Frontotemporal Dementia With Parkinsonism
Parkinsonism in frontotemporal dementia is usually seen in the behavioral variant, rather than in association with primary progressive aphasia, and can develop either before or during the development of the classical FTD syndrome. It can closely mimic idiopathic PD or have features suggestive of PSP or CBS. It is seen in association with underlying tau, TDP-43, or FUS pathology, as well as corresponding mutations in several genes, which include MAPT, PGRN, C9ORF72, FUS, and TARDBP. Rigidity and bradykinesia tend to be the more prominent parkinsonian features, with rest tremor occurring rarely. There is variable responsiveness to levodopa .
Environmental Factors And Exposures
Exposure to pesticides and a history of head injury have each been linked with PD, but the risks are modest. Never drinking caffeinated beverages is also associated with small increases in risk of developing PD.
Low concentrations of urate in the blood is associated with an increased risk of PD.
Drug-induced parkinsonism
Different medical drugs have been implicated in cases of parkinsonism. Drug-induced parkinsonism is normally reversible by stopping the offending agent. Drugs include:
Parkinson’s Disease Diet And Nutrition
Maintaining Your Weight With Parkinson’s Disease
Malnutrition and weight maintenance is often an issue for people with Parkinson’s disease. Here are some tips to help you maintain a healthy weight.
- Weigh yourself once or twice a week, unless your doctor recommends weighing yourself often. If you are taking diuretics or steroids, such as prednisone, you should weigh yourself daily.
- If you have an unexplained weight gain or loss , contact your doctor. He or she may want to modify your food or fluid intake to help manage your condition.
- Avoid low-fat or low-calorie products. . Use whole milk, whole milk cheese, and yogurt.
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What Is Parkinson’s Disease
Parkinson’s disease is the second most common neurodegenerative disorder and the most common movement disorder. Characteristics of Parkinsons disease are progressive loss of muscle control, which leads to trembling of the limbs and head while at rest, stiffness, slowness, and impaired balance. As symptoms worsen, it may become difficult to walk, talk, and complete simple tasks.
The progression of Parkinson’s disease and the degree of impairment varies from person to person. Many people with Parkinson’s disease live long productive lives, whereas others become disabled much more quickly. Complications of Parkinsons such as falling-related injuries or pneumonia. However, studies of patent populations with and without Parkinsons Disease suggest the life expectancy for people with the disease is about the same as the general population.
Most people who develop Parkinson’s disease are 60 years of age or older. Since overall life expectancy is rising, the number of individuals with Parkinson’s disease will increase in the future. Adult-onset Parkinson’s disease is most common, but early-onset Parkinson’s disease , and juvenile-onset Parkinson’s disease can occur.
The Role Of Dementia And Age
Dementia also plays an important role in survival with Parkinsons. By the end of the above study, nearly 70% of the population with Parkinsons had been diagnosed with dementia, and those with dementia had a lower survival rate as compared to those without.
This means that those with dementia were more likely to die during the six-year period than those without dementia. In addition, scientific studies have shown that increasing age is linked to an increased risk of death.
Its important to remember that how a persons Parkinsons disease manifests and progresses is variable, and a persons neurologist cannot accurately predict individual life expectancy.
There are simply no key signs or symptoms that allow a doctor to perfectly predict longevity. An older age and the presence of dementia are simply associated with an increased risk of dying.
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What Are The Different Forms Of Parkinsonism
There are three main forms of parkinsonism, as well as other related conditions.
Most people with parkinsonism have idiopathic Parkinsons disease, also known as Parkinsons. Idiopathic means the cause is unknown.
The most common symptoms of idiopathic Parkinsons are tremor, rigidity and slowness of movement.
Vascular parkinsonism affects people with restricted blood supply to the brain. Sometimes people who have had a mild stroke may develop this form of parkinsonism.
Common symptoms include problems with memory, sleep, mood and movement.
Some drugs can cause parkinsonism.
Neuroleptic drugs , which block the action of the chemical dopamine in the brain, are thought to be the biggest cause of drug-induced parkinsonism.
