Transcript Alice in Wonderland Syndrome

Patient #1: “Lewis”
• Previously well 11-year-old boy
• Sudden onset of strange sensations:
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he was moving too fast
he was alternately very tall or shrinking
his left hand was huge
sounds were abnormally loud
Patient #1: “Lewis”
• During episodes, patient was alert, oriented
but frightened
• Episodes last approx 20 minutes and
occurred approx once per week for 1 month
Patient #1: “Lewis”
• No hx of seizures, h/a, psych, head trauma,
infection, anoxia
• No meds, street drugs
• FHx: migraine
• General, neuro and psych exams all normal
• EEG: normal
• Attacks aborted by ergotamine and caffeine
Patient #2: “Carole”
• 17-year-old white female, previously well
• Presented with distortion of size and shape
of objects seen at a distance.
• Associated with fatigue and sore throat.
• Episodes occurred q 30 min while awake
• PMx and FHx: unremarkable
• Meds: none, no hallucinogens
Patient #2: “Carole”
O/E:
• Generalized lymph node enlargement
• Spleen palpable 2 cm BCM
• Grayish pharyngeal exudate
• Neurology exam normal
• Ophthalmological exam normal
• Psychiatric evaluation normal
Patient #2: “Carole”
Labs:
• Mono-spot positive
• Heterophil agglutinin titer: 1:960
• WBC 18
• 30% atypical lymphs (Downey cells)
• ESR 12
• Liver enzymes normal
• Collagen disease w/u negative
EEG: normal
Patient #2: “Carole”
• Course: Patient treated with IV steroids.
Sore throat disappeared immediately, spleen
became nonpalpable within a week, lymph
nodes returned to normal size.
• Visual distortions gradually decreased over
time and disappeared spontaneously after 7
months.
Alice in Wonderland Syndrome
Or
Through Neurology Class and What
Alyson Found There
A. Shaw, PGY-2
CHEO, October 1999
Alice in Wonderland Syndrome
• Syndrome named for
Lewis Carroll’s
Alice’s Adventures in
Wonderland (1865)
• Protagonist, 10 yearold Alice, experiences
changes in body size
and perception as she
travels through
Wonderland.
Alice in Wonderland Syndrome
• First described by
Lippman in 1952
• Literary name
given by Todd in
1955
Lippman CW. J Nerv Ment Dis. 1952
Todd J. Can med assoc J. 1955
Clinical Picture
Complex symptoms which are frightening to those
affected:
• Bizarre perceptual distortions (metamorphopsia)
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Micropsia: objects or persons seem too small
Macropsia: objects or persons seem too large
Distorted distance of objects
Objects may appear elongated or swollen
Achromatopsia: disappearance of colour
Clinical Picture
• Bizarre perceptual distortions
(metamorphopsia) con’t:
– Palinopsia: preservation of visual images
– Optic allesthesia: false orientation of objects in
space
– Polyopia: one object appearing as two or more
– Inverted vision
– Illusional symptoms
Clinical Picture
In addition to metamorphosia, children have been
referred for:
• Irrelevant or incoherent speech (“Do cats eat
bats?”, Jabberwocky, poem parodies)
• Talking to themselves (“I give myself very good
advice, but I very seldom follow it.”)
• Feeling of levitation (running with the red queen)
Clinical Picture
• Impaired sense of passage of time
• Zooming of the environment
• Illusional symptoms (seeing wire as snake, rag as
a cat, baby as a pig)
• Feeling “detached” (“grin without a cat”, “off with
her head!”)
