Lewis Carroll's personality and the possibility of epilepsy.

Lewis Carroll's classic Alice in Wonderland describes Alice's fantastical experiences so similarly to the actual phenomenology of the eponymous syndrome, that it has been previously suggested that Carroll himself experienced it. The syndrome is mostly associated with migrainous aura, and naturally, Carroll was postmortemly "diagnosed" as a migraineur. However, when considering his unique personality profile, it appears that he might have had temporal lobe epilepsy.

A Twist in Perception: A Case of an Eight-Year-Old Female With Alice in Wonderland Syndrome.

The article aims to explore the challenges involved in diagnosing and managing Alice in Wonderland Syndrome (AIWS) in pediatric cases, focusing on an eight-year-old female with perceptual distortions affecting vision, hearing, and time perception. AIWS, a rare neurological phenomenon, manifests as distortions in the perception of the body and external stimuli. The lack of established diagnostic criteria, particularly in the pediatric population, complicates accurate identification. The presented case illustrates visual anomalies, auditory abnormalities, and tachysensia, emphasizing the multisensory nature of AIWS. The temporal association with underlying causes, such as migraines and viral infections, highlights the need for a comprehensive evaluation. The Acharya Vinoba Bhave Rural Hospital management approach involves a systematic assessment, identification of underlying chronic conditions, and targeted treatment. Migraine prophylaxis, utilizing prescription drugs and a low-tyramine diet, plays a central role. The limited use of antipsychotics underscores the neurological origin of AIWS. The article contributes valuable insights into pediatric AIWS, advocating for further research and awareness. The article also aims to highlight the lack of established diagnostic criteria for AIWS, particularly in the pediatric population, and to present a systematic management approach based on a specific case study. The multidisciplinary collaboration, regular follow-ups, and patient education constitute a comprehensive approach to enhance understanding and alleviate symptoms in AIWS cases.

Beyond Vertigo: Vestibular, Aural, and Perceptual Symptoms in Vestibular Migraine.

PURPOSE: To review the vestibular, aural, and perceptual symptoms of vestibular migraine (VM) that may present alongside vertigo. RECENT FINDINGS: Increased research attention to the wide spectrum of symptoms presenting in VM patients has improved understanding of this disorder, with recent identification of five different VM phenotypes. Research into the clinical overlap between VM and other chronic vestibular syndromes such as persistent postural-perceptual dizziness and mal-de-debarquement syndrome reveals a range of vestibular symptoms and hints at pathophysiological connections between migraine and vestibular dysfunction. Studies of migraine treatment for hearing loss suggest patients presenting with aural symptoms may have an underlying diagnosis of migraine and deserve a trial of migraine preventives. Research into the neurologic basis of the perceptual disorder Alice in Wonderland syndrome has revealed brain areas that are likely involved and may help explain its prevalence in VM patients. VM is a sensory processing disorder that presents with more than just vertigo. Understanding the range of potential symptoms improves diagnosis and treatment for migraine patients whose diagnosis may be missed when only the symptoms identified in the diagnostic criteria are considered.

Síndrome de Alicia en el País de las Maravillas secundario a infección por SARS-CoV-2 / Alice in Wonderland syndrome in SARS-CoV-2 infection

El síndrome de Alicia en el País de las Maravillas es un conjunto de trastornos complejos de la percepción visual con múltiples etiologías, siendo las infecciones las más frecuentes en Pediatría. Es un cuadro poco frecuente, pero es importante conocerlo por su carácter generalmente autolimitado. Presentamos el caso de un niño de 11 años que, a las 48 horas de infección por SARS-CoV-2, inició distorsión visual de la forma corporal, micropsias y macropsias. Se descartaron otras causas orgánicas, manteniéndose actitud expectante dada su evolución benigna. Se destaca la aparición de este síndrome en contexto clínico de infección por SARS-CoV-2, no habiendo sido descrita hasta ahora su asociación en la literatura. (AU)

The Alice in Wonderland syndrome is defined by the presence of visual perception disorders with several etiologies, being infectious the most frequent in pediatrics. It is a rare clinical condition, but it is important to recognize it because of the generally self-limited nature. We present an 11 years old child who presented visual body distortions, micropsia and macropsia 48 after SARS-CoV-2 infection. Other organic causes were discarded, maintaining an expectant treatment because of its benign evolution. It is noteworthy that this syndrome associated with SARS-CoV-2 infection has not yet been published. (AU)

[A case of Alice in Wonderland syndrome after Epstein-Barr virus (EBV) encephalitis: a mimicry of focal epileptic seizure].

