Micropenia syndrome in children and adolescents: pathogenesis, clinical picture, diagnosis


Alice in Wonderland syndrome
Alice in Wonderland. 1865. Artist - John Tenniel.
MeSHD062026

Alice in Wonderland syndrome
(AIWS) is a disorienting neurological condition that manifests itself in a person’s impaired visual perception of his body or its individual parts, a violation of the “body diagram” (macrosomatognosia - the feeling of the body as larger) or microsomatognosia (as a smaller one)[1].
A person may also experience other sensations of size distortion, such as micropsia, macropsia, pelopsia or teleopsia. Size distortion in other sensory modalities may occur. The disorder's English name comes from Lewis Carroll's Alice's Adventures in Wonderland. The name of the syndrome was given by British psychiatrist John Todd (1914-1987), sometimes in honor of himself Alice in Wonderland syndrome is called Todd syndrome
[2]. In Russian psychiatric literature, a disorder of perception of one’s own body is called “violation of the body diagram” or “autometamorphopsia” (from ancient Greek αὐτός - oneself, μετά - between, μορφή - form, ὄψις - vision)[3].

Clinical picture

The subject perceives the apparent size and proportions of his body to be significantly smaller or larger than they actually are. In general, the body appears far away or extremely close at the same time. But, regardless of the terminology, the essence of the phenomenon is as follows: the eye is not damaged, the changes affect only the psyche.

Not only visual perception is affected, but also hearing, touch and also one’s own body image; the syndrome continues even when the eyes are closed [ source not specified 341 days

].
For most people, this happens before bed. Secondary features of the syndrome include restlessness, apraxia and agnosia [ source not specified 341 days
].

Sometimes the patient feels a clear disproportion of body parts[3]. For example, the torso reaches 100 meters, the legs extend to the center of the Earth, and the head becomes the size of an apple[3].

Feelings of changes in body schema can be isolated, or they can occur with other psychopathological manifestations[3]. However, this change in perception is often painful[3].

It is possible to correct the altered body diagram with vision: looking at himself in the mirror, he discovers the normal parameters of his head (although he feels it, for example, is 10 meters long), looking at his legs, a person is convinced that they are of normal size, and not many meters [3 ].

Who discovered Alice in Wonderland syndrome and what does Carroll have to do with it?

Alice in Wonderland syndrome is a neurological condition in which a person's perception of surrounding objects, space, or parts of his own body changes. This syndrome was first recorded by British psychiatrist John Todd in 1955, which is why it is sometimes called “Todd’s syndrome.” In Russian mental literature one can find the names “body diagram disturbance” or “autometamorphopsia” associated with this syndrome.


To describe the symptoms, Todd used an analogy with the heroine of a Lewis Carroll fairy tale, namely an episode where Alice either drinks from a bottle and becomes tiny, or eats a piece of pie and grows to huge sizes.

During Alice in Wonderland syndrome, a person's mental perception of his body is disrupted. Vision remains fine; disturbances are associated only with the peculiarities of brain function at this moment.

Alice syndrome manifests itself in different ways, here is what a person in this condition may experience:

  • macrosomatognosia, when one’s own body or part of it begins to seem disproportionately huge to a person. To correct this condition, it is sometimes advised to go to a mirror - in it a person sees that his size has remained the same, although he feels like a giant;
  • microsomatognosia. It's the opposite effect of making yourself look tiny;
  • micropsia. Feeling that objects around are larger than they should be;
  • macropsia, in which everything around seems small;
  • teleopsia, when the sense of depth of space increases, and objects located nearby seem very far away.

Most often, people who experience Alice in Wonderland syndrome experience it before bed, and it is accompanied by anxiety and disorientation.


Here's how the heroine of the Insider article describes Alice in Wonderland syndrome:

My eyes were starting to feel strange, like they were being squeezed. Suddenly I noticed that the room around me began to seem as if I was looking at it from the wrong end of a telescope. My bedroom furniture, posters and decorations, and walls seemed tiny, as if they were the size of a dollhouse. At the same time, I felt huge, as if I could reach the ceiling with my fingertips.

This happened to me often when I was a child, most often when I was particularly tired, sick or stressed. I didn’t have words to explain what happened to me at night, I only knew that it was unusual and like a dream, but I understood that it was not a dream.

As I got older, the episodes became increasingly rare, and eventually I forgot they ever happened.

According to some assumptions, Carroll himself suffered from this syndrome as a child, which is why he came up with this episode in Alice's Adventures in Wonderland.