The symptoms of drug-induced parkinsonism tend to stay the same only in rare cases do they progress in the way that Parkinsons symptoms do.
Drug-induced parkinsonism only affects a small number of people, and most will recover within months and often within days or weeks of stopping the drug thats causing it.
Disturbances In Autonomic Function
Autonomic dysfunction may present prior to the diagnosis or become apparent with disease progression or be induced by medication . All areas of autonomic function may be affected and this has been reported to affect daily life of over 50% of patients . The autonomic dysfunction is considered because of involvement of both the central and peripheral postganglionic autonomic nervous system . Orthostatic hypotension affects 30â40% of patients. This is defined as a fall in systolic blood pressure of > 20 mm Hg or in diastolic blood pressure > 10 mm Hg on either standing or head-up tilt to at least 60 degrees within 3 min . On assuming the upright posture, hypotension-induced hypoperfusion of the brain can result in dizziness, visual disturbances and impaired cognition that may precede loss of consciousness. In PD, the blood pressure drop may last several minutes . Duration of PD may be unrelated to the occurrence of orthostatic hypotension . In elderly PD patients, this may mainly occur after food intake .
Gastrointestinal symptoms are common. There is slowing of mobility of the gastrointestinal tract with symptoms such as postprandial fullness and gastric retention, but slow-transit constipation is by far the most common, occurring in 70â80% . Patients may also experience difficulties in rectal evacuation because of rectal sphincter dysfunction .
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Dementia With Lewy Bodies
- Dementia with Lewy bodies is a progressive, neurodegenerative disorder in which abnormal deposits of a protein called alpha-synuclein build up in multiple areas of the brain.
- Dementia with Lewy bodies is second to Alzheimers as the most common cause of degenerative dementia that first causes progressive problems with memory and fluctuations in thinking, as well as hallucinations. These symptoms are joined later in the course of the disease by parkinsonism with slowness, stiffness and other symptoms similar to PD.
- While the same abnormal protein is found in the brains of those with PD, when individuals with PD develop memory and thinking problems it tends to occur later in the course of the disease.
- There are no specific treatments for DLB. Treatment focuses on symptoms.
Stages Of Parkinsons Disease
This disease usually progresses through four or five distinct stages, and an individuals life expectancy depends on which stage he or she is in. During the first stage of Parkinsons, the individual might not even know he or she has the disease, and only minor symptoms are present. As the disease progresses through the next few stages, the symptoms become much more pronounced. Issues such as tremors and muscle rigidity begin to make everyday activities more difficult. By the fourth and fifth stages, seniors lose their independence, and the symptoms become life-altering.
An in-home caregiver can be a fantastic source of assistance for a senior with Parkinsons. When considering elder care. Garland, families should make sure their senior loved ones have the resources they need to maintain their independence and remain healthy. Trusted in-home care professionals can assist seniors with daily tasks like cooking, bathing, and exercise, and they can also encourage them to focus on healthier lifestyle habits.
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The Diagnosis Of Idiopathic Parkinsons Disease
The first component to the diagnosis of PD is establishing that the patient has parkinsonism. This is a clinical diagnosis and relies on three key elements: bradykinesia, tremor, and rigidity. Of these, bradykinesia must be present, with at least one of the other two. PD is an asymmetrical condition, so during the clinical assessment, the parkinsonism should be more apparent on one side and may be purely unilateral in early disease . is a classical illustration of parkinsonism, with a description by William Gowers.
A case of Parkinsons disease as described and illustrated by William Gowers: the aspect of the patient is very characteristic. The head is bent forward, and the expression of the face is anxious and fixed, unchanged by any
What Are The Different Stages Of Parkinsons Disease
Each person with Parkinsons disease experiences symptoms in in their own unique way. Not everyone experiences all symptoms of Parkinsons disease. You may not experience symptoms in the same order as others. Some people may have mild symptoms others may have intense symptoms. How quickly symptoms worsen also varies from individual to individual and is difficult to impossible to predict at the outset.