A 30-year-old man who received infliximab for treatment of Crohn's disease developed Epstein-Barr virus (EBV) encephalitis, which responded well to therapy; however, he had left lower visual field loss following treatment. The patient noticed peculiar symptoms 9 months after recovery from encephalitis; objects in his view appeared smaller or larger than their actual size (micropsia/macropsia). Moreover, it appeared that objects outside moved faster or slower than their actual speed of movements and moving objects appeared as a series of many consecutive snap shots. His vision was blurred, and he had visual difficulties and a sensation that his body was floating. These symptoms mainly appeared following fatigue and persisted over approximately 10 years. Based on cerebrospinal fluid analysis, brain MRI, N-isopropyl-p-123I-iodoamphetamine with single photon emission computed tomography, fluorodeoxyglucose positron emission tomography, and electroencephalography, we excluded both recurrent encephalitis and focal epileptic seizures. By taking all symptoms and other evaluation findings into account, the patient most likely suffered from "Alice in Wonderland syndrome" which is primarily associated with cortical dysfunction in the right temporo-parieto-occipital area as the consequence of previous acute EBV encephalitis.

Two Cases of Alice in Wonderland Syndrome with a Right Occipital Lobe Lesion Caused by Isolated Cortical Venous Thrombosis.

Alice in Wonderland syndrome (AIWS) is extremely rare, occurring more often in young individuals than in older adults. Symptoms of this syndrome typically include an altered body image, size perception, and time perception. However, the pathophysiology and lesions responsible for this syndrome remain unclear. In most cases, specific lesions cannot be identified using computed tomography or magnetic resonance imaging. Two patients with isolated cortical venous thrombosis in the right occipital area experienced transient visual symptoms of AIWS. Furthermore, a literature search indicated that AIWS with visual distortions is associated with right occipital lobe lesions, supporting the findings of our study.

Neuroimaging markers of Alice in Wonderland syndrome in patients with migraine with aura.

Background: The Alice in Wonderland syndrome (AIWS) is a transient neurological disturbance characterized by sensory distortions most frequently associated with migraine in adults. Some lines of evidence suggest that AIWS and migraine might share common pathophysiological mechanisms, therefore we set out to investigate the common and distinct neurophysiological alterations associated with these conditions in migraineurs. Methods: We conducted a case-control study acquiring resting-state fMRI data from 12 migraine patients with AIWS, 12 patients with migraine with typical aura (MA) and 24 age-matched healthy controls (HC). We then compared the interictal thalamic seed-to-voxel and ROI-to-ROI cortico-cortical resting-state functional connectivity between the 3 groups. Results: We found a common pattern of altered thalamic connectivity in MA and AIWS, compared to HC, with more profound and diffuse alterations observed in AIWS. The ROI-to-ROI functional connectivity analysis highlighted an increased connectivity between a lateral occipital region corresponding to area V3 and the posterior part of the superior temporal sulcus (STS) in AIWS, compared to both MA and HC. Conclusion: The posterior STS is a multisensory integration area, while area V3 is considered the starting point of the cortical spreading depression (CSD), the neural correlate of migraine aura. This interictal hyperconnectivity might increase the probability of the CSD to directly diffuse to the posterior STS or deactivating it, causing the AIWS symptoms during the ictal phase. Taken together, these results suggest that AIWS in migraineurs might be a form of complex migraine aura, characterized by the involvement of associative and multisensory integration areas.

Alice in wonderland syndrome "through the looking-glass" in a rare presentation of non-convulsive status epilepticus in cerebral venous sinus thrombosis and COVID-19.

Alice in Wonderland Syndrome (AIWS) is a rare perceptual disorder, rarely associated with epileptic etiology. We report the case of a 23-year-old man with subacute onset of right peri-orbital headache and visual misperceptions consistent with AIWS Type B, who underwent laboratory tests, brain CT with venography, ophthalmic examination, and neurological assessment that turned out to be normal except for visuospatial difficulties and constructional apraxia. A nasopharyngeal SARS-CoV2 swab taken as screening protocol was positive. The EEG performed because of the persistence of AIWS showed a focal right temporo-occipital non-convulsive status epilepticus; a slow resolution of clinical and EEG alterations was achieved with anti-seizure medications. Brain MRI showed right cortical temporo-occipital signal abnormalities consistent with peri-ictal changes and post-contrast T1 revealed a superior sagittal sinus thrombosis, thus anticoagulant therapy was initiated. AIWS is associated with temporo-parieto-occipital carrefour abnormalities, where visual and somatosensory inputs are integrated to generate the representation of body schema. In this patient, AIWS is caused by temporo-occipital status epilepticus without anatomical and electroencephalographic involvement of the parietal region, consistent with the absence of somatosensory symptoms of the syndrome. Status epilepticus can be the presenting symptom of cerebral venous sinus thrombosis (CVST) which, in this case, is possibly due to the hypercoagulable state associated with COVID-19.