Notes

  1. AJ Larner.
    A Dictionary of Neurological Signs. - Springer, 2020. - P. 15. - ISBN 978-3-319-29821-4.
  2. 1 2 3 J. Murray Longmore, Murray Longmore, Ian Wilkinson.
    Oxford Handbook of Clinical Medicine. - OUP Oxford, 2010. - P. 708. - ISBN 978-0-19-923217-8.
  3. 1 2 3 4 5 6 7 V.V. Marilov.
    General psychopathology. - M.: "Academia", 2002. - P. 18. - 224 p. — ISBN 5-7695-0838-8.
  4. M. Cinbis, S. Aysun (1992). "Alice in Wonderland syndrome as an initial manifestation of Epstein-Barr virus infection." British Journal of Ophthalmology
    .
    BMJ. 76
    (5): 316–316. DOI:10.1136/bjo.76.5.316. ISSN 0007-1161. (English)
  5. E. Lahat, G. Eshel, A. Arlazoroff (1991). ""Alice in wonderland" syndrome: a manifestation of infectious mononucleosis in children." Behavioral neurology
    .
    4
    (3): 163–166. DOI:10.3233/BEN-1991-4304. ISSN 0953-4180. PMID 24487499. (English)
  6. Mastria G., Mancini V., Viganò A., Di Piero V. (2016). "Alice in Wonderland Syndrome: A Clinical and Pathophysiological Review". Biomed Res Int
    .
    2016
    . DOI:10.1155/2016/8243145. PMID 28116304. (English)

Alice in Wonderland syndrome: what is it and does it need to be treated?

In the fairy tale “Alice in Wonderland,” the main character experiences a variety of unusual sensations. Objects around her live their own lives: they shrink, grow, and change their speed of movement. However, some people are forced to live their entire lives “like in a fairy tale.” EtCetera looked into what Alice in Wonderland syndrome is and what danger it poses?

Alice in Wonderland syndrome is a rare neurological disorder characterized by a distorted perception of reality. The name of the syndrome is borrowed from the book of the same name by Lewis Carroll. As in a fairy tale, a person cannot understand what is really happening to him and what is imaginary to him.

Most often, Alice in Wonderland syndrome occurs in children aged 5 to 13 years , but can sometimes occur in adults.

The syndrome manifests itself in two main forms: macropsia and micropsia.


Micropsia is a mental condition in which objects appear smaller than they are. For example, a book may appear to the patient to be the size of a matchbox.

Macropsia is a mental condition in which objects appear much larger than they are. During attacks, objects “grow” right before the patient’s eyes.

WHAT IS THE DANGER? These hallucinations are perceived by all human senses, not just vision. Therefore, it is very difficult for the patient to cope with them and sensibly assess what is happening. That is why during attacks the world is full of dangers for such a person. Without outside help he will not even be able to cross the road.

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CAUSES. Alice in Wonderland syndrome is diagnosed both as an independent disease and as a symptom of other diseases, and as a consequence of drug use.

Doctors identify several main reasons that provoke the occurrence of this syndrome.

Migraine. Neurological diseases, which are accompanied by severe attacks of headache, are the main cause of the syndrome. There are opinions that Lewis Carroll described in his book his own hallucinations that appeared during migraine attacks.


Schizophrenia. This mental disorder is often accompanied by hallucinations and impaired perception of reality.

Mononucleosis. An acute infectious disease that affects the brain and affects its functioning.

Epilepsy. This neurological disorder, which causes sudden seizures, can also cause hallucinations.

Brain tumors. Neoplasms put pressure on certain areas of the brain, thereby disrupting its functioning. This may trigger visions.

Also, Alice in Wonderland syndrome can manifest itself when taking hallucinogenic drugs and using narcotic substances. Symptoms disappear after all decay products are removed, but can significantly “undermine” an unstable psyche.

SEIZURES. Attacks can last from a few seconds to several hours. Some patients may remain in this state for a week. As a rule, children do not experience fear when attacks occur. Objects that get smaller and larger do not frighten them, and sometimes even amuse them. Adults, on the contrary, tend to panic because they understand that what is happening is unnatural. Relatives need to surround the patient with care, show attention and patience.

TREATMENT. If the cause of the syndrome is another disease, then after its disappearance the attacks will pass.

When the syndrome manifests itself during migraine attacks, attention must be paid to prevention. The patient should normalize sleep and avoid stressful situations. It is also advisable to review your diet and exclude from your diet foods that can cause a migraine attack: chocolate, coffee, alcohol, cheese, citrus fruits.

Taking painkillers can reduce the duration and severity of the attack. In other cases, no “special” treatment is provided.

