Grand Budapest Hotel: Inclusion Body Myositis

INCLUSIOTOSIS(IBM):

IBM is a rare inflammatory muscle disease that slowly progresses weakness and eventually the wasting away of both distal and proximal muscles. Muscles in the legs and arms are most obvious areas of the wasting away of muscle tissue. The disease slowly progresses and is diagnosed as a chronic, degenerative neuromuscular disease in which inflammatory cells invade muscle tissue causing progressive muscle weakness and wasting. IBM differs from other types of Myositis in that there is currently no known effective treatment for the disease. (B)

IBM is known to cause slow, progressive asymmetrical weakness and atrophy of the muscles of the wrists and fingers, muscles in the front of the thigh, and muscles that lift the front of the foot. Patients who have the disease can gradually lose the ability to walk, experience falls and injuries, lose hand function, and have swallowing difficulties. Some patients also experience muscle pain. The first muscles affected in Inclusion Body Myositis are usually those of the wrists and fingers, and the muscles at the front of the thigh. The muscles that lift the front of the foot also may be affected.Muscle weakness is often asymmetrical, more prominent on one side than the other. (B)

 TYPES OF IBMs:

There are two forms of IBMs- inflammatory and non-inflammatory. Inflammatory IBM is the most common form of IBM called 'Sporadic Inclusion Body Myositis' (sIBM). It is classified as an Idiopathic Inflammatory Myopathy, an inflammatory muscle disease with no known cause. It is also considered an Acquired Myopathy. This means that it is not caused by genetic inheritance.

It is the most common inflammatory muscle disease in adults older than 50 and is found in men more often than in women. Symptom onset occurs gradually, typically over a period of months or years, most often after the age of 50. It is not uncommon for people with IBM to become wheelchair bound within 10 to 15 years of disease onset. The cause of inflammatory myopathies is unclear. For some reason, in the case of sIBM, the body’s immune system turns against itself, referred to as an autoimmune response, and damages muscle tissue. The cause of the progressive muscle degeneration that occurs in IBM is unclear as well. (C)

Inflammatory cells invading muscle tissues is one characteristic of IBM, but the disease is distinct from other inflammatory myopathies in that muscle degeneration also occurs. Inclusion Body Myositis is named for the clumps of discarded cellular material — the “bodies” — that collect in the muscle tissues. These are apparent on the pathology reports for a muscle biopsy.

Inherited Inclusion Body Myositis (hIBM) is a disease which is genetically inherited, often appearing in other family members, and frequently starts with muscle weakness in early adulthood, occasionally as early as the teen years. This form of the disease is not categorized as an Acquired Myopathy or an Idiopathic Inflammatory Myopathy. The early symptoms include index finger weakness, difficulty walking, tripping and loss of balance. The disease typically progresses to the hands, shoulders, and neck. In this form of the disease, organs are rarely affected, nor are the muscles in the face and eyes, those used for breathing, digestion, and the heart. (C)

Each case of IBM may be quite different from others.  There have been cases where people originally diagnosed with Polymyositis (PM) but were not responding to standard treatments later found they actually had IBM or a non-inflammatory Myopathy such as a form of Muscular Dystrophy.the lack of attention given to neurological conditions and mental health, but it is important to reiterate the scale of the challenge cancer poses to health and social care.

Cancer remains the leading cause of death in the UK, and 2.5 million people are currently living with a cancer diagnosis; this is due to rise to 4 million by 2030. More people than ever before survive in the long term, but often in poor health, requiring extensive support for the rest of their life.ut it would be a mistake to address any long-term condition in isolation.

It is often not as clearcut as a person solely living with cancer or another serious illness. We estimate that currently 1.8 million people with cancer are living with at least one other long-term condition such as dementia or heart disease. Mental health is another important example of this; depression and anxiety often go hand in hand with serious illnesses including cancer.

