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QUOTE FOR THE WEEKEND:

Cotard delusion, also known as or nihilistic delusion or walking corpse syndrome or Cotard’s syndrome , is a rare mental disorder in which the affected person holds the delusional belief that they are already dead, do not exist, are putrefying, or have lost their blood or internal organs.

Healthline.com

Cotard’s syndrome

Cotard delusion, also known as or nihilistic delusion or walking corpse syndrome or Cotard’s syndrome , is a rare mental disorder in which the affected person holds the delusional belief that they are already dead, do not exist, are putrefying, or have lost their blood or internal organs.

Signs and Symptoms: 

One of the main symptoms of Cotard delusion is nihilism. Nihilism is the belief that nothing has any value or meaning. It can also include the belief that nothing really exists. People with Cotard delusion feel as if they’re dead or rotting away. In some cases, they might feel like they’ve never existed.

While some people feel this way about their entire body, others only feel it in regard to specific organs, limbs, or even their soul.

Depression is also closely related to Cotard delusion. A 2011 review of existing research about Cotard delusion notes that 89% of documented cases include depression as a symptom.

Other symptoms include:

  • anxiety
  • hallucinations
  • hypochondria
  • guilt
  • preoccupation with hurting yourself or death

Researchers aren’t sure what causes Cotard delusion, but there are a few possible risk factors. Several studies indicate that the average age of people with Cotard delusion is about 50. It can also occur in children and teenagers. People under the age of 25 with Cotard delusion tend to also have bipolar depression. Women also seem to be more likely to develop Cotard delusion.

In addition, Cotard delusion seems to occur more often in people who think their personal characteristics, rather than their environment, cause their behavior. People who believe that their environment causes their behavior are more likely to have a related condition called Capgras syndrome. This syndrome causes people to think their family and friends have been replaced by imposters. Cotard delusion and Capgras syndrome can also appear together.

Other mental health conditions that might increase someone’s risk of developing Cotard delusion include:

  • bipolar disorder
  • postpartum depression
  • catatonia
  • depersonalization disorder
  • dissociative disorder
  • psychotic depression
  • schizophrenia

Cotard delusion also seems to be associated with certain neurological conditions, including:

  • brain infections
  • brain tumors
  • dementia
  • epilepsy
  • migraines
  • multiple sclerosis
  • Parkinson’s disease
  • stroke
  • traumatic brain injuries

QUOTE FOR FRIDAY:

“Stone Man Syndrome. Extremely rare disease of connective tissue. A mutation of the body’s repair mechanism causes fibrous tissue (muscle, tendon, ligament) to be ossified when damaged. In some cases, injuries can cause joints to become permanently frozen in place. The gene that causes ossification is normally deactivated after a fetus bones are formed in the womb, but in patients with FOP, the gene keeps working.”

MAYO CLINIC

Fibrodysplasia Ossificans Progressiva/Stone Man Syndrome

Fibrodysplasia ossificans progressiva (FOP) 

It is a disorder in which skeletal muscle and connective tissue, such as tendons and ligaments, are gradually replaced by bone (ossified). This condition leads to bone formation outside the skeleton (extra-skeletal or heterotopic bone) that restricts movement.

This process generally becomes noticeable in early childhood, starting with the neck and shoulders and moving down the body and into the limbs. People with FOP are born with abnormal big toes (hallux valgus) which can be helpful in making the diagnosis. Trauma, such as a fall or invasive medical procedure, or a viral illness may trigger episodes of muscle swelling and inflammation (myositis). These flareups lasts for several days to months and often result in permanent bone growth in the injured area.

FOP is almost always caused by a mutation at the same place in the ACVR1 gene (The ACVR1 gene provides instructions for making the activin receptor type-1 (ACVR1) protein, which is a member of a protein family called bone morphogenetic protein (BMP) type I receptors.)  and is inherited in an autosomal dominant manner. This condition occurs in about 1 in 1,600,000 newborns and about 800 people worldwide are known to have FOP.

SIGNS AND SYMPTOMS

-Fibrodysplasia ossificans progressiva (FOP) is characterized by the gradual replacement of muscle tissue and connective tissue (such as tendons and ligaments) by bone, restricting movement. This process generally becomes noticeable in early childhood, starting with the neck and shoulders and proceeding down the body and into the limbs.