[Temporary and persistent visual phenomena in neurology]. / Passagere und anhaltende visuelle Phänomene in der Neurologie.

Temporary and persistent visual phenomena are a frequent reason for a neurological presentation. The diagnosis can usually be made with the help of a structured anamnesis with determination of the time of onset, the course and symptoms as well as the monocular vs. binocular manifestation. The visual aura in migraine is certainly the most frequent entity to be differentiated. In particular, persistent visual phenomena such as visual snow syndrome, hallucinogen persisting perception disorder and the Charles Bonnet syndrome (CBS) seem to be underdiagnosed in clinical practice for various reasons and are probably not that rare. Instrumental diagnostics are mostly used for exclusion diagnostics and are a component of a complete patient education, but can be indicative for certain questions (CBS, epilepsy). This article presents the most frequent visual phenomena from the neurological practice and their differential diagnoses, guided by a case history.

Functional connectivity alterations in migraineurs with Alice in Wonderland syndrome.

BACKGROUND AND PURPOSE: Alice in Wonderland syndrome (AIWS) is a neurological disorder characterized by erroneous perception of the body schema or surrounding space. Migraine is the primary cause of AIWS in adults. The pathophysiology of AIWS is largely unknown, especially regarding functional abnormalities. In this study, we compared resting-state functional connectivity (FC) of migraine patients experiencing AIWS, migraine patients with typical aura (MA) and healthy controls (HCs). METHODS: Twelve AIWS, 12 MA, and 24 HCs were enrolled and underwent 3 T MRI scanning. Independent component analysis was used to identify RSNs thought to be relevant for AIWS: visual, salience, basal ganglia, default mode, and executive control networks. Dual regression technique was used to detect between-group differences in RSNs. Finally, AIWS-specific FC alterations were correlated with clinical measures. RESULTS: With respect to HCs, AIWS and MA patients both showed significantly lower (p < 0.05, FDR corrected) FC in lateral and medial visual networks and higher FC in salience and default mode networks. AIWS patients alone showed higher FC in basal ganglia and executive control networks than HCs. When directly compared, AIWS patients showed lower FC in visual networks and higher FC in all other investigated RSNs than MA patients. Lastly, AIWS-specific FC alterations in the executive control network positively correlated with migraine frequency. CONCLUSIONS: AIWS and MA patients showed similar FC alterations in several RSNs, although to a different extent, suggesting common pathophysiological underpinnings. However, AIWS patients showed additional FC alterations, likely due to the complexity of AIWS symptoms involving high-order associative cortical areas.

Multisensory perceptual distortion including auditory distortions in Alice in Wonderland syndrome: a case report.

Alice in Wonderland syndrome (AIWS) is a rare perceptual disorder characterized mainly by perceptual distortions of visual objects and one's own body. While there are many case reports of visual and somatosensory distortions associated with AIWS, little is known about auditory distortion. Therefore, we present the case of a 22-year-old right-handed woman who described having auditory as well as visual and somatosensory distortion experiences and a family history of AIWS. The subject reported experiencing multisensory perceptual distortions, where she sees other people's faces as larger and hears their voices as louder at the same time. This particular case suggests that auditory distortion - which contributes to constructing the perception of the surrounding space and the body - may also be characterized as a perceptual symptom of AIWS.