STUDIES:

Participating in research studies is essential to discovering new, more effective tests and treatments for different conditions. It is very important to have knowledge of rare diseases like myositis and its forms. ( dermatomyositis, Inclusion-Body Myositis, Juvenile Myositis, Polymyositis). The different types of studies conducted on myositis are the following.

BYM338:

Novartis is enlisting sites to test this drug (which has been given breakthrough status) for inclusion-body myositis. Recruitment has begun in Houston, Texas and Phoenix, Arizona. Other sites selected but not recruiting as yet are in Orange, California; Miami, Florida; Kansas City, Kansas; Baltimore, Maryland; Boston, Massachusetts; Columbus, Ohio; Portland, Oregon; and Dallas, Texas. In addition, there are five sites in Italy and one in Denmark. 

The NIH Twin-Sib Study:

 risk factors and mechanisms for the development of systemic autoimmune diseases in adults and children. This study evaluates same-gender siblings within four years of age where one has been diagnosed within the last four years with rheumatoid arthritis, polyarticular juvenile idiopathic arthritis, lupus, systemic sclerosis or polymyositis/dermatomyositis/inclusion body myositis, and the other has no autoimmune disease.

The MYORISK Study: 

to determine if those with myositis, and particularly those with anti-synthetase syndrome (myositis with frequent interstitial lung disease and arthritis), have experienced different environmental exposures before disease onset than other polymyositis/dermatomyositis patients and healthy controls. Adult or juvenile polymyositis/dermatomyositis patients diagnosed within the last two years are eligible for enrollment.

The Myositis in Military Personnel Study:

compares those who developed any form of myositis during U.S. active duty service, with matched persons who did not develop an autoimmune disease during active duty to assess factors that might have led to the development of their myositis.

*These studies involve a single visit to the NIH Clinical Center in Bethesda, MD, the NIEHS Clinical Research Unit in Research Triangle Park, NC, a collaborating center, or a local physician's office to complete patient/parent and physician questionnaires and a blood draw. MYORISK patients will also collect a home dust sample. Patients may undergo a more thorough clinical evaluation if they enroll at the NIH Clinical Center. Compensation for enrollment will be $100 for each patient.

RESEARCH:

Similar to Parkinson’s disease, Inclusion Body Myositis (rare condition where the muscles waste away), affecting mainly older people, though there are other forms of myositis that are found in younger people. There is currently no treatment, and cuts to social services and the health service mean access to support such as physiotherapy, occupational therapy and speech therapy is spread very thin. (A)

Awareness of myositis among professionals is also very low, so patients have to become noisy advocates working through charities, in this case the Muscular Dystrophy Association, or individually. The cost to the health service through emergency hospital admissions, bed-blocking and severe disability is high, and access to specialist services is minimal.(A)

Increasing the profile of neurological conditions should be a much higher priority, not least because I have been told that the incidence of diagnosed cases is rising, perhaps because some people are being “cured” of higher-profile conditions such as cancer. It can be tiring having to tell every professional you meet the name of your illness and then explain what it is. (A)

Links:
(A)http://www.theguardian.com/society/2016/may/09/living-with-an-illness-thats-barely-understood
(B)https://understandingmyositis.org/types-of-myositis/inclusion-body-myositis/?gclid=CIP_-Nzt18wCFVZahgodd30BbQ
(C) http://www.myositis.org/explore-research/clinical-trials

*Please note! These are not my images! They were found on various tumblr sites! Please let me know if any are yours so that I can give you credit for them. Thanks so much & enjoy~
screenshots by:  https://film-grab.com/2014/07/10/the-grand-budapest-hotel/

Hidden Figures: Dementia

 Dementia:

Not a specific disease, dementia, is a group of conditions characterized by the impairment of at least two brain functions, such as memory loss and judgment. Symptoms include forgetfulness, limited social skills, and thinking abilities so impaired that it interferes with daily functioning. Medications and therapies may help manage symptoms. Some causes are reversible. A group of thinking and social symptoms that interferes with daily functioning. Affecting more than 3 million lives in America alone, dementia is very common. It can be chronic, lasting for years or be a lifelong condition. Unfortunately, it can not be cured, but treatments can help.