-The formation of extra-skeletal bone causes progressive loss of mobility as the joints become affected. Speaking and eating may also become difficult as the mouth becomes affected. Over time, people with FOP may become malnourished because of the inability to eat. They may also develop breathing difficulties as a result of extra bone formation around the rib cage that restricts expansion of the lungs.

-Any trauma to the muscles of an individual with FOP (a fall or an invasive medical procedure) may trigger episodes of muscle swelling and inflammation followed by more rapid ossification in the injured area. Flare-ups may also be caused by viral illnesses such as the flu.

-Affected individuals may also have short thumbs and other skeletal abnormalities.

Inheritance

-Fibrodysplasia ossificans progressiva is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder.

-Most cases of fibrodysplasia ossificans progressiva result from new mutations in the gene. These cases occur in people with no history of the disorder in their family. In only a small number of cases, an affected person has inherited the mutation from one affected parent.

How this disease is diagnosed:

-Making a diagnosis for a genetic or rare disease can often be challenging. Healthcare professionals typically look at a person’s medical history, symptoms, physical exam, and laboratory test results in order to make a diagnosis. The following resources provide information relating to diagnosis and testing for this condition. If you have questions about getting a diagnosis, you should contact a healthcare professional.

-The Genetic Testing Registry (GTR) provides information about the genetic tests for this condition. The intended audience for the GTR is health care providers and researchers. Patients and consumers with specific questions about a genetic test should contact a health care provider or a genetics professional.

TREATMENT:

There is currently no definitive treatment.  However, a brief course of high-dose corticosteroids, such as Prednisone, started within the first 24 hours of a flare-up, may help reduce the intense inflammation and tissue swelling seen in the early stages of fibrodysplasia ossificans progressiva.  Other medications, such as muscle relaxants, mast cell inhibitors, and aminobisphosphonates, if appropriate, should be closely monitored by a physician.  Surgery to remove heterotopic and extra-skeletal bone is risky and can potentially cause painful new bone growth.

References:

 

  • Fibrodysplasia ossificans progressiva. Genetics Home Reference (GHR). August 2007; http://ghr.nlm.nih.gov/condition/fibrodysplasia-ossificans-progressiva.
  • FOP Fact Sheet. International Fibrodysplasia Ossificans Progressiva Association. http://www.ifopa.org/what-is-fop/overview.html. Accessed 6/5/2014.
  • Pignolo R, Kaplan F. Pediatric Fibrodysplasia Ossificans Progressiva. E-medicine. July 30, 2009; http://emedicine.medscape.com/article/1007104-overview. Accessed 3/17/2011

 

QUOTE FOR THURSDAY:

“This syndrome can affect multiple senses, including vision, touch, and hearing. You may also lose a sense of time. Time may seem to pass faster or slower than you think.  Alice in Wonderland Syndrome primarily affects children and young adults. Most people grow out the disordered perceptions as they age, but it’s still possible to experience this in adulthood.”

Healthline  (healthline.com)

Part II Alice in Wonderland

Genetic and environmental influences

While there currently is no identified genetic locus/loci associated with Alice in Wonderland Syndrome, observations suggest that a genetic component does exist. AiWS does appear to be passed on from parent to child, with one case study showcasing a grandmother, mother, son, and daughter all with Alice in Wonderland Syndrome. In addition, there is an established hereditary trait of migraines. Examples of environmental influences on the incidence of AiWS include the use of the drug topiramate and potentially the dietary intake of tyramine. Further research is required to establish the genetic and environmental influences on Alice in Wonderland Syndrome.

Alice in Wonderland

Alice in Wonderland Syndrome was named after Lewis Carroll’s famous 19th-century novel Alice’s Adventures in Wonderland. In the story, Alice, the title character, experiences numerous situations similar to those of micropsia and macropsia. The thorough descriptions of metamorphosis clearly described in the novel were the first of their kind to depict the bodily distortions associated with the condition. Speculation has arisen that Carroll may have written the story using his own direct experience with episodes of micropsia resulting from the numerous migraines he was known to suffer from. It has also been suggested that Carroll may have suffered from temporal lobe epilepsy.

