-old previously healthy boy was referred to our clinic with complaints of distorted vision. He had episodes when he saw objects larger (macropsia) or smaller and farther away than they were (microtelepsia), with his vision fluctuating between these two conditions ([Fig. 1]). Sometimes he also perceived a prolongation of objects (palinopsia with illusory visual spread). These visual distortions had appeared a couple of weeks before, during a febrile illness with fatigue that spontaneously resolved over a few days. Symptoms had a rapid onset, lasted from some minutes to several hours. Initially, they had occurred several times per day but later reduced in frequency. Furthermore, he had episodes in which for some minutes he perceived a sudden increase in the volume of sounds (macroacusia). These perceptual distortion were not associated with headache. The patient had no family history of migraine and was taking no drug. Neurological and opthalmological examination reports were normal. Blood laboratory testing showed only a mild relative neutropenia (20.2%; reference values: 33.0–74.0%), lymphocytosis (66.2%; reference values: 22.0–51.0), and monocytosis (0.8%; reference values: 1.5–8.5%), with normal white blood cell count and inflammation parameters. Antistreptolysin-O titer was increased (435; reference value: 0–150). Serology for EBV, cytomegalovirus, rubella virus, Toxoplasma gondii, Borrelia burgdorferi, measurement of antitransglutaminase antibodies, and rheumatoid factor and novel coronavirus disease 2019 (COVID-19) serology testing yielded normal/negative results. Protein electrophoresis and immunofixation electrophoresis were also normal. A head magnetic resonance imaging (1.5-Tesla contrast-enhanced image including diffusion weighted imaging sequences) was entirely normal, and a continuous electroencephalogram including intermittent photic stimulation revealed no abnormality, even during the visual symptoms. Considering the spontaneous reduction in the frequency of symptoms, we refrained from performing a lumbar puncture and decided not to start a specific therapy. Symptoms gradually reduced in intensity and duration over the following 2 months and eventually disappeared.
In our patient, despite a comprehensive diagnostic workup, we did not identify a specific etiology for the AIWS. However, the symptom onset related to an acute febrile illness, the epidemiological context, mild blood abnormalities, benign course, and exclusion of other causes suggest an association with a viral infection (possibly due to influenza virus). Remarkably, this case manifested also with macroacusia, which has been exceptionally reported in AIWS; it confirms that perceptual distortion in AIWS can involve somesthetic and other nonvisual modalities. As Alice herself would have said, the more reports on its semiology appear, the more AIWS becomes “curiouser and curiouser.” Pediatricians and child neurologists should be aware of common and uncommon features of AIWS to ensure a correct initial diagnosis and perform an appropriate diagnostic workup.
05 October 2020 (online)
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