Alzheimer's Disease:

This is the most common form of dementia that is seen in patients. It is considered a slow progressive brain disease that begins before symptoms even emerge. Roughly about 60 to 80 percent develop Alzheimer's Disease. Early symptoms of individuals struggling with Alzheimer's Disease include having trouble remembering recent conversations, names, or events as well as apathy and depression. As the disease progresses with time, so do the symptoms. Communication is impaired, there is poor judgement, disorientation, confusion, behavior changes, and difficulty speaking, walking, and swallowing. The brain is altered from the deposits of protein fragment Beta-Amyloid Plaques, twisted strands of tau proteins (tangles), nerve cell damage, and cell death in the brain. 

Vascular dementia:

Previously known as multi-infarct or post-stroke dementia, vascular dementia is less common as a sole cause of dementia than Alzheimer’s, accounting for about 10 percent of dementia cases. Individuals have impaired judgement or inability to make conscious decisions. They are unable to plan or organize is usually the first symptoms of Vascular Dementia compared to that of Alzheimer's where the individual has memory loss as their initial symptoms. The reason why people suffering with VD have trouble making decisions is because of the blockage of blood vessels or brain damage that causes strokes or bleeding in the brain. The location, number and size of the brain injury determines how the individual's thinking and physical functioning are affected.

Brain imaging can often detect blood vessel problems implicated in vascular dementia. In the past, evidence for vascular dementia was used to exclude a diagnosis of Alzheimer's disease (and vice versa). That practice is no longer considered consistent with pathologic evidence, which shows that the brain changes of several types of dementia can be present simultaneously. When any two or more types of dementia are present at the same time, the individual is considered to have "mixed dementia" (see entry below).

Dementia with Lewy Bodies (DLB):

People with dementia with Lewy bodies often have memory loss and thinking problems common in Alzheimer's, but are more likely than people with Alzheimer's to have initial or early symptoms such as sleep disturbances, well-formed visual hallucinations, and slowness, gait imbalance or other parkinsonian movement features. Lewy bodies are abnormal aggregations (or clumps) of the protein alpha-synuclein. When they develop in a part of the brain called the cortex, dementia can result. Alpha-synuclein also aggregates in the brains of people with Parkinson's disease, but the aggregates may appear in a pattern that is different from dementia with Lewy bodies. The brain changes of dementia with Lewy bodies alone can cause dementia, or they can be present at the same time as the brain changes of Alzheimer's disease and/or vascular dementia, with each abnormality contributing to the development of dementia. When this happens, the individual is said to have "mixed dementia."

Mixed Dementia:

In mixed dementia abnormalities linked to more than one cause of dementia occur simultaneously in the brain. Recent studies suggest that mixed dementia is more common than previously thought. Characterized by the hallmark abnormalities of more than one cause of dementia —most commonly, Alzheimer's and vascular dementia, but also other types, such as dementia with Lewy bodies. As Parkinson's disease progresses, it often results in a progressive dementia similar to dementia with Lewy bodies or Alzheimer's. Problems with movement are common symptoms of the disease. If dementia develops, symptoms are often similar to dementia with Lewy bodies. Alpha-synuclein clumps are likely to begin in an area deep in the brain called the substantia nigra. These clumps are thought to cause degeneration of the nerve cells that produce dopamine.

 Frontotemporal Dementia : 

Includes dementias such as behavioral variant FTD (bvFTD), primary progressive aphasia, Pick's disease, corticobasal degeneration and progressive supranuclear palsy. Typical symptoms include changes in personality and behavior and difficulty with language. Nerve cells in the front and side regions of the brain are especially affected. No distinguishing microscopic abnormality is linked to all cases. People with FTD generally develop symptoms at a younger age (at about age 60) and survive for fewer years than those with Alzheimer's.