In general, the disease progresses from early stage to mid-stage to mid-late-stage to advanced stage. This is what typically occurs during each of these stages:
Early stage
Early symptoms of Parkinsons disease are usually mild and typically occur slowly and do not interfere with daily activities. Sometimes early symptoms are not easy to detect or you may think early symptoms are simply normal signs of aging. You may have fatigue or a general sense of uneasiness. You may feel a slight tremor or have difficulty standing.
Often, a family member or friend notices some of the subtle signs before you do. They may notice things like body stiffness or lack of normal movement slow or small handwriting, lack of expression in your face, or difficulty getting out of a chair.
Mid stage
Mid-late stage
Standing and walking are becoming more difficult and may require assistance with a walker. You may need full time help to continue to live at home.
Advanced stage
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Demographic And Clinical Characteristics
Patients recruited were aged between 46 and 80years old . The majority of patients reported living with their spouse and just over half of the sample were retired , although several patients also reported working full or part time . Almost all of the patients were white . Patients were at stage 1, 2, or 3 on the Hoehn and Yahr scale. It was not possible to recruit patients above stage 3 as the increased limitations in physical and cognitive functioning reduces patients ability to participate in two-hour interviews. The recruiting clinicians reported that 11 patients had complete functional independence, seven patients had modified functional independence, whereas the remaining two required minimal assistance. Further details of the sample are included in Table .
Table 2 Demographic and clinical characteristics of GBA-PDa and iPDb patient samples
The expert clinicians identified by the sponsor were based in the US, Israel and Italy. The clinicians were all neurologists or clinical geneticists and treated a mixture of GBA-PD and iPD patients. The clinicians reported that between 20 and over 200 GBA-PD patients were treated at their clinical site. The US-based clinician recruited seven of the GBA-PD patients who were interviewed as part of the study.
What Lifestyle Changes Can I Make To Ease Parkinsons Symptoms
Exercise: Exercise helps improve muscle strength, balance, coordination, flexibility, and tremor. It is also strongly believed to improve memory, thinking and reduce the risk of falls and decrease anxiety and depression. One study in persons with Parkinsons disease showed that 2.5 hours of exercise per week resulted in improved ability to move and a slower decline in quality of life compared to those who didnt exercise or didnt start until later in the course of their disease. Some exercises to consider include strengthening or resistance training, stretching exercises or aerobics . All types of exercise are helpful.
Eat a healthy, balanced diet: This is not only good for your general health but can ease some of the non-movement related symptoms of Parkinsons, such as constipation. Eating foods high in fiber in particular can relieve constipation. The Mediterranean diet is one example of a healthy diet.
Preventing falls and maintaining balance: Falls are a frequent complication of Parkinson’s. While you can do many things to reduce your risk of falling, the two most important are: 1) to work with your doctor to ensure that your treatments whether medicines or deep brain stimulation are optimal and 2) to consult with a physical therapist who can assess your walking and balance. The physical therapist is the expert when it comes to recommending assistive devices or exercise to improve safety and preventing falls.
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Symptoms Of Parkinsons Disease
Image: Sir William Richard Gowers, neurologist, researcher, and artist, drew this illustration in 1886 as part of his documentation of Parkinsons Disease. The image appeared in his book, A Manual of Diseases of the Nervous System, still used today by medical professionals as a primary reference for this disease. By Beao, License: Public domain
The typical clinical triad of Parkinsons disease consists of akinesia, rigor, and tremor. Partially, postural instability is considered as the core symptom. Typically, the tremor is a resting tremor, which initially starts on one side, implying that it manifests asymmetrically.
N.B.
Depending on the most prominent symptom, one can distinguish between tremor dominant, akinetic-rigid, and equivalent types. In the equivalent type Parkinsons disease, the 3 symptoms of the clinical triad are roughly equally distinct.
Additionally, the patients gait provides the 1st clue. In hypo- or akinesia, the patients gait is marked by small steps and a stooped torso. The arms swing less, usually on one side.
Further symptoms occurring before the onset of classical Parkinsons symptoms include disorders of REM sleep and impaired smell either in the form of hyposmia or anosmia. Pain may involve the shoulder/neck region or the extremities.
N.B.
