Among all forms of progressive dementia, Lewy body dementia (LBD, in brief) is the most widespread. This disorder only takes place once in a while and especially in people who do not have any history of the ailment in the family. Nevertheless, there have been rare cases when the disease was passed on hereditarily.
Lewy body dementia affects the cells in the cortex (outer stratum) of the brain as well as some of the cells located in the middle of the brain - substantia nigra. Soon after these cells are affected by the disease, they die. However, several remaining cells in the brain's region called substantia nigra comprise anomalous formations called Lewy bodies and these are the characteristic of the disease. In addition to substantia nigra, the Lewy bodies may also be present in the cortex of the brain.
In fact, the Lewy bodies enclose a protein known as alpha-synuclein, which has been associated with Parkinson's disease as well as many other maladies. While researchers occasionally denote these disorders in a group as 'synucleinopathies', they are yet to ascertain the reason that leads the alpha-synuclein protein builds up within the nerve cells in Lewy body dementia.
Some of the characteristics of Lewy body dementia are similar to those of Alzheimer's disease as well as Parkinson's disease. Like in the case of Alzheimer's disease, Lewy body dementia also results in perplexity and confusion, while it may even cause firm muscles, sluggish movements and shudders as they happen in patients suffering from Parkinson's disease.
However, the most remarkable sign of the Lewy body dementia is perhaps the visual delusions experienced by the patients. In fact, when people experience this symptom, it is often considered to be the first sign of developing the disease. The delusions experienced by patients suffering from Lewy body dementia are likely to vary from abstract forms or colors to tête-à-tête with the loved ones who have already died.
In people enduring Lewy body dementia, anomalous disc-shaped formations known as Lewy bodies develop in some areas of the brain that are associated with judgment and mobility. The risks of developing the disease enhances with advancing age. However, it is believed that this form of dementia roughly develop in a very small number of people. Precisely speaking, Lewy body dementia (LBD) actually affects below one per cent of the populace above the age of 65 years.
The signs and symptoms appear very late in the patient's life, after they are approximately 65 years of age, and may vary from one patient to another. The most common signs and symptoms of the disease are mentioned below.
Cerebral or reasoning problems
There may be occasions when people enduring Lewy body dementia will have symptoms resembling those associated with Alzheimer's disease, such as confusion, loss of memory and inability to concentrate or poor attentiveness.
Disorderly movements
People suffering from Lewy body dementia may often endure symptoms common to Parkinson's disease, such as sluggish movement, firm muscles, shudders or a hobbled walk.
Delusions
Lewy body dementia patients may suffer from conditions where they are likely to have false perceptions regarding other persons or any situation.
Visual illusions
Patients suffering from Lewy body dementia (LBD) may often see colors, forms, animals and people that may not actually exist before them. In fact, visual hallucinations are considered to be the first signs of developing LBD.
Sleep disorders or sleeplessness
People suffering from Lewy body dementia may suffer from sleep disorders which may actually lead them to physically enact their dreams while they are asleep.
It is regrettable to note that till date there is no specific cure for Lewy body dementia and most of the treatments for this disease include medications used to cure Parkinson's disease and/ or Alzheimer's disease. In fact, the known treatments of LBD are intended to regulate the symptoms of Parkinson's disease found in some LBD patients as well as the psychiatric symptoms of the disease.
It may be mentioned here that occasionally, Lewy body dementia patients response remarkably to the treatment using drugs meant for healing Parkinson's disease and/ or cholinesterase inhibitors like the ones used to treat Alzheimer's disease. A number of researches conducted on LBD have hinted that using neuroleptic drugs like clozapine and olanzapine may also prove to be useful in lessening the psychiatric symptoms of this malady.
However, the problem is that administering neuroleptic drugs to patients suffering from Lewy body dementia may result in grave adverse after-effects. Hence, it is advisable that LBD patients ought to first try other forms of therapies before using neuroleptic drugs. And In case it is essential to use the neuroleptic drugs, the patients need to be examined strictly.
It has been observed that the anomalous disc-shaped formations known as Lewy bodies are often present in people enduring Alzheimer's disease and Parkinson's disease. Such findings by researchers imply that Lewy body dementia is closely associated to the other aspects that cause dementia or the fact that this disorder occasionally exists along with Parkinson's disease and/ or Alzheimer's disease in some patients.
This type of brain disorder is very uncommon, but fatal and affects approximately one person in a million people each year globally. The symptoms of Creutzfeldt-Jakob disease, also called CJD in brief, generally start becoming noticeable in patients above 60 years of age and majority of the patients affected by this brain malady succumb to it within a year of diagnosis.
It has been found that majority of the incidences of Creutzfeldt-Jakob disease takes place once in a while and in most cases the patients do not usually have any prior risk factors that may result in the deadly and rare disease. Many researchers are of the view that this particular brain disorder is caused by an anomalous type of a protein known as prion.
Although Creutzfeldt-Jakob disease is considered to be a very rare disease that occurs sporadically and often to people having no risk factor of the malady, it has been found that approximately five to ten per cent of the CJD cases in the United States are inherited. In such cases, the disease is passed from one generation to another owing to a change in the gene of the protein called prion.
It is interesting to note that this disease may also be acquired in exceptional cases. Creutzfeldt-Jakob disease is generally acquired when there is an exposure to an affected brain or the tissues of the nervous system by means of specific medical processes. However, scientists are yet to find any proof that would suggest that the malady is infectious either through the air or by means of coming into careless contact with any person suffering from the ailment.
Initially, patients enduring this rare, but fatal brain disorder suffer from problems like absence of muscular synchronization, changes in personality counting damaged memory, perceptiveness and thinking. Such patients may even experience harmed eyesight. The symptoms of Creutzfeldt-Jakob disease may also comprise dejection and sleeplessness.
With the advancement of the malady, the cerebral damage of the patients becomes acute. Most of the patients also suffer from myoclonus (a sudden spasm or jerk of a muscle or collection of muscles) and lose their eyesight. In the final stages of the disease, the patients suffer from immobility as well as verbal communication and ultimately go into a stage of coma. These patients may also develop pneumonia and other contagions which may facilitate their death.
Creutzfeldt-Jakob disease is a part of the family of the human and animal ailments classified as the transmissible spongiform encephalopathies (TSEs). In other words, TSEs are different forms of encephalopathies distinguished by spongy deterioration of the brain tissue and said to be caused by the ingestion of a venomous protein or virus.
The term spongiform denotes the typical appearance of diseased brain that is filled with holes till the time they appear like sponges when seen under a microscope. Among the different forms of TSEs, Creutzfeldt-Jakob disease is most widespread. The other forms of transmissible spongiform encephalopathies include the Gerstmann-Straussler-Scheinker (GSS) disease and the fatal familial insomnia.
It may be noted that some years ago, scientists have detected a new form of Creutzfeldt-Jakob disease, known as variant CJD (vCJD in brief), in several regions of the United Kingdom as well as in different European nations. The preliminary signs of vCJD differ from those of the typical CJD. Besides, unlike in the case of the classic CJD when the first symptoms of the disease become noticeable only after the patient is 60 years old, in the instance of vCJD, the disease crops up in younger people.
Several studies conducted on the subject hints that variant CJD may be caused due to the consumption of beef obtained from cattle suffering from a TSE ailment known as bovine spongiform encephalopathy (BSE). This condition is also denoted as the 'mad cow disease'.
