Neurological Movement Disorder That Affects Million People In Europe

 

Parkinson’s disease (PD) is a chronic and progressive movement disorder, involves the malfunction and death of vital nerve cells in the brain. The specific group of symptoms that an individual experiences varies from person to person. Primary motor signs of Parkinson’s disease are tremor of the hands, rigidity or stiffness of the limbs and trunk and bradykinesia or slowness of movement.

The motor symptoms of Parkinson’s disease result from the death of dopamine-generating cells in the substantia nigra, a region of the midbrain. In 1817 an English doctor, James Parkinson describes the characteristic of this disease.

The four primary symptoms of Parkinson’s disease are: tremor, rigidity, Bradykinesia or akinesia and Postural Instability. In addition to the cardinal signs of Parkinson’s, there are many other motor symptoms associated with the disease as: Freezing, Micrographia, Mask-like Expression, Unwanted Accelerations, Dystonia, Speech problems, such as softness of voice or slurred speech caused by lack of muscle control.

Parkinson’s involves the malfunction and death of vital nerve cells in the brain, called neurons. Parkinson’s primarily affects neurons in an area of the brain called the substantia nigra. Some of these dying neurons produce dopamine, a chemical that sends messages to the part of the brain that controls movement and coordination. As PD progresses, the amount of dopamine produced in the brain decreases, leaving a person unable to control movement normally.

Causes and Risk Factors

Parkinson’s disease in most people is idiopathic (having no specific known cause). However, a small proportion of cases can be attributed to known genetic factors. Other factors have been associated with the risk of developing PD, but no causal relationships have been proven.   At least 5% -15% of cases are known to occur because of a mutation in one of several specific genes, transmitted in either an autosomal-dominant or autosomal-recessive pattern. medications that can cause drug-induced Parkinson disorders; these medications are dopamine blockers, as well, as noted on the PD Caregiver website, and can also cause movement disorders. An example of this class of medication is haloperidol, or Haldol, which is used for patients with schizophrenia.

SIGNS AND SYMTOMS

Prkinson’s disease affects movement, producing motor symptoms. Non-motor symptoms, which include autonomic dysfunction,neuropsychiatric problems (mood, cognition, behavior or thought alterations), and sensory and sleep difficulties, are also common. Some of these non-motor symptoms are often present at the time of diagnosis and can precede motor symptoms.

Tremor is the most apparent and well-known symptom. Tremor (involuntary, rhythmic shaking of a limb, head, or entire body) – The most recognized symptom of Parkinson’s disease, tremor often starts with an occasional tremor in one finger that eventually spreads to the whole arm. The tremor may affect only one part or side of the body, especially in the early stages of the disease. Not everyone with Parkinson’s disease has tremor.

Rigidity (stiffness or inflexibility of the limbs or joints) – The muscle rigidity experienced with Parkinson’s disease often begins in the legs and neck. Rigidity affects most people. The muscles become tense and contracted, and some people may feel pain or stiffness. In early stages of Parkinson’s disease, rigidity is often asymmetrical and it tends to affect the neck and shoulder muscles prior to the muscles of the face and extremities. With the progression of the disease, rigidity typically affects the whole body and reduces the ability to move.

Bradykinesia or akinesia  (slowness of movement) is another characteristic feature of PD, and is associated with difficulties along the whole course of the movement process, from planning to initiation and finally execution of a movement. Performance of sequential and simultaneous movement is hindered. Bradykinesia is commonly a very disabling symptom in the early stages of the disease. Initial manifestations are problems when performing daily tasks which require fine motor control such as writing, sewing or getting dressed. Clinical evaluation is based in similar tasks such as alternating movements between both hands or both feet. Bradykinesia is not equal for all movements or times. It is modified by the activity or emotional state of the subject, to the point that some people are barely able to walk yet can still ride a bicycle. Generally people with PD have less difficulty when some sort of external cue is provided.

Other recognized motor signs and symptoms include gait and posture disturbances such as festination (rapid shuffling steps and a forward-flexed posture when walking), speech and swallowing disturbances including voice disorders, mask-like face expression or small handwriting, although the range of possible motor problems that can appear is large.

Parkinson’s disease is a progressive disorder of the nervous system that affects movement. It develops gradually, sometimes starting with a barely noticeable tremor in just one hand. But while a tremor may be the most well-known sign of Parkinson’s disease, the disorder also commonly causes stiffness or slowing of movement.

