Alice thinks she is having a stroke. the best method to use to diagnose a stroke would be a(n):

• Overview
• Symptoms and Causes
• Prevention

Stroke Patient Education

A stroke, or "brain attack," occurs when a blood vessel in the brain becomes blocked or bursts. The brain cannot store oxygen, so it relies on a network of blood vessels to provide it with blood that is rich in oxygen. A stroke results in a lack of blood supply, causing surrounding nerve cells to be cut off from their supply of nutrients and oxygen. When tissue is cut off from its supply of oxygen for more than three to four minutes, it begins to die.

Types of stroke

Strokes can appear as hemorrhagic strokes, ischemic strokes or transient ischemic attacks.

• Hemorrhagic stroke — This type of stroke takes place when a weakened blood vessel in the brain ruptures. A hemorrhage, or bleeding from the blood vessel, occurs suddenly. The force of blood that escapes from the blood vessel can also damage surrounding brain tissue. Hemorrhagic stroke is the most serious kind of stroke. About 13% of all strokes are hemorrhagic. There are two types of hemorrhagic strokes: intracerebral and subarachnoid. Intracerebral hemorrhages are more common and occur when a blood vessel in the deep tissue of the brain ruptures. Subarachnoid hemorrhages usually occur when an aneurysm (a blood-filled pouch ballooned out from an artery) ruptures and bleeds into the space between the brain and the skull. This type of hemorrhagic stroke is most often caused by high blood pressure.
• Ischemic stroke — This type of stroke occurs when a blood vessel in the brain develops a clot and cuts off the blood supply to the brain. A blood clot that forms in a blood vessel in the brain is called a “thrombus.” A blood clot that forms in another part of the body, such as the neck or lining of the heart, and travels to the brain is called an “embolus.” Blood clots often result from a condition called “atherosclerosis,” the build-up of plaque with fatty deposits within blood vessel walls. About 87% of all strokes are ischemic. Treatment for ischemic strokes depends on how quickly after the symptoms start the stroke victim arrives at the hospital. In eligible patients, a medication called tPA (tissue plasminogen activator) may be given. This medication works to dissolve the clot and help restore blood flow. In other patients, a stroke specialist may recommend a mechanical thrombectomy. This is where a specialized doctor threads a catheter through an artery in the groin up through the body to the brain and uses a clot retrieval device to grab the clot and pull it out.
• Transient ischemic attack (TIA) — A TIA should be treated as seriously as a stroke. A TIA has the same symptoms as a stroke, but they only last several minutes, or up to 24 hours. Unlike a stroke, a TIA does not kill the brain cells, so there is no lasting damage to the brain. A TIA is considered a serious warning sign of stroke. About 1 in 3 people who have a TIA will go on to have a stroke.

The effects of a stroke depend on the extent and the location of damage in the brain. Among the many types of disabilities that can result from a stroke are:

• Inability to move part of the body (paralysis).
• Weakness in part of the body.
• Numbness in part of the body.
• Inability to speak or understand words.
• Difficulty communicating.
• Difficulty swallowing.
• Vision loss.
• Memory loss, confusion or poor judgment.
• Change in personality; emotional problems.

Why does a stroke affect different parts of the body?

Nerve cells in the brain tissue communicate with other cells to control functions including memory, speech and movement. When a stroke occurs, nerve cells in the brain tissue become injured. As a result of this injury, nerve cells cannot communicate with other cells, and functions are impaired. If a stroke occurs on the right side of the brain, the left side of the body is affected, and vice versa.

If you want to prevent a stroke, you must understand the risk factors that lead to stroke as well as the strategies that are used to reduce stroke. Make sure that you know the warning signs. If you see stroke warning signs, call 9-1-1! Most of the stroke warning signs are painless:

• Sudden numbness or weakness of the face, arm or leg, particularly on one side of the body.
• Sudden difficulty understanding or speaking. May have either slurred speech or confused speech.
• Sudden difficulty seeing in one eye or both eyes.
• Severe dizziness and/or sudden loss of balance, coordination, or ability to walk
• Sudden and severe headache for no reason

Last reviewed by a Cleveland Clinic medical professional on 07/11/2018.

References

• Stroke - Critical Care Emergency Medicine (//0-accessemergencymedicine.mhmedical.com.library.ccf.org/content.aspx?bookid=522&Sectionid=41291783) Accessed 11/18/2021.
• Neurological Emergencies Diagnosis & Treatment: Pediatric Emergency Medicine (//0-accessemergencymedicine.mhmedical.com.library.ccf.org/content.aspx?bookid=1175&Sectionid=65111505) Accessed 11/18/2021.
• National Stroke Association (//www.stroke.org) Accessed 11/18/2021.
• Centers for Disease Control and Prevention: Stroke (//www.cdc.gov/stroke/) Accessed 11/18/2021.