• Hyperaccusis, tinnitus
• Personality changes, crying easily (“pool of
tears”)
Irrelevant or Incoherent Speech
As Alice was falling down the rabbit hole:
Down, down, down. There was nothing else to do, so Alice
soon began talking again…Dinah, my dear! I wish you
were down here with me! [Dinah was the cat] There are no
mice in the air, I’m afraid, but you might catch a bat, and
that’s very like a mouse, you know. But do cats eat bats, I
wonder?” And here Alice began to get rather sleepy, and
went on saying to herself, in a dreamy sort of way, “Do
cats eat bats? Do cats eat bats?” and sometimes, “Do bats
eat cats?” for, you see, as she couldn’t anser either
question, it didn’t much matter which way she put it.
Alice in Wonderland Syndrome
• “I get all tired out
from pulling my head
down from the ceiling.
My head feels like a
balloon; my neck
stretches and my head
goes to the ceiling.”
Lippman 1952
Alice in Wonderland Syndrome
After finishing off the “EAT ME” cake:
“Curiouser and curiouser!” cried Alice (she was so
much surprised, that for the moment she quite
forgot how to speak good English.) “Now I’m
opening out like the largest telescope that ever
was! Goodbye, feet!” (for when she looked down
at her feet, they seemed to be almost out of sight,
they were getting so far off)…Just at this moment
her head struck against the roof of the hall: in fact
she was now rather more than nine feet high…
Alice in Wonderland Syndrome
“I feel as though my
body is growing larger
and larger until it
seems to occupy the
whole room.”
Lippman 1952
Alice in Wonderland Syndrome
Differential Diagnosis
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Migraine
Epilepsy
Cerebral lesions
Intoxication with hallucinogenic drugs
Hyperpyrexia
Schizophrenia
AWS in Juvenile Migraine
• AWS first described as hallucinations
pathognomonic of migraine
• Seen alone, considered an acephalic
migraine equivalent
• Acephalic migraines found in 2% of
migraineurs referred to pediatric neurology
• May also be headache prodrome
Shevell M. A guide to migraine equivalents. Contemporary Pediatrics. 1998
AWS in Juvenile Migraine
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Typically between ages 5 and 12 years
Auras last between 10 to 60 minutes
Not functionally disabling
Between episodes child is well
Usually personal or family hx of migraine
More Differential Diagnosis
Infectious encephalopathy
• Infectious mononucleosis
• Coxsackievirus B1
• Varicella
EBV Infection
• CNS involvment in 1-10% of cases
• Neurologic manifestations of EBV:
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Meningoencephalitis
Encephalitis
Guillain-Barre syndrome
Mononeuritis
Seizures
Chorea
Acute psychotic reaction
Liaw SB. Pediatric Neurology. 1991
AWS in EBV Infection
• First reported by Copperman SM, Clin
Pediatr 1977
• 3 patients ages 9, 17, and 18 years with
metamorphopsia present in 6-month period
• Dx’ed with infectious mononucleosis based
on positive clinical, hematologic, and
serologic findings.
AWS in EBV infection
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None had PMHx or FHx of migraine or sz
None had hallucinogenic drug exposure
EEGs all normal
Distorted perception improved with
resolution of underlying mononucleosis
• Suggests association with metamorphopsia
and infectious mono may not be infrequent
AWS in EBV Infection
Several other reports since Copperman’s:
• Sanguineti G. J Infect Dis. 1983
• Eshel G. Ped Infect Dis J. 1987
• Liaw SB. Pediatr Neurol. 1991
• Cinbis M, Aysun S. Brit J Ophthal. 1992
• Ho CS. Acta Pediatrica Sinica. 1992
– 10 children (3-10 years) with documented EBV
AWS and EBV Infection
• Metamorphopsia may appear before,
during, or after resolution of all clinical
symptoms
• No neurologic deficits observed
• EEG: Normal or parieto-temporal slow
wave activity
• CT: Normal
Lahat E et al. Bevioural Neurology. 1991
AWS and Coxsackievirus B1
• Frequent clinical features:
Biphasic fever
Vomiting
Headache
respiratory sx
diarrhea
abdo pain
• CNS involvement in 15-56%
Aseptic meningitis
Paralysis
Transverse myelitis
Peripheral neuritis
encephalitis
GBS
cerebellar ataxia
rash
AWS and Coxsackievirus B1
Wang SM, Pediatr Infect Dis J, 1996
• 4-year-old boy with distortion of body form,
bizarre behavior, irrelevant speech x 3 days
• Fever, cough, abdo pain, watery diarrhea
• O/E: mild fever, injected throat, nil else
• EEG: normal
• Labs: Coxsackie B1 in CSF and rectal swab
AWS and Varicella
Soriani S, Pediatr Infect Dis J, 1998
• 4 year-old girl c/o parents changing size and walls
closing down on her x 2 days
• PMX: uncomplicated chicken pox 2 weeks prior
• O/E: Normal except crusted varicella lesions
• CSF normal
• EBV and Coxsackievirus Ab titers negative
• EEG: bilateral posterior slowing
• Sx persisted x 3 weeks, EEG normal after 2 weeks
Other AWS Associations
• Takaoka K. Psychopathology. 1999.
