Migrainous , Visual Snow, and “Alice in Wonderland” Syndrome in Childhood Reena Gogia Rastogi, MD,* Juliana VanderPluym, MD,† and Kara Stuart Lewis, MD, FAHS*

Migraine is a condition that is common in the pediatric and adolescent population. Among children with , visual aura can consist of either negative or positive features or both. Reports of sensory auras can also be elicited with a careful history. The under- standing of the types of aura, as well as their relation to the more typical features of migraine, are discussed. The similar phenomena of visual snow and Alice in Wonderland syndrome in children are also described in detail. Semin Pediatr Neurol 23:14-17 C 2016 Published by Elsevier Inc.

Introduction migraine, with little to no following. In many migrai- neurs, however, the visual aura is a consistent warning Migraine is a common disorder affecting approximately 6%- before the onset of the migraine pain, and can be used to 1 8% of children. Prepubertal patients have a different sex treat early and more efficiently. In pediatric patients, the predilection and may report different symptoms. Among ability of the child to reliably distinguish the symptoms and children with migraine, 54.1% describe having an aura of get to treatment quickly can be more of a challenge. which 71% are visual auras.1 Consequently, visual symp- toms with or without headaches are a common chief complaint in children. In the youngest ones, description of the aura may be difficult to elicit, and an understanding of Visual Snow the types of aura is helpful to the clinician caring for these Many children with migraine may present with visual aura, patients. Typical visual aura symptoms can have either as noted earlier. Prolonged or persistent visual symptoms, “ ” “ ” negative features such as vision loss or positive features however, are rare. Once the clinician has ruled out fl such as ashing, shimmering, or scintillating patterns. Visual structural, ophthalmologic, or epileptic etiologies, the differ- symptoms are the most common feature of the migraine ential diagnosis often includes conditions such as persistent aura, but others can involve sensory loss or tingling or a migraine aura without infarction, substance-induced (hallu- of heaviness of a limb. Some individuals can experi- cinogens), psychogenic, or malingering. Primary persistent ence both the visual and sensory auras within the same visual disturbance should be added to this differential as fi attack, and when this is the rst occurrence, they can be well. It is important to make the diagnosis of visual snow, as mistaken by many as a warning of a stroke. it is often refractory to treatment, so patient education Many aura symptoms are said to be dynamic, with a build regarding prognosis and setting realistic treatment expect- up in the intensity, developing gradually over 5-20 minutes ations is crucial. and lasting for less than 60 minutes. The headache with Visual snow is a disease of young adults with a reported migrainous features usually develops after the aura, or it can mean age of onset of 21 Ϯ 9 years and no obvious sex begin before the complete resolution of it. In some predilection.2 Despite few pediatric case reports,3 1-quarter individuals, the visual aura is the only symptom of the of the patients report visual snow for as long as they could remember (eg, from childhood).2 Consequently, it is From the nBarrow Neurological Institute at Phoenix Children’s Hospital, important for physicians taking care of pediatric patients University of Arizona College of Medicine, Phoenix, AZ. to be aware of this condition. † Department of Pediatrics, University of Alberta, Edmonton, Alberta, The literature on visual snow is scant but recent large Canada. fi Address reprint requests to Reena Gogia Rastogi, Divison of , studies have contributed a wealth of knowledge to this eld. Phoenix Children’s Hospital, 1919 E Thomas Rd, Ambulatory Building, A 3-step study examining patients with visual snow, which 3rd Floor, Phoenix, AZ 85016. E-mail: [email protected] involved a retrospective chart review (n ¼ 22), analysis of an

14 1071-9091/16/$-see front matter & 2016 Published by Elsevier Inc. http://dx.doi.org/10.1016/j.spen.2016.01.006 Syndromes in Childhood 15