Gulliver’s Travels

Alice in Wonderland Syndrome’s symptom of micropsia has also been related to Jonathan Swift’s novel Gulliver’s Travels. It has been referred to as “Lilliput sight” and “Lilliputian hallucination”, a term coined by British physician Raoul Leroy in 1909, based on the small people that inhabited the island of Lilliput in the novel.

Etiology

Complete and partial forms of the Alice in Wonderland syndrome exist in a range of disorders, including epilepsy, intoxicants, infectious states, fevers, and brain lesions. Furthermore, the syndrome is commonly associated with migraines, as well as the use of psychoactive drugs. It can also be the initial symptom of the Epstein–Barr virus (see mononucleosis), and a relationship between the syndrome and mononucleosis has been suggested. Epstein-Barr Virus appears to be the most common cause in children, while for adults it is more commonly associated with migraines.

Cerebral hypotheses

AiWS can be caused by abnormal amounts of electrical activity causing abnormal blood flow in the parts of the brain that process visual perception and texture. Nuclear medical techniques using technetium, performed on patients during episodes of Alice in Wonderland syndrome, have demonstrated that AiWS is associated with reduced cerebral perfusion in various cortical regions (frontal, parietal, temporal and occipital), both in combination and in isolation. It has been hypothesized that any condition resulting in a decrease in perfusion of the visual pathways or visual control centers of the brain may be responsible for the syndrome. For example, one study used single photon emission computed tomography to demonstrate reduced cerebral perfusion in the temporal lobe in patients with AiWS. Other theories exist that suggest the syndrome is a result of unspecific cortical dysfunction (e.g. from encephalitis, epilepsy, decreased cerebral perfusion), or reduced blood flow to other areas of the brain.  Other theories suggest that disordered body image perceptions stem from within the parietal lobe. This has been demonstrated by the production of disturbances of body image through electrical stimulation of the posterior parietal cortex. Other researchers suggest that metamorphopsias may be a result of reduced perfusion of the non-dominant posterior parietal lobe during migraine episodes.

Throughout all the neuroimaging studies, several cortical regions (including the temporoparietal junction within the parietal lobe, and the visual pathway, specifically the occipital lobe) are associated with the development of Alice in Wonderland syndrome symptoms.

Migraines

The role of migraines in Alice in Wonderland syndrome is still not understood, but both vascular and electrical theories have been suggested. For example, visual distortions may be a result of transient, localized ischaemia (an inadequate blood supply to an organ or part of the body) in areas of the visual pathway during migraine attacks. In addition, a spreading wave of depolarization of cells (particularly glial cells) in the cerebral cortex during migraine attacks can eventually activate the trigeminal nerve’s regulation of the vascular system. The intense cranial pain during migraines is due to the connection of the trigeminal nerve with the thalamus and thalamic projections onto the sensory cortex. Alice in Wonderland syndrome symptoms can precede, accompany, or replace the typical migraine symptoms.

Diagnosis

Alice in Wonderland syndrome is a disturbance of perception rather than a specific physiological change to the body’s systems. The diagnosis can be presumed when other causes have been ruled out and if the patient presents symptoms along with migraines and complains of onset during the day (although it can also occur at night). As there are no established diagnostic criteria for Alice in Wonderland syndrome, there is likely to be a large degree of variability in the diagnostic process and thus it is likely to be poorly diagnosed.

Prognosis

Whatever the cause, the bodily related distortions can recur several times a day and may take some time to abate. Understandably, the person can become alarmed, frightened, and panic-stricken throughout the course of the hallucinations—maybe even hurt themselves or others around them. The symptoms of the syndrome themselves are not harmful and are likely to disappear with time. The outcome is typically not harmful, especially in children, and most patients outgrow these episodes. The long-term prognosis typically depends on the root cause of the syndrome, and it is the underlying condition which must be evaluated and treated. Often, the difficulty lies within the patient’s reluctance to describe their symptoms out of fear of being labeled with a psychiatric disorder.