– 46 year old man with regular consuption of
cough syrup containing dl-methylephedrine
hydrochloride
• Mizuno M, et al. Psychopathology. 1998.
– 56 year old man with metamorphosia and
distorted perception of time 3 weeks prior to
onset of depressive disorder
Pathophysiology
Not fully determined. Hypotheses:
• Vasoconstrictive phase of migraine
• In EBV: Encephalitis or encephalopathy localized
to the parietal lobe
• Variations in symptoms result from different areas
of the brain affected
– Formed visual hallucinations likely from temporal or
occipital lobe dysfunction
Golden G. Pediatrics, 1979.
Lahat E et al. Behavioural Neurology. 1991.
Pathophysiology
• Kuo YT, et al. Pediatric Neurology. 1998
– 4 children with Alice in Wonderland Syndrome
– No hx of migraine, epilepsy, hallucinogens, or
psychosis
– Normal CSF, EEG, neurol and ophthal exams
– SPECT brain scans during acute stage of AWS
– Hypoperfusion of temporal lobe, occipital lobe,
and perisylvian area associated with AWS
Pathophysiology
• Focal inflammation resulting from EBV or
other infections causes focal brain
parenchymal edema and dysfunction and at
the same time decreases the regional
cerebral blood flow.
Kuo YT, et al. Cerebral Perfusion in Children With Alice in Wonderland
Syndrome. Pediatr Neurol 1998;19:105-108.
Treatment and Prognosis
• Course is self-limited and transitory
• Average duration of symptoms: 1 month
• Expect complete recovery without
neurologic sequelae
• No specific therapy during acute episodes
• Reassurance and understanding
• Treat the underlying condition
“The Rushes”
• A migraine variant with
hallucinations of time in
relation to both speech and
movement
• Named for “Alice” sequel,
Through the Looking
Glass and What Alice
Found There
Dooley J, Gordon K, Camfield P. Clinical
Pediatrics. 1990.
“The Rushes” (con’t)
“Alice never could quite make it out, in
thinking it over afterwards, how it was that they
began: all she remembers is that they were
running hand in hand, and the Queen went so fast
that it was all she could do to keep up with her:
and still the Queen kept crying “Faster! Faster!”
but Alice felt she could not go faster, though she
had no breath left to say so.
The most curious part of the thing was, that
the trees and the other things round them never
changed their places at all: however fast they
went, they never seemed to pass anything.”
Conclusions
• Awareness of AWS and common infections might
prevent incorrect diagnosis and treatment of other
conditions associated with AWS such as migraine,
epilepsy, or psychiatric condition.
• Associated with migraine, the various AWS auras
provide a valuable opportunity to learn about the
functional organization of the brain.
Conclusions
Lewis Carroll is a name that should be
familiar to pediatricians for his contribution
to an increasingly recognized pediatric
condition, his genuine devotion to children,
and as an illustration of the marriage of arts
and medicine.