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Figure 1. High signal intensity of the left parieto-occipital cortex on diffusion-weighted MRI (DWI; b = 1,000).

Figure 2. EEG showing periodic sharp-wave complexes over the left parieto-occipital region, suggestive of CJD.

What makes this case unique, is that our patient presented with a relatively large number of metamorphopsias (which, in the extant literature, is reported in only 15% of all clinical cases of AIWS) and that CJD was the most likely cause. Since a diagnosis of definite CJD can only be established post mortem by autopsy, it was unfortunate that we were unable to obtain autopsy consent. Still, MRI findings have been found to be positive in 83% of the cases of probable CJD (vs. in 98% of the cases of definite CJD) (12). Therefore, even though head MRI is not the gold standard, a diagnosis of CJD was still very likely in our patient, especially considering our additional neurological work-up, which failed to yield an alternative diagnosis that could explain the clinical picture, including its rapid decline and dramatic outcome.

Alice in Wonderland Syndrome

As described by John Todd in 1955, AIWS is characterized by perceptual distortions reminiscent of the visual distortions, time distortions, bodily changes, derealization, and depersonalization experienced by Alice during her stay in Wonderland, as described in Lewis Carroll's classic children's book Alice's Adventures in Wonderland (1, 13). Todd, and before him, Lipmann (14), suggested that Carroll–whose real name was Charles Lutwidge Dodgson–had found inspiration for these peculiar phenomena in perceptual distortions he had experienced himself in the context of migraine. Although this hypothesis was followed by several historical treatises alleging that migraine was indeed the most likely source of Dodgson's experiences (15, 16), others have put forward the equally tantalizing hypothesis that, in his case, it was rather epilepsy that caused them (17) or perhaps substance abuse (18). Although we will probably never know for sure what made Dodgson experience these symptoms, a careful reconstruction of his medical history indicates that their most likely source was infectious disease, from which the author suffered on numerous occasions from childhood onwards, literally until the last day of his life (19). It is very likely that Dodgson had experienced these distortions himself (rather than having based his heroine's experiences on third-hand information), with a whopping 13 different symptoms described throughout the book, long before Todd had come up with the concept of AIWS.

To understand what makes AIWS stand out from other perceptual syndromes and disorders, we need to realize that distortions differ from hallucinations and illusions in that they are neither newly formed percepts of something that is not there (hallucination), nor actual objects or scenes mistakenly judged to be something else (illusion). Instead, distortions are percepts, experienced by a waking individual, which are based on appropriate stimuli from the outside world, of which a highly specific aspect is altered in a consistent manner [(20); for definitions, see Table 1]. Today, some 55 different types of perceptual distortion are known that may occur in the context of AIWS (4). The ones most frequently reported are micropsia (seeing things smaller than they are), macropsia (seeing things larger than they are), teleopsia (seeing things further away than they are), and dysmorphopsia (seeing straight lines as wavy). The provision that such aspects are altered in a consistent manner, means that the distortion applies to anything within the patient's visual range. Thus, people suffering from plagiopsia will typically see all things as slanted, in the same direction, and under the same degree, while those with prosopometamorphopsia will typically see one particular alteration in all the faces they perceive, such as heavy eyebrows or one eye that is markedly abducted to the nose or human faces changing into dragon faces (21, 22). The nature of these distortions may vary over time, but once present, all things perceived appear as if distorted in a similar manner.

Table 1. Definitions of hallucination, illusion, and distortion [after (20)].

The distinction between hallucination, illusion and distortion is not merely an academic issue. On the contrary, their phenomenological differences are thought to reflect differences in underlying mechanisms. Pathophysiologically, perceptual distortions are attributed to structural or functional lesions of discrete parts of the perceptual network, such as area V4 in hyperchromatopsia and V5 in akinetopsia (23). There are eight known groups of underlying etiology, comprising infectious diseases (encephalitis), central nervous system (CNS) lesions (stroke, brain tumor), peripheral nervous system (PNS) lesions (eye disease, middle-ear disease), paroxysmal neurologic disorders (epilepsy, migraine), psychiatric disorders (depression, schizophrenia), medications, illicit substances, and a miscellaneous group which includes hypnagogia and sensory deprivation (4). Thus, so-called “clinical cases” of AIWS always warrant diagnostic work-up, including neurological and psychiatric consultation, head MRI and EEG, and, when indicated, other auxiliary investigations (e.g., ophthalmologic examination, ENT consultation). Although evidence-based treatments are as yet to be developed, it is good clinical practice to aim therapy at the (probable) underlying cause (4, 19).

