What are the classic symptoms, physical examination findings, and supporting examinations required, such as Electroencephalogram (EEG), for Alice in Wonderland syndrome in children?

Alice in Wonderland Syndrome in Children

Classic Symptoms

Alice in Wonderland syndrome (AIWS) in children presents most characteristically with self-experienced paroxysmal body-image distortions—including alterations in the perceived size, shape, mass, or position of their own body—often accompanied by visual perceptual disturbances such as micropsia (objects appearing smaller) or macropsia (objects appearing larger). 1

Core Perceptual Distortions

• Body image disturbances occur in approximately 45% of pediatric cases, manifesting as distortions of the size, mass, shape, or spatial position of the patient's own body 2, 1
• Visual perceptual distortions are present in 90% of children, including micropsia and/or macropsia (objects appearing smaller or larger than actual size) 2
• Metamorphopsia (distortion of object shape) occurs in 85% of pediatric cases 2
• Displacement of objects in visual space affects 80% of children with AIWS 2
• Time distortions occur in 45% of cases, typically experienced as acceleration of time 2
• Derealization (sensation of unreality) is reported in 30% of pediatric patients 2

Episode Characteristics

• Episodes typically occur multiple times per day in 95% of affected children 2
• Individual episodes are brief, lasting less than 3 minutes in 90% of cases 2
• Onset is acute in 85% of pediatric cases and progressive in 15% 2
• The average age at diagnosis is 9.5 years (range 4-16 years) 2

Important Clinical Distinction

True AIWS requires body-image distortions; isolated visual perceptual disorders without self-perceived distortions of body size, shape, or form do not meet diagnostic criteria and should not be labeled as AIWS. 1 This distinction is critical because cases with isolated visual illusions differ by age and etiology from those involving somesthetic perceptual disorders 1.

Physical Examination Findings

The physical examination in children with AIWS is typically normal, as this is primarily a perceptual disorder without objective neurological deficits in most cases. 2

Essential Examination Components

• Complete neurological examination including cranial nerve assessment, motor and sensory function evaluation, and cerebellar function testing to exclude structural neurological pathology 3, 4
• Comprehensive ophthalmological assessment including visual acuity testing, binocular alignment evaluation, extraocular muscle function, and fundoscopic examination to rule out primary ophthalmological causes 3, 5
• Facial nerve function assessment using the House-Brackmann scale if any facial asymmetry is noted 4, 6
• Evaluation for signs of infection, particularly viral illness, as infectious etiologies (especially Epstein-Barr virus) account for approximately 45% of pediatric AIWS cases 2
• Assessment for migraine features, as migraine is the second most common etiology in children, accounting for approximately 40% of cases 2

Critical Pitfall to Avoid

Do not assume benign etiology based on normal physical examination alone—while 80% of pediatric AIWS cases resolve without recurrence, serious underlying conditions including encephalitis, epilepsy, and rarely progressive neurological diseases must be excluded through appropriate investigations 7, 8, 2.

Supporting Examinations Required

In clinical cases of AIWS, auxiliary investigations including blood tests, EEG, and brain MRI are strongly advised to identify the underlying etiology and exclude serious pathology. 7

Neuroimaging

• Brain MRI with contrast is the preferred initial imaging study to evaluate for intracranial pathology, encephalitis, or structural abnormalities 4, 6
• Neuroimaging was normal in all patients in one pediatric series of 20 cases, but this does not eliminate the need for imaging given the potential for serious underlying conditions 2
• MRI is superior to CT for detecting posterior fossa abnormalities and encephalitic changes 3

Electroencephalography (EEG)

• EEG should be performed in all children with AIWS to evaluate for epileptic activity or other electrophysiological abnormalities 7, 2
• EEG was abnormal in 55% of pediatric cases in one series: one patient showed an epileptic focus (left temporal), and 10 patients demonstrated posterior slow waves 2
• The sensitivity of EEG for predicting subsequent epilepsy is low (15% sensitivity, 33% positive predictive value), but abnormal findings may guide further evaluation 3

Visual Evoked Potentials

• Visual evoked potentials (VEPs) may show higher amplitude in children with AIWS, as demonstrated in 5 of 7 tested patients in one series 2
• VEPs can provide supportive evidence of altered visual processing but are not routinely required for diagnosis 2

Laboratory Investigations

• Serological testing for Epstein-Barr virus is essential, as EBV infection is the most common infectious cause of pediatric AIWS, accounting for 5 of 9 infectious cases in one series 2
• Complete blood count, inflammatory markers, and metabolic panel to evaluate for systemic infection or metabolic derangements 7
• Cerebrospinal fluid analysis should be considered if encephalitis is suspected, particularly with fever, altered mental status, or progressive symptoms 8

Etiological Distribution in Children

The principal etiologies in pediatric AIWS are:

• Infectious causes (45%), predominantly Epstein-Barr virus infection 2, 9
• Migraine (40%) 2
• Toxins (10%) 2
• Epilepsy (5%) 2

Prognosis and Follow-up

• 80% of pediatric AIWS cases do not have recurrence, indicating generally benign prognosis 2
• Treatment should be directed at the underlying condition (antiviral support for EBV, migraine prophylaxis for migraine-associated cases, antiepileptics for epilepsy) 7
• Reassurance that symptoms are not harmful suffices in approximately 50% of cases 7
• Close monitoring is essential as rare progressive conditions like Creutzfeldt-Jakob disease can present with AIWS, though this is exceedingly rare in children 8

When to Escalate Care

Immediate pediatric neurology referral is warranted if:

• Symptoms are progressive rather than episodic 8
• Cognitive decline, behavioral changes, or other neurological signs develop 8
• EEG shows epileptic activity requiring antiepileptic management 2
• MRI reveals structural abnormalities or encephalitic changes 7