DIAGNOSIS

No tests can conclusively show that you have Parkinson’s disease. Your doctor will base a diagnosis on your symptoms, medical history and the results of some simple exercises.

People may be given levodopa and resulting relief of motor impairment tends to confirm diagnosis. The finding of Lewy bodies in the midbrain on autopsy is usually considered proof that the person had Parkinson’s disease. The progress of the illness over time may reveal it is not Parkinson’s disease, and some authorities recommend that the diagnosis be periodically reviewed. To estimate the degree of disease severity using the results obtained from rating scales for Parkinson’s disease (Hoen and Yahr Staging of PD; UPDRS) and assist in the treatment and prognosis of the disease. UPDRS (Unified Parkinson’s Disease Rating Scale) is a scale consisting of three different sets of issues related to mental state – mood, behavior, ability to perform activities of daily living and motor activity. Maximum number of points is 199, which is also the most serious form of disability due to illness.

Additional diagnostic tests such as computed tomography scan (CT) or magnetic resonance imaging (MRI) can help eliminate some neurological or vascular disorders with similar symptoms, but not to prove the disease. Prior to the examination, the nurse helps the patient to take off clothes, take off shoes and socks, and attempts to establish communication with the patient in order to reduce fear and explained the procedure to be carried out during the examination.

PREVENTION

The goal of treatment is to achieve the continuous stimulation of dopaminergic neurons using the anti-Parkinson’s drug (medicament for the treatment of Parkinson’s disease). Levodopa is one of the most effective drugs and still is the gold standard in the treatment of Parkinson’s disease. In the brain, is directly converted to dopamine and thus compensates for the lack of physiological dopamine and because of which leads to the development of the disease. The most commonly administered in combination with inhibitors of decarboxylase enzymes which prevent its rapid decomposition and allow a greater amount of levodopa reaches the brain.

Levodopa has been the most widely used treatment for over 30 years. L-DOPA is converted into dopamine in the dopaminergic neurons by dopa decarboxylase. Since motor symptoms are produced by a lack of dopamine in the substantia nigra, the administration of L-DOPA temporarily diminishes the motor symptoms.

Only 5–10% of L-DOPA crosses the blood–brain barrier. The remainder is often metabolized to dopamine elsewhere, causing a variety of side effects including nausea, dyskinesia and joint stiffness. Carbidopa and benserazide are peripheral dopa decarboxylase inhibitors, which help to prevent the metabolism of L-DOPA before it reaches the dopaminergic neurons, therefore reducing side effects and increasing bioavailability. They are generally given as combination preparations with levodopa. Existing preparations are carbidopa/levodopa (co-careldopa) and benserazide/levodopa (co-beneldopa). Levodopa has been related to dopamine dysregulation syndrome, which is a compulsive, and pounding. There are controlled release versions of levodopa in the form intravenous and intestinal infusions that spread out the effect of the medication. These slow-release levodopa preparations have not shown an increased control of motor symptoms or motor complications when compared to immediate release preparations.

Tolcapone inhibits the COMT enzyme, which degrades dopamine, thereby prolonging the effects of levodopa. It has been used to complement levodopa; however, its usefulness is limited by possible side effects such as liver damage. A similarly effective drug, entacapone, has not been shown to cause significant alterations of liver function. Licensed preparations of entacapone contain entacapone alone or in combination with carbidopa and levodopa.

Levodopa preparations lead in the long term to the development of motor complications characterized by involuntary movements called dyskinesias and fluctuations in the response to medication. When this occurs a person with PD can change from phases with good response to medication and few symptoms (“on” state), to phases with no response to medication and significant motor symptoms (“off” state). For this reason, levodopa doses are kept as low as possible while maintaining functionality. Delaying the initiation of therapy with levodopa by using alternatives (dopamine agonists and MAO-B inhibitors) is common practice. A former strategy to reduce motor complications was to withdraw L-DOPA medication for some time. This is discouraged now, since it can bring dangerous side effects such as neuroleptic malignant syndrome. Most people with PD will eventually need levodopa and later develop motor side effects.