Disfagia como síntoma de presentación de síndrome de Alicia en el País de las Maravillas / Dysphagia as a presenting symptom of Alice in Wonderland syndrome

Varón de 6 años que acude a consulta de Gastroenterología infantil por cuadro de disfagia y ansiedad asociada con la alimentación. Tras descartarse esofagitis eosinofílica y enfermedad por reflujo gastroesofágico es diagnosticado de disfagia fóbica. El cuadro clínico progresa y cursa con disfagia nocturna asociada a macropsias y micropsias. Finalmente, presenta clínica diurna. Se descartan trastornos epilépticos y tumorales del sistema nervioso central y se realizan potenciales visuales evocados con amplitudes gigantes, siendo diagnosticado de síndrome de Alicia en el País de las Maravillas. La clínica digestiva y alucinatoria mejora de forma espontánea hasta la resolución (AU)

A 6-year-old male attended the Pediatric Gastroenterology office for dysphagia and anxiety associated with feeding, once eosinophilic esophagitis and gastroesophageal reflux disease were ruled out, he was diagnosed with phobic dysphagia. The symptoms progressed to nocturnal dysphagia associated with macropsias and micropsias and finally he presented with daytime symptoms. Epileptic and tumoral disorders of the central nervous system were ruled out and visual evoked potentials with giant amplitudes were performed, finally diagnosing Alice in Wonderland Syndrome. The digestive and hallucinatory symptoms improved spontaneously until resolution. (AU)

Alice in Wonderland syndrome: a lesion mapping study.

BACKGROUND AND PURPOSE: Alice in Wonderland syndrome (AIWS) is a rare neurological disorder, characterized by an erroneous perception of the body schema or surrounding space. It may be caused by a variety of neurological disorders, but to date, there is no agreement on which brain areas are affected. The aim of this study was to identify brain areas involved in AIWS. METHODS: We conducted a literature search for AIWS cases following brain lesions. Patients were classified according to their symptoms as type A (somesthetic), type B (visual), or type C (somesthetic and visual). Using a lesion mapping approach, lesions were mapped onto a standard brain template and sites of overlap were identified. RESULTS: Of 30 lesions, maximum spatial overlap was present in six cases. Local maxima were identified in the right occipital lobe, specifically in the extrastriate visual cortices and white matter tracts, including the ventral occipital fasciculus, optic tract, and inferior fronto-occipital fasciculus. Overlap was primarily due to type B patients (the most prevalent type, n = 22), who shared an occipital site of brain damage. Type A (n = 5) and C patients (n = 3) were rarer, with lesions disparately located in the right hemisphere (thalamus, insula, frontal lobe, hippocampal/parahippocampal cortex). CONCLUSIONS: Lesion-associated AIWS in type B patients could be related to brain damage in visual pathways located preferentially, but not exclusively, in the right hemisphere. Conversely, the lesion location disparity in cases with somesthetic symptoms suggests underlying structural/functional disconnections requiring further evaluation.

Jean-Martin Charcot (1825-1893) and the "Alice in Wonderland syndrome".

The "Alice in Wonderland syndrome" (AIWS) is a neurological disorder characterized by altered body schema perception, visual, or somesthetic symptoms, which is frequently associated with migraine. In this article, we present the earliest known description of symptoms attributable to AIWS in the medical literature. During a lecture held on November 22, 1887, at the Salpêtrière, Jean-Martin Charcot (1825-1893) examined a patient with somesthetic symptoms (partial macrosomatognosia) in the context of migraine with aura. Although this condition was not known at the time, Charcot tried to provide an accurate semiological and nosographic framework of this case, attributing the complex of symptoms to migraine with aura and epilepsy with sensory symptoms. With intellectual honesty and clinical prudence, Charcot correctly pointed to a disturbance in the excitability of cortical areas responsible for processing and perceiving sensory stimuli.

On Perception and Consciousness in HPPD: A Systematic Review.

Hallucinogen-persisting perception disorder (HPPD) features as a diagnostic category in the DSM-5, ICD-11, and other major classifications, but our knowledge of the phenomenology of the perceptual symptoms involved and the changes in consciousness during the characteristic "flashbacks" is limited. We systematically evaluated original case reports and case series on HPPD to define its phenomenology, associated (psycho)pathology, and course. Our search of PubMed and Embase yielded 66 relevant publications that described 97 people who, together, experienced 64 unique symptoms of HPPD. Of these, 76% concerned symptoms characteristic of Alice in Wonderland syndrome, over 50% non-visual symptoms, and 38% perceptual symptoms not clearly linked to prior intoxication states. This is in contrast with the DSM-5 diagnostic criteria for HPPD. Even though less than half of the patients showed a protracted disease course of over a year, a third achieved remission. However, in patients with co-occurring depression (with or without anxiety) HPPD symptoms persisted longer and treatment outcomes were more often negative. Thus, unlike the acute stages of psychedelic drug intoxication, which may be accompanied by altered states of consciousness, HPPD is rather characterized by changes in the content of consciousness and an attentional shift from exogenous to endogenous phenomena. Since HPPD is a more encompassing nosological entity than suggested in the DSM-5, we recommend expanding its diagnostic criteria. In addition, we make recommendations for clinical practice and future research.