Treatment

At present, Alice in Wonderland syndrome has no standardized treatment plan. Often, treatment methods revolve around migraine prophylaxis, as well as the promotion of a low tyramine diet. Drugs that may be used to prevent migraines include: anticonvulsants, antidepressants, calcium channel blockers, and beta blockers. Other treatments that have been explored include repetitive transcranial magnetic stimulation (rTMS). Further research is required to establish an effective treatment regime.

Epidemiology

The lack of established diagnostic criteria or large-scale epidemiological studies on Alice in Wonderland syndrome means that the exact prevalence of the syndrome is currently unknown. One study on 3,224 adolescents in Japan demonstrated the occurrence of macropsia and micropsia to be 6.5% in boys and 7.3% in girls, suggesting that the symptoms of AiWS may not be so rare.

It appears that the male/female ratio is dependent on the age range being observed. Studies showed that younger males (age range of 5 to 14 years) were 2.69 times more likely to experience AiWS than girls of the same age, while there were no significant differences between students of 13 to 15 years of age. Conversely, female students (16- to 18-year-olds) showed a significantly greater prevalence.

The average age of the start of Alice in Wonderland syndrome is six but it is very normal for some to experience the syndrome from childhood to their late 20’s. It is also thought that this syndrome is hereditary because many parents who have AiWS report their children having it as well.

QUOTE FOR WEDNESDAY:

“Alice in Wonderland syndrome is one of the most fascinating neurological symptoms described in the medical literature. Estimated to occur among about 10-20% of the population, Alice in Wonderland syndrome is an infrequent event that is believed to occur only a few times throughout the lives of most affected individuals. It is an experience that people can describe with varying levels of detail, the consistent feature being a fleeting sense of dystrophism without associated long-term or short-term disability.”

NeurologyTimes (neurology.com)

Alice in Wonderland

Alice in Wonderland Syndrome (AiWS), also known as Todd’s syndrome or dysmetropsia, is a disorienting neuropsychological condition that affects perception. People may experience distortions in visual perception such as micropsia (objects appearing small), macropsia (objects appearing large), pelopsia (objects appearing to be closer than they are), or teleopsia (objects appearing to be further away than they are). Size distortion may occur in other sensory modalities as well.

The syndrome is sometimes called Todd’s syndrome, in reference to an influential description of the condition in 1955 by Dr. John Todd (1914-1987), a British Consultant Psychiatrist at High Royds Hospital at Menston in West Yorkshire. Todd discovered that several of his patients experienced severe headaches causing them to see and perceive objects as greatly out of proportion. They have altered sense of time and touch, as well as distorted perceptions of their own body. Although having migraine headaches, none of these patients had brain tumors, damaged eyesight, or mental illness that could have caused similar symptoms. They were also all able to think lucidly and could distinguish hallucinations from reality, however, their perceptions were skewed.

Since Lewis Carroll had been a well-known migraine sufferer with similar symptoms, Todd speculated that Carroll had used his own migraine experiences as a source of inspiration for his famous 1865 novel Alice’s Adventures in Wonderland. Carroll’s diary reveals that in 1856 he consulted William Bowman, an eminent ophthalmologist, about the visual manifestations of the migraines he regularly experienced. Since Carroll had these migraine symptoms for years before writing Alice’s Adventures, it seemed reasonable that Carroll had used his experiences as inspiration.

AiWS is often associated with migraines, brain tumors, and psychoactive drug use. It can also be the initial symptom of the Epstein–Barr Virus (see mononucleosis). AiWS can be caused by abnormal amounts of electrical activity resulting in abnormal blood flow in the parts of the brain that process visual perception and texture.

Anecdotal reports suggest that the symptoms are common in childhood, with many people growing out of it in their teen years. It appears that AiWS is also a common experience at sleep onset and has been known to commonly arise due to a lack of sleep.

Signs and symptoms

AiWS is often associated with migraines. AiWS affects the sense of vision, sensation, touch, and hearing, as well as one’s own body image. Nausea, dizziness, and agitation are also commonly associated symptoms with Alice in Wonderland Syndrome.