Table “Visual Snow” Syndrome Criteria as Proposed by Schankin et al2 A. Visual snow: dynamic, continuous, tiny dots in the entire visual field lasting longer than 3 mo B. Presence of at least 2 additional visual symptoms from the 4 following categories: 1. . At least 1 of the following: after images (different from retinal after images) or trailing of moving objects 2. Enhanced entoptic phenomena. At least 1 of the following: excessive floaters in both eyes, excessive blue field , self-light of the eye, or spontaneous 3. 4. (impaired night vision) C. Symptoms are not consistent with typical migraine visual aura D. Symptoms are not better explained by another disorder

internet survey (n ¼ 235), and a prospective semistructured Visual snow could not be distinguished from persistent telephone interview (n ¼ 78), proposed criteria for visual migraine aura using diffusion-weighted and perfusion- snow syndrome (Table).2 The core symptom of primary weighted magnetic resonance imaging as there was no persistent visual disturbance is “visual snow.”4-6 Liu et al4 evidence of decreased water diffusion or cerebral perfusion 5 18 were the first to describe this phenomenon in 4 patients in either patient group. [ F]-2-fluoro-2-deoxy-D-glucose (patients 6-9) among a case series of 10 patients with positron emission tomography showed brain hypermetabo- persistent positive visual phenomena and migraine. Their lism in the right lingual gyrus and left cerebellar anterior neurologic and ophthalmologic examination were normal. lobe adjacent to the left lingual gyrus when comparing 17 Each described a chronic and persistent visual disturbance visual snow patients with 17 controls.7 Of note, there was a of their entire visual field resembling “television snow” or higher prevalence of migraine in the visual snow group, “TV static.”4 This classic and stereotypic description of which may have biased the results by showing an effect from continuous, dynamic tiny dots in the entire visual field, migraine rather than from visual snow.7 However, several similar to a poorly tuned analog television (TV), is the recent imaging studies in migraineurs did not show such hallmark feature of this evolving condition and is often interattacks hypermetabolism,9,10 suggesting that the find- volunteered by the patient without any prompting. With the ings in the lingual gyrus are likely because of visual snow advent of new technology (plasma and liquid crystal display alone. Given that the lingual gyrus is involved in visual TV, computers, and tablets), patient exposure to analog TVs memory11 and a number of higher-level visual functions and subsequently “TV static” or “TV snow” is rapidly including face analysis,12 hypermetabolism of the lingual declining. Consequently, this fitting analogy may become gyrus suggests that visual snow may be because of visual extinct and patients may have a more difficult time describ- postprocessing dysfunction.7 ing their visual disturbance. As such, patient illustrations of Visual snow is generally refractory to treatment. Therapy their symptoms are an invaluable and timeless tool. with a variety of abortive and prophylactic migraine treatments Visual snow is better viewed as a syndrome rather than an has been attempted,2-5 which despite providing relief for the isolated symptom. The study by Schankin et al2 identified a comorbid migraine headaches3 appearstohavenoeffecton number of associated visual symptoms: palinopsia (86%), the visual snow syndrome. Future research into pathophysi- enhanced entoptic phenomena such as floaters (81%) or ology and natural history of visual snow would hopefully blue field entoptic phenomenon (79%), photophobia (74%), provide insight into therapeutic strategies for this condition. and nyctalopia (68%). Nearly all patients reported at least 1 additional visual symptom and 92% had at least 3 additional visual symptoms. They also described common nonvisual Case Report symptoms, such as (62%). Migraine (59%) and migraine with aura (27%) are A.H. presented at 10 years of age with complaint that common comorbidities of visual snow.2 Historically, visual “things look small and far away” when she stares at them. snow was considered part of the spectrum of migraine aura This was episodic, but could occur 1-3 times a day. There but increasing evidence suggests that it is likely a unique were other times that she would describe “flashes in the entity associated with but not caused by migraine. Even the corner of her eye” for a few minutes. At this time, there were original description by Liu et al4 illustrated that although the no complaints of headaches, and she was seen first by an patients had a history suggestive of migraine, their visual ophthalmologist who found no ocular concerns. Mother had symptoms developed independently of the migraine events. a history of migraine with aura, and brought her to a child Comorbid migraine, however, can affect the presentation of neurologist. At the age of 12, she developed episodic visual snow, increasing the likelihood of having palinopsia, headaches with significant photophobia, but could not tie spontaneous photopsia, photophobia, and tinnitus.7 Clin- them temporally to the visual complaints. She underwent a ically, visual snow is very different from migraine aura, magnetic resonance imaging of the brain, which was which is commonly described as unilateral, movement of unremarkable. At the age of 13, her headaches had edges, positive and negative visual phenomena, and zigzag improved with the use of nortriptyline. She was able to lines.8 describe her sensations better and gave an example that she Download English Version: https://daneshyari.com/en/article/3090788

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