Transient Episode of Alice in Wonderland Syndrome After Ventriculoatrial Shunt Revision

Transient Episode of Alice in Wonderland Syndrome After Ventriculoatrial Shunt Revision

Accepted Manuscript Transient Episode of Alice in Wonderland Syndrome after Ventriculoatrial Shunt Revision Pouya Entezami, MD, Alexandra Paul, MD, Ma...

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Accepted Manuscript Transient Episode of Alice in Wonderland Syndrome after Ventriculoatrial Shunt Revision Pouya Entezami, MD, Alexandra Paul, MD, Matthew A. Adamo, MD, Alan S. Boulos, MD PII:

S1878-8750(18)32337-4

DOI:

10.1016/j.wneu.2018.10.041

Reference:

WNEU 10483

To appear in:

World Neurosurgery

Received Date: 16 August 2018 Revised Date:

3 October 2018

Accepted Date: 4 October 2018

Please cite this article as: Entezami P, Paul A, Adamo MA, Boulos AS, Transient Episode of Alice in Wonderland Syndrome after Ventriculoatrial Shunt Revision, World Neurosurgery (2018), doi: https:// doi.org/10.1016/j.wneu.2018.10.041. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

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Pouya Entezami, MD Alexandra Paul, MD Matthew A. Adamo, MD Alan S. Boulos, MD

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Transient Episode of Alice in Wonderland Syndrome after Ventriculoatrial Shunt Revision

Corresponding Author:

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Pouya Entezami, MD Albany Medical College 47 New Scotland Ave, MC-10 Albany, NY 12208-3479 Phone: 518-262-5088 Fax: 518-262-5400

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Department of Neurosurgery, Albany Medical College, Albany, NY, USA

Key words: Alice in Wonderland; Hydrocephalus; Ventriculoatrial shunt; Somesthetic hallucinations; Visual misperceptions; Cerebrospinal fluid (CSF)

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Running head: Alice in Wonderland Syndrome

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Financial support: None

Manuscript Word Count: 1155 Abstract Word Count: 149 References: 10 Figures: 2

Disclosure The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

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ABSTRACT INRODUCTION: Visual misperceptions as a result of neurosurgical disorders are well documented. Alice in Wonderland Syndrome (AWS) is typically reported in neurological and

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psychiatric disorders. We present a patient who developed symptoms of AWS post-operatively.

CASE PRESENTATION: A 48-year-old male presented in shunt failure, attributed to a

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proximal catheter occlusion. Operative revision with replacement of the proximal catheter was performed without incident. Post-operatively he complained of visual disturbances, including the

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perception that people had small heads on little bodies. Symptoms resolved post-operatively.

DISCUSSION: The patient’s symptoms were diagnosed as a transient episode of AWS. This was attributed to manipulation of the parieto-occipital cortex during the revision. The local

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inflammatory response from manipulation of that area is thought to have caused our patient’s symptoms.

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CONCLUSION: Visual disturbances caused by lesions along the optic radiations are common findings for neurosurgery patients, though AWS following surgical intervention has not

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previously been reported.

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INTRODUCTION It is often said that the eyes are the windows to the soul. This is described further in the

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field of neuroscience, where the complex and intricate optic pathway – beginning with the eyes, transferring signal through the optic nerves, the optic chiasm, the lateral geniculate bodies, and the geniculocalcarine fibers into the occipital lobe – is one of the most direct and reliable

pathway have been correlated to myriad disease processes.

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external methods for physicians to assess the nervous system.3 Lesions or disturbances in this

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One rarely reported entity is known as Alice in Wonderland Syndrome (AWS), first described by in the 1950’s.5,10 Much like Lewis Carroll’s character Alice, who traversed a realm of peculiar perceptions, AWS is a term used to describe body schema illusions and metamorphosia (extra-personal visual illusions).1,7,8 Patients afflicted by disturbances of the

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former are categorized as having Type A, the latter as having Type B, and those suffering from both are placed into the Type C category of AWS.1,6,7,9 This syndrome is typically seen in association with migraine headaches and epilepsy,

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with profound disturbances in how one views both the world and themselves. Several other etiologies have also been named, most commonly Epstein-Barr virus (EBV) infections.1,6-8 Yet

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one thing remains synchronous among those with this diagnosis, which is a lack of a psychotic state.7,8 The involvement of the visual pathway has been confirmed by changes in visual evoked potentials in those experiencing an episode of AWS, with a return to baseline after symptom resolution.4 Despite the various etiologies previously reported, to our knowledge this is the first reported case secondary to surgical intervention.