 CJD or Creutzfeldt-Jakob Disease:

CJD is the most common human form of a group of rare, fatal brain disorders affecting people and certain other mammals. Variant CJD (“mad cow disease”) occurs in cattle, and has been transmitted to people under certain circumstances. Rapidly fatal disorder that impairs memory and coordination and causes behavior changes. Results from misfolded prion protein that causes a "domino effect" in which prion protein throughout the brain misfolds and thus malfunctions.

 

Normal Pressure Hydrocephalus:

Symptoms include difficulty walking, memory loss and inability to control urination. Caused by the buildup of fluid in the brain. Can sometimes be corrected with surgical installation of a shunt in the brain to drain excess fluid.

 Huntington’s disease:

This progressive brain disorder caused by a single defective gene on chromosome 4. Include abnormal involuntary movements, a severe decline in thinking and reasoning skills, and irritability, depression and other mood changes. The gene defect causes abnormalities in a brain protein that, over time, lead to worsening symptoms.

Wernicke- Korsakoff syndrome:

Chronic memory disorder that is caused by severe deficiency of thiamine (vitamin B-1). The most common cause is alcohol misuse. Memory problems may be strikingly severe while other thinking and social skills seem relatively unaffected. Thiamine helps brain cells produce energy from sugar. When thiamine levels fall too low, brain cells cannot generate enough energy to function properly.

Research:

Researchers of University of Leicester conducted a study where they observed and recorded the ways in which lower levels of toxic by-products were produced by the nervous system of fruit flies. These fruit flies were previously engineered to have beta amyloid plaque build ups which causes neurodegeneration. Neurodegeneration negatively affects crawling and climbing abilities of these flies and shortened their lifespans. The collected data from the study showed that by lowering the levels of toxic metabolites (aka: the by-products of biological processes in the nervous system of the flies) can have a neuroprotective effect. (B)

Dr Doug Brown, Director of Research and Development at Alzheimer’s Society, said:

'Studies in fruit flies can help us to better understand some of the underlying biological changes that happen in Alzheimer’s disease. However, they only represent a very simplistic version of the condition – behavioural changes seen in fruit flies, such as slow crawling and climbing, are very different from the symptoms people with Alzheimer’s disease experience.(B)

Each year, roughly about 200,000 individuals will be diagnosed with dementia and the last time there was a new treatment presented to the public for patients with Alzheimer's disease was in 2002. So it is important that more research is being conducted so that people being diagnosed for dementia or those living with dementia can live more functioning and manageable lives. (B)

Alzheimer's Society is now uniting the Medical Research Council with the Alzheimer's UK Research team to tackle dementia thanks to a 50 million euro investment. The Prime Minister hopes to bring together scientists and experts from across the globe to transform the landscape of dementia research. The aim is to establish the very first Dementia Research Institute in the UK and to secure UK's reputation as the world leader in research and development against dementia. He call's it his 'Challenge on Dementia 2020'. Their government even invested £150 million to success of the program. (C)

The focus of this program will be to have a network of regional centers where hundreds of researchers will be able to interact with each other so that innovative scientific discoveries can be made more frequently that will help people world wide. The discovery of the mechanisms of the development and progression of dementia will improve health care strategies and improve care while reducing the risk of dementia for future generations. (C)

Links:
(A)http://www.theguardian.com/commentisfree/2016/feb/29/secret-life-dementia-sufferer-carer-iron-fridge
(B)https://www.alzheimers.org.uk/site/scripts/news_article.php?newsID=2595
(C)https://www.alzheimers.org.uk/site/scripts/news_article.php?newsID=2598

*Please note! These are not my images! If any are yours please let me know so that I can give you credit for them~ Also these images have no relation whatsoever between the people in these images and the Diseases I am discussing about. Thanks so much~ Enjoy!