As the disease progresses further, vegetative symptoms of psychopathological nature such as frontal dementia or depression can occur.
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Clinical Functional Assessment: Mobilityselfcare Cognition/behavior/affective State
- Reduced manual dexterity may impair buttoning clothes, tying shoelaces, writing and computer use.
- Mobility deficits in addition to gait include difficulty with transitional movements such as sit to stand, and bed, bath and car transfers.
- Patients may have slower or more abnormal mobility with dual tasking.
- Other functional issues include swallow dysfunction and communication impairment related to dysarthria and hypophonia.
- Driving safety assessments should be considered depending on driving history and deficits noted on examination.
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The Motor Symptoms Of Parkinson’s Disease
| Step 1: Diagnosis of Parkinsonian syndrome |
| Bradykinesia |
| And at least one of the following |
| Muscular rigidity |
| Levodopa response for 5 years or more |
| Clinical course of 10 years or more |
- Source: Hughes et al. .
Postural stability may be affected either early or later in the disease process and this may lead to falls and injuries. Early falls are atypical for those who are younger at onset but age is an independent risk factor for falls in PD and, in the elderly, the disease is sometimes first diagnosed in hospitals after a fall. A study by Wood et al. showed that falls occurred in 68% of 109 patients with PD with a mean age of 75 years and mean disease duration of 3 years. Another study reported falls in 62% of patients with PD . Predictors of falls other than older age include duration of disease, dementia, symmetrical onset, postural and autonomic instability .
Oral motor disorders are common. Speech disturbances such as very quiet and hurried speech occur in more than half of the patients , swallowing problems have been reported in 40â80% and a quarter of the patients report dribbling of saliva .
Neurodegeneration With Brain Iron Accumulation
Neurodegeneration with brain iron accumulation patients present with a progressive extrapyramidal syndrome associated with iron deposition in the basal ganglia. The two main syndromes are outlined here, although there are additional syndromes including neuroferritinopathy and aceruloplasminemia. The most common of the NBIA disorders is pantothenate kinase-associated neurodegeneration , resulting from mutations on the PANK2 gene, accounting for 50%. The classic syndrome manifests in early childhood with a combination of pyramidal and extrapyramidal features . PKAN can also rarely present in early adulthood. There are typical MRI findings, with a central hyperintensity with surrounding low signal on T2 images in the globus pallidus, giving the so-called eye-of-the-tiger sign .
The second main type of NBIA is PLA2G6-associated neurodegeneration . When onset occurs in infancy, PLAN causes progressive motor and mental retardation with cerebellar ataxia, seizures, and pyramidal signs. However, onset can occur later in life which leads to an atypical syndrome that may mimic PD, with rest tremor, rigidity, and bradykinesia and a good response to levodopa. However, patients also exhibit additional features including eye-movement abnormalities and pyramidal signs .
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Expert Clinician Discussion Of Symptoms And Impacts
The expert clinicians confirmed that the symptom and impact concepts in the draft conceptual model were all relevant for GBA-PD patients. Specifically, clinicians confirmed that all of the most important symptoms and impacts were experienced by the patients that they treated with GBA-PD. Clinicians also identified REM sleep disorder and a decline in visual acuity as symptoms of GBA-PD. REM sleep disorder was only reported by GBA-PD patients in the sample however, a decline in visual acuity was reported by both GBA-PD and iPD patients. The alignment between the findings from the clinician interviews and the GBA-PD patient interviews provides support for the generalizability of the results of this study.
Further, the clinicians commented that GBA-PD and iPD patients disease experience is broadly similar. However, clinicians agreed that cognitive/psychiatric symptoms might present earlier and progress more rapidly in GBA-PD patients, as compared to iPD patients.
Specific Secondary Or Associated Conditions And Complications
Due to motor impairments, PD patients are at high risk for falls with potential fractures. Dysphagia is common in advanced PD, and aspiration pneumonia is the most common cause of PD-related deaths.1 Nonmotor symptoms are frequently seen in PD these include urge urinary incontinence, constipation, depression or anxiety disorder, cognitive impairment, sleep disorders and pain syndromes.
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