Individuals with AiWS can experience hallucinations or illusions of expansion, reduction or distortion of their own body image, such as microsomatognosia (feeling that their own body or body parts are shrinking), or macrosomatognosia (feeling that their body or body parts are growing taller or larger). These changes in perception are collectively known as metamorphosias, or Lilliputian hallucinations.

People with certain neurological diseases have experienced similar visual hallucinations. These hallucinations are called “Lilliputian”, which means that objects appear either smaller or larger than reality.

Patients may experience either micropsia or macropsia. Micropsia is an abnormal visual condition, usually occurring in the context of visual hallucination, in which the affected person sees objects as being smaller than they are in reality. Macropsia is a condition where the individual sees everything larger than it actually is.

One 17-year-old man described his odd symptoms by the following: “Quite suddenly objects appear small and distant or large and close. I feel as [if] I am getting shorter and smaller ‘shrinking’ and also the size of persons are not longer than my index finger (a lilliputian proportion). Sometimes I see the blind in the window or the television getting up and down, or my leg or arm is swinging. I may hear the voices of people quite loud and close or faint and far. Occasionally, I experience attacks of migrainous headache associated with eye redness, flashes of lights and a feeling of giddiness. I am always conscious to the intangible changes in myself and my environment”.

Although a person’s eyes are normal, they will often ‘see’ objects as the incorrect size, shape or perspective angle. Therefore, people, cars, buildings, houses, animals, trees, environments, etc., look smaller or larger than they should be. Further, depth perception can be altered whereby perceived distances are incorrect. For example, a corridor may appear to be very long, or the ground may appear too close.

Zoopsias is an additional hallucination that is sometimes associated with Alice in Wonderland Syndrome. Zoopsias involves hallucinations of either swarms of small animals (e.g. ants and mice etc.), or isolated groups of larger animals (e.g. dogs and elephants etc.). This experience of zoopsias is shared in a variety of conditions, such as delirium tremens.

The person affected by Alice in Wonderland syndrome may also lose a sense of time, a problem similar to the lack of spatial perspective. Time seems to pass very slowly, akin to an LSD experience. The lack of time and space perspective also leads to a distorted sense of velocity. For example, one could be inching along ever so slowly in reality, yet it would seem as if one were sprinting uncontrollably along a moving walkway, leading to severe, overwhelming disorientation.

Sufferers of Alice in Wonderland Syndrome can often experience paranoia as a result of disturbances in sound perception. This can include amplification of soft sounds or misinterpretation of common sounds.

In addition, some people may, in conjunction with a high fever, experience more intense and overt hallucinations, seeing things that are not there and misinterpreting events and situations. Less frequent symptoms sometimes described in Alice in Wonderland Syndrome patients include loss of limb control and dis-coordination, memory loss, lingering touch and sound sensations, and emotional instability.

It has been noted that patients are often reluctant to describe their symptoms due to fear of being labeled with a psychiatric disorder. It is usually easy to rule out psychosis as those with Alice in Wonderland Syndrome are typically aware that their hallucinations and distorted perceptions are not ‘real’, and they have not lost touch with reality. Furthermore, younger patients who frequently experience Alice in Wonderland syndrome may struggle to describe their unusual symptoms, and thus, it is recommended to encourage children to draw their visual illusions during episodes. It appears that the symptoms of AiWS do not change in severity over the course of the syndrome, and though the symptoms may acutely impact the patient’s life, Alice in Wonderland syndrome typically resolves itself within weeks or months. Furthermore, AiWS symptoms occur transiently during the day for short periods of time, with most patients describing their symptoms as lasting anywhere between 10 seconds to 10 minutes. This, combined with the typically short duration of the syndrome, suggests that Alice in Wonderland Syndrome typically causes a relatively short-term disruption of normal functioning. However, symptoms can be debilitating when experienced, and the individual should exercise caution, for example when driving, as the symptoms can appear rapidly. Symptom severity influences whether or not the individual will be able to hold a job during these periods of misperception.

Come back tomorrow for Part II on Alice in Wonderland!

 

QUOTE FOR TUESDAY:

“January is National Blood Donor Month, declared by President Richard Nixon in 1970, to pay tribute to voluntary blood donors and increase donation by others.  The Red Cross needs to collect more than 13,000 donations every day to keep the blood supply ready and available to meet the needs of about 2,600 hospitals, clinics and cancer centers across the country. They rely on voluntary blood donors.”