A century of prosopometamorphopsia studies.

Prosopometamorphopsia is an extremely rare disorder of visual perception characterised by facial distortions. We here review 81 cases (eight new ones and 73 cases published over the past century) to shed light on the perception of face gestalts. Our analysis indicates that the brain systems underlying the perception of face gestalts have genuine network properties, in the sense that they are widely disseminated and built such that spatially normal perception of faces can be maintained even when large parts of the network are compromised. We found that bilateral facial distortions were primarily associated with right-sided and bilateral occipital lesions, and unilateral facial distortions with lesions ipsilateral to the distorted hemifield and with the splenium of the corpus callosum. We also found tentative evidence for the involvement of the left frontal regions in the fusing of vertical hemi-images of faces, and of right parietal regions in the fusing of horizontal hemi-images. Evidence supporting the remarkable adaptability of the network comes from the relatively high recovery rates that we found, from the ipsilateral hemifield predominance of hemi-prosopometamorphopsia, and from a phenomenon called cerebral asthenopia (heightened visual fatigability) which points to the dynamic nature of compensatory mechanisms maintaining normal face perception, even in chronic cases of prosopometamorphopsia. Finally, our analysis suggests that specialised networks for the representation of face gestalts in familiar-versus-unfamiliar faces and for own-versus-other face may be present, although this is in need of further study.

Visual Perception in Migraine: A Narrative Review.

Migraine, the most frequent neurological ailment, affects visual processing during and between attacks. Most visual disturbances associated with migraine can be explained by increased neural hyperexcitability, as suggested by clinical, physiological and neuroimaging evidence. Here, we review how simple (e.g., patterns, color) visual functions can be affected in patients with migraine, describe the different complex manifestations of the so-called Alice in Wonderland Syndrome, and discuss how visual stimuli can trigger migraine attacks. We also reinforce the importance of a thorough, proactive examination of visual function in people with migraine.

Illusions, hallucinations, and visual snow.

Illusions and hallucinations are commonly encountered in both daily life and clinical practice. In this chapter, we review definitions and possible underlying mechanisms of these phenomena and then review what is known about specific conditions that are associated with them, including ophthalmic causes, migraine, epilepsy, Parkinson's disease, and schizophrenia. We then discuss specific syndromes including the Charles Bonnet syndrome, visual snow syndrome, Alice in Wonderland syndrome, and peduncular hallucinosis. The scientific study of illusions and hallucinations has contributed significantly to our understanding of how eye and brain process vision and contribute to perception. Important concepts are the distinction between topologic and hodologic mechanisms underlying hallucinations and the involvement of attentional networks. This chapter examines the various ways in which pathological illusions and hallucinations might arise in relation to the phenomenology and known pathology of the various conditions associated with them.

Síndrome de Alicia en el país de las maravillas secundario a consumo de montelukast. Presentación de caso / Alice in wonderland syndrome secondary to medication with montelukast. Case presentation

RESUMEN La condición neurológica definida por la aparición de alteraciones en la percepción, usualmente interpretada como fenómenos extraños de metamorfosis y despersonalización, se reconoce como síndrome de Alicia en el país de las maravillas. Se presenta el caso de una paciente femenina de 9 años de edad, con el diagnóstico de síndrome de Alicia en el país de las maravillas secundario a medicación crónica con montelukast. El diagnóstico del síndrome psiquiátrico se realizó teniendo en consideración los antecedentes patológicos personales y el examen físico. El síndrome de Alicia en el país de las maravillas tiene un carácter benigno, sumamente infrecuente, y aunque su etiología no es del todo conocida, su aparición como reacción adversa a medicamentos es una opción que debe ser siempre considerada por el médico actuante (AU).

ABSTRACT The neurological condition defined by the appearance of alterations in perception usually interpreted as strange phenomena of metamorphosis and depersonalization is recognized as Alice in wonderland syndrome. The case of a 9-year-old female patient is presented, with the diagnosis of Alice in Wonderland syndrome secondary to chronic medication with montelukast. The diagnosis of the psychiatric syndrome was made taking into account personal pathological history and physical examination. Alice in Wonderland syndrome has a benign, extremely rare character and although its etiology is not fully known, its appearance, as an adverse reaction to medications, is an option that should always be considered by the acting physician (AU).