Pathophysiology

Alice in Wonderland syndrome is explained by functional and structural aberrations of the perceptual system. In general, central pathology is considered the most common cause. However, most features are attributed to centrally located neurons and even to cells that selectively respond to specific types of sensory input.

For example, area V4 of the extraordinary visual cortex responds selectively to color, whereas area V5 responds to motion. Both regions respond to shape and depth, but bilateral loss of V4 function results in achromatopsia (inability to see color).

Bilateral loss of V5 results in akineopsia (inability to see movement). A person is unable to visually perceive vertical lines (plagiopsia) or lines at other angles. Explained by the loss of function of neurons grouped in all horizontal layers of the visual cortex.

An example is complex types of prosometamorphopsia, in which human faces are perceived as animal faces.

Types of disease

Alice in Wonderland syndrome can manifest itself in two main forms, which are distinct from each other. There are two forms of the disease:

  • macropsia;
  • micropsia.

Patients with macropsia see surrounding objects, including parts of their own body, in an enlarged form. With micropsia, a completely opposite situation can be observed. Things seem tiny or toy-like to the patient. Disturbances in the perception of surrounding objects may be accompanied by a loss of the ability to distinguish the real sizes and proportions of things, as well as temporal and spatial disorientation. It becomes difficult for patients with such an unusual disease to exist in society, for example, to go out in public, communicate with friends, or simply make new acquaintances.

Hallucinations that occur in patients are perceived not only by the visual organs, but by the entire body. Therefore, it becomes difficult for them to navigate in space, understand the true dimensions of objects and take the necessary actions in a given situation. An ordinary day, filled with everyday problems, can turn into a real disaster for the patient. Those suffering from the syndrome cannot even cross the road at a traffic light without assistance, because they are not able to correctly assess the danger posed by cars approaching from both sides.

Origin of the term

There is also a distortion of the sense of time or a feeling of “scaling” of the environment. Episodes are mostly short, often less than an hour, occurring up to several times a day with unpredictable onset.

Essentially, patients suffering from AIWS experience distorted time, space and bodies. They feel that their bodies are changed in size and have visual hallucinations.


Figure 1 Alice experiences whole-body macrosomatognosia. Illustration by John Tenniel (1865)

The term "Alice in Wonderland syndrome" was coined in 1955 by British psychiatrist John Todd (1914-1987) to cover a group of disorders "... closely related to, but not limited to, migraine and epilepsy."

As suggested by Todd, the group included deralization, depersonalization, hyperscremation, hyposmetia, somatopsychic ambivalence, and illusory changes in the size, distance, or position of stationary objects in the visual field; illusory feelings of levitation; illusory changes in the passage of time.

By the way, Todd is not the first to describe these individual characteristics.


Figure 2. (A) Alice experiences partial macrosomatognosia, (B) Alice experiences whole-body microsomatognosia. Illustrations by John Tenniel (1890)

Many of them appeared in the literature on hysteria, in soldiers with occipital wounds after World War II. In 1933–1952, Coleman and Lippman compared these cases and experiences of Alice in Wonderland syndrome, although they did not turn the name into an eponym.

Lippmann was the first to suggest that the bodily changes experienced by Alice were inspired by body schema illusions that Lewis Carroll himself experienced.

Carroll (pseudonym of the British mathematician Charles Lutwidge Dodgson, 1832-1898) had migraines. His diaries indicate that the attacks were preceded by auditory phenomena.

An alternative hypothesis is that Dodgson may have experimented with magic mushrooms, fly agaric mushrooms. Todd chose a memorable nickname for a group of symptoms that had hitherto been described in isolation from each other.

Causes, signs

The reasons for the occurrence are still unknown. The symptoms of Alice in Wonderland syndrome are legion. They are classified into 8 main groups. One of these groups is “substance-induced,” known as hallucinogenic perceptual disorder (HPPD).

Includes perceptual impairments that occur during (or after stopping) the use of psychoactive substances, medications (for example, cough medicines).

The first symptom of Alice in Wonderland syndrome (AIWS) is altered body image. A person misunderstands the size of his parts. Most often, the head and arms appear disproportionate; in general, one perceives an increase rather than a decrease in their size.

The other most significant manifestation is that the patient does not accurately perceive the size of various other objects. A person loses his sense of time.

For him, time passes either slowly or too quickly. Some people experience severe hallucinations; can visualize things that are not there, get the wrong impression about situations and events. In addition, auditory and tactile perception is distorted.

Not many doctors know about the disorder. Common migraine (aura, visual disturbances, hemicranial headache, nausea, vomiting) is an important cause of AIWS.