American Red Cross

Why we need blood donor’s!

 

Why do we need blood donor’s?

We need to make sure that we have enough supplies of all blood groups and blood types to treat all types of conditions.

By giving blood, every donor helps us meet the challenge of providing life-saving products whenever and wherever they are needed.

The American Red Cross states the following facts:

  • Every two seconds someone in the U.S. needs blood.
  • Approximately 36,000 units of red blood cells are needed every day in the U.S.
  • Nearly 7,000 units of platelets and 10,000 units of plasma are needed daily in the U.S.
  • Nearly 21 million blood components are transfused each year in the U.S.
  • The average red blood cell transfusion is approximately 3 pints.
  • The blood type most often requested by hospitals is type O.
  • The blood used in an emergency is already on the shelves before the event occurs.
  • It is estimated that sickle cell disease affects 90,000 to 100,000 people in the U.S. About 1,000 babies are born with the disease each year. Sickle cell patients can require frequent blood transfusions throughout their lives.
  • The number of whole blood and red blood cell units collected in the U.S. in a year: 13.6 million
  • The number of blood donors in the U.S. in a year: 6.8 million
  • Although an estimated 38 percent of the U.S. population is eligible to donate blood at any given time, less than 10 percent of that eligible population actually do each year.
  • Blood cannot be manufactured – it can only come from generous donors.
  • Type O negative blood (red cells) can be transfused to patients of all blood types. It is always in great demand and often in short supply. Type O is the “Universal Blood Donor”
  • Type AB positive plasma can be transfused, it’s the “Universal Blood Recepient”.

This allows pt’s diagnosed with this illness to experience:

  • Bleeding due to lack of platelets
  • shortness of breath due to lack of RBC’s carrying oxygen (02) to the tissues of the body.
  • Dizziness again due to lack of 02 carried to the brain with anemic, & bleeding causing your B/P to be low (orthostatic b/p-changing your position anemic and blood dropping from the brain to cause dizziness) also.
  • cognitive impairment due to lack of 02 to the brain since RBC count is low in the body.
  • Bruising due to low platelets.
  • petachiae (small red/purple spots on the skin)
  • susceptibility to infections

Conditions for needing blood donors:

Aplastic anemia occurs when bone marrow stops making enough blood forming stem cells. In all three blood lines; red blood cells, white blood cells and platelets, patients with aplastic anemia have a low blood count. The bone marrow is found to be aplastic which means there is a low growth of blood forming stem cells.

Cancer of all types –  Where with or without the treatments of chemo or radiation in the end cancer itself kills all good cells that are created by our bone marrow.  Chemo or radiation kill the bad cells = cancer cells and good cells = RBCs, WBCs, Platelets.  So blood transfusion commonly needed in cancer patients.

Anemia– Lack of RBCs in the body.

Conditions causing bleeding in the body like GI bleed, hemmoragic stroke, endometriosis, hemophilia, and simple patients in the OR that bleed and need blood transfusions in the OR and ICU and even possibly on the Med/Surg or Telemetry unit.  I could go on with types of conditions the deciding factor that makes the doctor order the blood transfusion is obviously heavy bleeding occurring right in front of the surgeon’s or ER MDs eyes or checking the Complete Blood Count called a CBC looking at the hemoglobin (Hmg)-think of it at the fluids in the bloodstream and looking at the Hematocrit (Hct)-think of it as the solids in the bloodstream and if the Hg is critical 6-7 than one or two blood transfusions are ordered.

Treatments:

  • Treatments vary on a case by case basis. Age is often the determining factor for which treatment to use. Stem cell transplantation may be used for individuals younger than 30 years and who have a matched sibling donor=Blood Donor Needed.
  • Stem cell transplantation is a procedure which replaces defective bone marrow with healthy cells. Around 80% of patients make a complete recovery using stem cell transplantation.
  • For older patients with aplastic anemia, immune suppressing therapy with anti-thymocyte globulin(ATG) and cyclosporin is typically used. Around 70-80% of aplastic anemia patients respond to this treatment.