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CASE PRESENTATION Our patient is a 48-year-old male with a history of cerebrospinal fluid (CSF) diversion

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who has undergone multiple shunt revisions due to hardware failure. These prior episodes and revisions did not result in any psychological disturbances, despite radiographic hydrocephalus and clinical deterioration. During his last admission the shunt was converted from a pleural into a

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ventriculoatrial shunt. Roughly one year after his last revision, he presented to the hospital in acute shunt failure, with headache, lethargy, and Perinaud’s syndrome – his usual shunt failure

exploration and revision of the shunt.

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symptoms. CT imaging revealed hydrocephalus (Figure 1a) and he was taken emergently for

Intraoperatively, he was found to have a proximal occlusion, and the proximal catheter was replaced via neuroendoscopic technique. There was no bleeding encountered, and the

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proximal catheter was removed without difficulty as it was not adherent to the parenchyma. The location of the catheter was unchanged, and the same tract was used. His symptoms of hydrocephalus resolved shortly after the operation and imaging showed resolution, with decrease

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in ventricular caliber back to his normal baseline (Figure 1b). However, shortly after waking up from anesthesia, he complained of new visual

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disturbances. He felt that his body was much larger than before, and he had significant difficulty picking up objects in front of him. In addition, he saw everyone – family, nurses, and doctors alike – as if they had small heads on full-size bodies (Figure 2). He was not significantly disturbed by his symptoms and was completely aware that these were visual symptoms and not a change in reality. In fact, after a few hours he found it comical. His symptoms had resolved by that evening, after roughly 12 hours.

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DISCUSSION Our patient had manipulation of the parieto-occipital cortex during revision of a

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ventricular catheter, using neuroendoscopy (Figure 1c). Not only is revising a proximal catheter a known irritant of the brain parenchyma, the use of neuroendoscopy in this manner requires significant irrigation. Though there are obvious benefits to using this technique, our patient

developed a transient episode of macrosomatognosia (feeling that his whole body was larger than

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it is in reality) and micropsia (seeing things smaller than they are).1,7,9 Due to the presence of

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both Type A and B symptoms, our patient falls into the category of Type C AWS. Of note, his EBV PCR was negative. In addition, it is important to distinguish these symptoms and AWS from acute psychosis or drug induced phenomena, which our patient was free from. He was not started on any new medications and was free from withdrawal from other substances including alcohol. He did not have any predisposing psychiatric history. Finally, there were no vital sign

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changes or other physiological deviations during this case, and the patient had not had visual disturbance events from similar anesthesia in the past.

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Another differential diagnosis for this episode of psychosis include the effects of ventriculomegaly on visual perception, though our patient’s symptoms began post-operatively

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after the hydrocephalus had radiographically resolved. Certainly the relatively quick resolution of symptoms in our patient does hint that the effects of increased intracranial pressure may have contributed to this episode of AWS. Several optic pathway locations along the cerebrum have been proposed as the offending area in AWS, though the exact location responsible is unclear. During development, individuals create a personal image by integrating visual and sensory information; it is thought that

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disturbances of this construct are involved in the development of AWS.1,7 The initial description of AWS by Todd in 1955 implicates the parietal lobe as the area of concern, due to its role in

and the associated visual cortex have been named as well.2,3,7,8

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integrating visual and somatosensory information.10 Several other areas along the visual pathway

Reports of soldiers suffering from similar visual disturbances following injuries to the occipital lobes are found in the literature, as well as more direct measures of brain activity in

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those with AWS symptoms.1 One study performed technetium-99m hexamethylpropyleneamine

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tomography (SPECT) studies on several patients with AWS, and found decreased cerebral perfusion along the visual cortex.3 Similar patterns have been seen in functional magnetic resonance imaging (fMRI), which though rarely done for AWS, have shown patients to have reduced activation in the primary and extrastriate visual cortical regions in the occipital lobes and