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Temporal epilepsy is another causative factor. Brain tumors can cause temporary seizures. Alice syndrome is common among children. Chronic cases are completely untreatable.

A person suffering from the disorder may have distortions and hallucinations several times throughout the day. These manifestations are not harmful or dangerous and disappear over time.

In addition, there is a known connection with Lyme disease, mononucleosis, and H1N1 influenza infection.

Alice in Wonderland syndrome is a mysterious phenomenon

Alice in Wonderland ... It turns out that this is not only a fairy-tale phenomenon with which we are familiar thanks to the film of the same name, it is a real disease that belongs to the category of rare and mysterious. Another name for this syndrome is micropsia (macropsia). Its essence is that with this neurological condition, a person perceives reality in a distorted form. That is, objects in the perception of a person suffering from this disease are seen by him not as they really are. They appear either reduced (micropsia) or increased (macropsia) in size. It becomes difficult for patients to determine the true dimensions of things around them, as well as the distance to them, and disorientation occurs.

As in the fairy tale about Alice by Lewis Carroll, a person cannot understand what is a real event and what just seems to him. It is assumed that the author of this tale suffered from migraines, and before each attack he experienced micropsia. Children under the age of thirteen are most often affected by the disease. As you get older, attacks may weaken, occur less frequently, and even disappear completely.

CAUSES OF THE DISEASE: There are several main reasons that provoke the occurrence of this syndrome:

Migraine is the main cause of the syndrome. Characterized by regular, severe and painful attacks of headache. Schizophrenia is a polymorphic mental disorder associated with the breakdown of thinking processes, as well as emotional reactions. It is often accompanied by hallucinations and impaired perception of reality. Mononucleosis is an acute infectious disease that affects the lymphatic system and affects the functioning of the brain. In most cases, its causative agent is the Epstein-Barr virus. Epilepsy , a neurological disorder that manifests itself in the form of sudden seizures, is also a precipitating factor for micropsia (macropsia). Brain tumors. Against the background of tumors in the brain, unusual visions may appear, since the tumors put pressure on its areas, thereby disrupting its functioning. Also, Alice in Wonderland syndrome can occur when taking hallucinogenic drugs or using drugs.

TREATMENT: The disease is extremely rare and poorly understood. It is diagnosed after the patient complains. In order to differentiate the disease, electroencephalography and computed tomography are prescribed. The neurological manifestation of the disease is presented in the form of a disorder in the parietal region of the brain.

Unfortunately, at present there are no clear methods that provide a quick and unambiguous recovery. However, most often this disease is treated as migraine. Therapy is selected taking into account all the features of the syndrome. The duration of the attacks can be seconds, or they can last for several days. In medical practice, there have been several cases where the syndrome was observed for years. With this disease, the retina (the patient’s eyes) remains absolutely healthy, changes occur in the psyche, and the brain distorts the information received from the eyes.

As for measures to prevent this disease, it is important to adhere to a work and rest schedule, proper nutrition, and also, if possible, try to avoid stressful situations. Surround the patient with care and attention, be patient. A patient with this disease is prohibited from driving a car or swimming in water.

Thus, Alice in Wonderland syndrome is a rare, not fully understood disease that disrupts a normal lifestyle. When you detect the first signs of the disease, it is important to immediately consult a doctor to determine treatment tactics and minimize its relapses.

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Phenomenology

Over the past 60 years, symptoms of Alice syndrome have included 42 visual, 16 equivocal, and other nonvisual. They represent distortions of sensory perception rather than hallucinations or illusions.

Hallucinations are perceptions that occur in the absence of a corresponding stimulus from the outside world, for example, a voice heard in the absence of a sound source.

Illusions have a source in the external world that is (often fleetingly) misperceived or misinterpreted. For example, a curtain moving in the wind may be mistaken for an intruder.

Like illusions, distortions are based on sensory impressions, but are characterized by very specific changes in particular aspects of the sensory input pattern.

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For example, all straight lines are perceived as wavy (dysmorphopsia), vertical lines are slanted (plagiopia), stationary objects are moving (kinetopsia), or all eyes are unnaturally large (prosometamorphopsia).

Strikingly, micropisia and macropisia are the most frequently described (58.6% - 45.0% among all patients). This indicates that they are the most common types of distortion known and therefore the best studied.

The duration of symptoms is short, from minutes to several days; sometimes they persist for many years or for life. A characteristic feature is visual fixation on an object - metamorphopsia occurs after a few minutes. After this time delay, objects are perceived distorted. During it, the process of perception is impossible.

The phenomenon is explained as a sign of cerebral asthenopia, cerebroasthenic syndrome (Unusual fatigue of the perceptual system).

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