Creutzfeldt-Jakob Disease

To our knowledge, AIWS has not been reported before in the context of CJD. With 1–1.5 cases per million population per year, CJD is an extremely rare disease that is fatal within a year in about 90% of those affected (24). The peak incidence is in the seventh decade, whereas younger (20–40 yrs) or older (>80 yrs) cases are much less common (25). CJD belongs to the transmissible spongiform encephalopathies or prion diseases. Prion diseases are characterized by the deposition of an abnormally misfolded isoform of the native prion protein, which is encoded by the prion gene (PRNP) on human chromosome 20. The mechanism for triggering this conformational change is unknown, but the accumulation of this abnormal prion protein leads to neuronal degeneration, astrocytic gliosis, and spongiform changes, accompanied by rapid cognitive decline, and ending in death. There are four known subtypes of CJD, comprising (i) sporadic CJD (sCJD), which is the most common form, accounting for approximately 85% of the cases; (ii) genetic CJD, which accounts for 10–15% of the cases and is associated with mutations of the prion gene (PRNP); (iii) the iatrogenic form of CJD, which accounts for approximately 1% of cases and is most frequently associated with prior treatment with human pituitary-derived hormones or human dura-mater grafts; and (iv) variant CJD (vCJD), which is a novel human prion disease found almost exclusively in the UK, and has been linked to the consumption of beef products contaminated with the causative agent of bovine spongiform encephalopathy (BSE) or “mad cow disease.” Moreover, based on the initial clinical presentation, six phenotypes have been identified, called cognitive, visual (Heidenhain variant), affective, classic, atactic (Oppenheimer-Brownell variant), and indeterminate (26). The Heidenhain variant was first described during the 1920s, and named after the German physiologist and histologist Rudolf Heidenhain (1834-1897). Representing some 20% of all cases of sporadic CJD (26–28), it is characterized by isolated visual symptoms at disease onset, such as visual field defects and loss of visual acuity, which are considered to be reflections of early involvement of occipital cortex (29). Since these visual defects may precede cognitive and motor symptoms by several weeks, it is not uncommon that CJD is not even suspected in such cases, and that patients are initially referred to an ophthalmologist.

Conclusion

We conclude that the possibility of a Heidenhain variant of CJD, although extremely rare, needs to be entertained in the differential diagnosis of AIWS, especially in the presence of rapid cognitive decline. Moreover, we conclude that AIWS may be relatively harmless in the majority of cases, but that “clinical” cases always warrant a proper diagnostic work-up before this conclusion can be drawn.

Limitations

Our knowledge of the numerous causes and manifestations of AIWS is still in its infancy. The syndrome was first conceptualized in 1955, but only the past few years have witnessed a steady rise in the number of publications on this disparate group of phenomena. Since the area is still very much in flux, our theoretical reflections on AIWS, although state-of-the-art, must be considered preliminary in nature. Secondly, the fact that we present here the first case report on AIWS in the context of CJD does not necessarily imply that our patient was the first person to ever suffer from a these two rare conditions. Notably, the Heidenhain variant of CJD can be expected to present more frequently with metamorphopsias. Therefore, our inability to find any prior case descriptions of symptoms of AIWS in the context of CJD may well be due to underreporting rather than to an actual rarity of the combination. A third and final limitation is that we were unable to obtain autopsy consent, which would have been necessary for making a diagnosis of definite CJD.

Ethics Statement

Written consent to publish was obtained from the patient's family.

Contribution to the Field Statement

Alice in Wonderland syndrome (AIWS) is a rare neurological disorder characterized by distortions of visual perception, the body image, and the experience of time. People may see things smaller than they are, feel their body alter in size or experience any of the syndrome's numerous other symptoms. Since there are also many known causes of AIWS, diagnosis requires a thorough neurological work-up. In children, the most common cause is brain inflammation; in adults, it is migraine. In the medical literature no more than 180 individual patients have been described, 50% of whom recovered. However, large-scale population studies indicate that fleeting, transient symptoms of AIWS are experienced by up to 30% of all healthy individuals. Accordingly, AIWS is generally considered fairly harmless in nature. We present here the first case description of an elderly male who suffered from AIWS due to Creutzfeldt-Jakob disease (CJD), an extremely rare neurodegenerative disorder. Our patient rapidly developed a state of dementia, and died within 2 months after his visual symptoms had begun. We conclude that AIWS is not always harmless, and that CJD, although extremely rare, must be on doctors' minds when the visual symptoms typical of AIWS are accompanied by rapid cognitive decline.

Author Contributions

TN contributed to the conception and design of the work, and to the acquisition, analysis, and interpretation of data for the work, drafted and revised the work, gave final approval for the final version to be published, and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. BtM, and SvdW contributed to the conception and design of the work, and to the acquisition, analysis, and interpretation of data for the work, revised the work, gave final approval for the final version to be published, and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. JDB contributed to the conception and design of the work, and to the analysis and interpretation of data for the work, drafted and revised the work, gave final approval for the final version to be published, and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Conflict of Interest Statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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