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increased activity in the parietal lobe.2,7,8

This case adds to the body of growing literature surrounding this symptomatology, as there is a tract along which offending surgical manipulation can be seen anatomically. Much like

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the reports of patients with visually identifiable lesions on imaging (including angiomas, tumors, and hemorrhages),1,7 this case shows a cortical location through the right parietal and occipital

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lobes where a catheter was passed. The amount of irrigation used to utilize this tract is another possible etiology for cortical irritation during this procedure. Similar to the reports of those with positive EBV, the inflammatory effect of the offending agent – in this case, the insertion of new ventricular catheter while utilizing irrigation through the neuroendoscope – was thought to have had an inhibitory effect on the optic pathway, resulting in disturbances in visual perception. The insertion of the new catheter in our patient directly affected the parieto-occipital area where visual and somatosensory information are united, likely resulting in a transient episode of AWS.

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As with other patients with this self-limited syndrome, our patient’s symptoms resolved without

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intervention.11

CONCLUSION

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Visual disturbances caused by lesions along the optic radiations are common findings for neurosurgery patients. AWS is an entity not previously reported following surgical intervention, and its prevalence is thought to be much higher than that which is reported. This case presentation is the first to describe the development of this syndrome following surgical

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intervention along the visual pathway. Though the authors have not identified an avoidable operative step, this case serves as a reminder of the fragility of cortical fibers, which – even in a transient fashion – can cause disturbing symptoms if offended. Further research is needed to

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directly pinpoint the location of lesions causing this syndrome.

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FIGURE LEGENDS Figure 1 – The patient presented to the hospital with symptoms of shunt failure, (a) confirmed

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on axial CT scan by the presence of hydrocephalus. After revision of the proximal catheter and valve, (b) the ventricles returned to his preoperative baseline. The position of the catheter (c) is

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through the right parietal lobe.

Figure 2 – Artist representation of the patient’s description of his symptoms. (Illustration by

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Matthew Adamo, MD).

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BIBLIOGRAPHY

6. 7. 8. 9. 10. 11.

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Weidenfeld, A., & Borusiak, P. (2011). Alice-in-Wonderland syndrome--a case-based update and long-term outcome in nine children. Childs Nerv Syst, 27(6), 893-896.

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3.

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2.

Blom JD: Alice in Wonderland syndrome: A systematic review. Neurol Clin Pract 6:259-270, 2016 Brumm K, Walenski M, Haist F, Robbins SL, Granet DB, Love T: Functional magnetic resonance imaging of a child with Alice in Wonderland syndrome during an episode of micropsia. J AAPOS 14:317-322, 2010 Kuo YT, Chiu NC, Shen EY, Ho CS, Wu MC: Cerebral perfusion in children with Alice in Wonderland syndrome. Pediatr Neurol 19:105-108, 1998 Lahat E, Berkovitch M, Barr J, Paret G, Barzilai A: Abnormal visual evoked potentials in children with "Alice in Wonderland" syndrome due to infectious mononucleosis. J Child Neurol 14:732-735, 1999 Lippman CW: Certain hallucinations peculiar to migraine. J Nerv Ment Dis 116:346351, 1952 Liu AM, Liu JG, Liu GW, Liu GT: "Alice in wonderland" syndrome: presenting and follow-up characteristics. Pediatr Neurol 51:317-320, 2014 Mastria G, Mancini V, Vigano A, Di Piero V: Alice in Wonderland Syndrome: A Clinical and Pathophysiological Review. Biomed Res Int 2016:8243145, 2016 O'Toole P, Modestino EJ: Alice in Wonderland Syndrome: A real life version of Lewis Carroll's novel. Brain Dev 39:470-474, 2017 Perdices M: The Alice in Worderland Syndrome. Neuropsychol Rehabil 28:189-198, 2018 Todd J: The syndrome of Alice in Wonderland. Can Med Assoc J 73:701-704, 1955

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ABBREVIATIONS Alice in Wonderland Syndrome – AWS

Computed tomography – CT Epstein-Barr virus – EBV functional magnetic resonance imaging – fMRI

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Polymerase chain reaction – PCR

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Cerebrospinal fluid – CSF

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Technetium-99m Hexamethylpropyleneamine Tomography – SPECT