Treating motor symptoms with surgery was once a common practice, but since the discovery of levodopa, the number of operations declined. Studies in the past few decades have led to great improvements in surgical techniques, so that surgery is again being used in people with advanced PD for whom drug therapy is no longer sufficient. Surgery for PD can be divided in two main groups: lesional and deep brain stimulation (DBS). Target areas for DBS or lesions include the thalamus, the globus pallidus or the subthalamic nucleus. Deep brain stimulation (DBS) is the most commonly used surgical treatment, developed in the 1980s by Alim-Louis Benabid and others. It involves the implantation of a medical device called a neurostimulator which sends electrical impulses to specific parts of the brain. DBS is recommended for people who have PD with motor fluctuations and tremor inadequately controlled by medication, or to those who are intolerant to medication, as long as they do not have severe neuropsychiatric problems. Other, less common, surgical therapies involve intentional formation of lesions to suppress overactivity of specific subcortical areas. For example, pallidotomy involves surgical destruction of the globus pallidus to control dyskinesia.

Exercise programs are recommended in people with Parkinson’s disease. There is some evidence that speech or mobility problems can improve with rehabilitation, although studies are scarce and of low quality. Regular physical exercise with or without physiotherapy can be beneficial to maintain and improve mobility, flexibility, strength, gait speed, and quality of life. When an exercise program is performed under the supervision of a physiotherapist, there are more improvements in motor symptoms, mental and emotional functions, daily living activities, and quality of life compared to a self-supervised exercise program at home. People with Parkinson’s may have many difficult decisions to make as the disease progresses such as wishes for feeding tube, non-invasive ventilator, and tracheostomy; wishes for or against cardiopulmonary resuscitation; and when to use hospice care. Palliative care team members can help answer questions and guide people with Parkinson’s on these complex and emotional topics to help them make the best decision based on their own values.

NUTRITION

According to Dr. Sc. Donatella Verbanac, BSc. Engineer of Medical Biochemistry, patients should follow a diet rich in fresh fruits, vegetables and whole grains. Moderately must use proteins and unsaturated fats and reduce saturated fats and sugars. It is very important fiber intake, digestion is the part that can also hurt due to poor gut motility, therefore, are advised to eat foods that are rich in dietary fiber. The water-soluble are some fiber (pectin from fruit), and some are not (cellulose coating grains and shell the pods).

For people who suffer from Parkinson’s disease is very important to take fluids during the day. It is necessary to take a few sips of water in small amounts throughout the day. It is best for this purpose to keep the water beside yourself or put in a visible place. With increasing of age (over 60) comes to a loss of the feeling of thirst, it can appear symptoms of dehydration: dry lips, mouth, skin, problems with sweating and retention of urine. The total daily amount of fluid mandatory should be about 2 liters.

Vitamins that are recommended to be taken are: vitamin C and E and the minerals zinc, magnesium and calcium. Vitamin B6 is made beneficial in combination with magnesium, some studies suggest that the vitamin reduces the action of dopamine, which is added as a medicament. Perhaps it is best to resort to smaller amounts of B-complex, or take a drink once a day in which the content of the B-complex is significant.

INTERESTING FACTS

James Parkinson was born in Shoreditch, London, England. He was the son of John Parkinson, an apothecary and surgeon practising in Hoxton Square in London. He was the oldest of three siblings, which included his brother William and his sister Mary Sedgewood. In 1784 Parkinson was approved by the City of London Corporation as a surgeon.

On 21 May 1783, he married Mary Dale, with whom he subsequently had eight children; two did not survive past childhood. Soon after he was married, Parkinson succeeded his father in his practice in 1 Hoxton Square. He believed that any worthwhile surgeon should know shorthand, at which he was adept. He is most famous for his 1817 work, An Essay on the Shaking Palsy in which he was the first to describe “paralysis agitans”, a condition that would later be renamed Parkinson’s disease by Jean-Martin Charcot.

The group from Imperial College and the Royal College of Art started the project by looking for ways to help Parkinson’s sufferers that were focussed not just on improving the ease of essential activities such as eating and sleeping, but activities that brought joy to people’s lives. The ARC pen uses high-frequency vibration motors to facilitate larger and clearer handwriting, and in an initial trial of 14 people with micrographia it was found to improve writing in 86 percent of cases. The motors inside the ARC stimulate the muscles in the hand, reducing the effort that is required to move the pen across paper.