1817 • Affects ~1 million in the U.S. • Onset typically between 50-60 years of age, and slowly progresses with age • Average onset is 62.4 years of age Background • Parkinson’s disease is a disorder that affects nerve cells in the part of the brain controlling muscle movement • Disease is progressive – signs/symptoms worsen over time • Eventually is disabling, but progresses gradually • Believed to be caused by genetics, environmental factors or a combination of the two • Idiopathic or unknown etiology • The exact cause of Parkinson's disease is unknown, although research points to a combination of genetic and environmental factors. • If a continuum existed, with exclusively genetic causes at one end and exclusively environmental causes at the other, different Parkinson's patients would likely fall at many different places along that continuum. Neurological Basis • “Neurodegenerative Disease” : caused by degeneration (dysfunction and death) of neurons within the brain • NORMAL BRAIN FUNCTION – Basal Ganglia • Cells in substantia nigra produce/release dopamine • Dopamine released by SN neurons lands on neurons of other brain centers, controlling their firing • Main targets are caudate nucleus and putamen (striatum) • This basal ganglia pathway is involved in regulation of movement • The basal ganglia are a group of nuclei situated deep and centrally at the base of the forebrain. • They have robust connections with the cerebral cortex and thalamus in addition to other areas of the brain. • Their vast system of communication allows them involvement with a variety of functions, including automatic and voluntary motor control, procedural learning relating to routine behaviors and emotional functions. • The association with other cortical areas ensures smoothly orchestrated movement control and motor behavior.
• The striatum, composed of the caudate and
putamen, is the largest nuclear complex of the basal ganglia. The striatum receives excitatory input from several areas of the cerebral cortex, as well as inhibitory and excitatory input from the dopaminergic cells of the substantia nigra pars compacta (SNc). These cortical and nigral inputs are received by the spiny projection neurons, • Today, we understand Parkinson's disease to be a disorder of the central nervous system that results from the loss of cells in various parts of the brain, including a region called the substantia nigra. • The substantia nigra cells produce dopamine, a chemical messenger responsible for transmitting signals within the brain that allow for coordination of movement. • Loss of dopamine causes neurons to fire without normal control, leaving patients less able to direct or control their movement. Parkinson's disease is one of several diseases categorized by clinicians as movement disorders. (https://www.michaeljfox.org/understanding- parkinsons) Neurological Basis
• PARKINSON’S BRAIN FUNCTION–
Basal Ganglia • Cells of substantia nigra degenerate • These cells can no longer produce adequate amounts of dopamine • Neurons of striatum, etc. are no longer well regulated, thus do not behave in normal manner • Results in loss of control of movements – leads to symptoms characteristic of Parkinson’s disease • In Parkinson disease, degeneration of the basal ganglia, along with damage to the dopamine-producing cells of the substantia nigra, hampers the proper functioning of the nerve pathway that controls movements of the muscles. • The muscles become excessively tense, a condition that gives rise to tremor and a rigid joint action. The movements of the body also begin to slow down because of this malfunction. Characteristic Symptoms • MOTOR • NONMOTOR • tremor • diminished sense • bradykinesia of smell • rigidity/freezing in • low voice volume place • foot cramps • lack of facial • sleep disturbance expression • depression • postural instability • constipation • stooped, shuffling gait • drooling Typically, symptoms begin on one side of the body and migrate over time to the other side. DIGNOSIS •There is no objective test (such as a blood test, brain scan or EEG) to make a definitive diagnosis of Parkinson's disease. •Instead, a doctor takes a careful medical history and performs a thorough neurological examination, looking in particular for two or more of the cardinal signs to be present. Frequently, the doctor will also look for responsiveness to Parkinson's disease medications as further evidence that Parkinson's is the correct diagnosis. •In 2011, the Food and Drug Administration (FDA) approved a specialized imaging technique called DaTscan that allows doctors to capture detailed pictures of the dopamine system in your brain. It is the first FDA-approved diagnostic imaging technique for the assessment of movement disorders such as Parkinson's disease. DaTscan alone can't diagnose Parkinson's disease by itself, but it can help confirm a physician's clinical diagnosis -- something that has never been possible before. Conventional Treatments: Medication • LEVODOPA (L-DOPA) • precursor to dopamine, converted to dopamine by nerve cells in the brain • Treatment with dopamine not possible, because dopamine can’t cross blood-brain barrier • Generally combined with carbidopa (Sinemet) – helps levodopa get to the brain ROLE OF SPEECH PATHOLOGIST •Speech-language pathology domains in Parkinson’s disease •With respect to Parkinson’s disease, speech- language pathology focuses on three domains: • – difficulty with speech • – difficulty with chewing and swallowing: dysphagia, choking and slow chewing and swallowing • – difficulty with controlling saliva: drooling or dribbling of saliva SPEECH PROBLEMS •About 75% of people with PD experience changes in speech and voice at some time during the course of the disease. These changes usually come on gradually and can vary from moderate to severe. How Do I Know if I Have Problems with Speech and Communication? This self-test can help you determine if you have a communication problem. Think about the following statements, and place a check mark next to the ones that apply to you. ❏ I am often asked to repeat a statement. ❏ People look slightly confused or as if they are trying hard to listen when I speak. ❏ My care partner says that I sometimes slur or mumble words. ❏ My care partner asks that I speak louder. ❏ I feel that my care partner is ignoring me or may need a hearing aid. ❏ I do not attend social gatherings as often as before. ❏ I notice that I often stop trying to communicate in a group where others seem to talk over me. ❏ I feel like people do not listen to me anymore ❏ I feel like people think that I don’t have anything interesting to say. ❏ I try to avoid the telephone. ❏ I need to clear my throat often. ❏ I cannot complete a conversation without feeling frustrated about my inability to communicate what I have to say. If you checked any of these Care Partner Speech and Communication Survey If you are a care partner, family member or friend who has regular contact with a person with PD, complete this questionnaire. Check the statements that are true for your family member or friend. •I have difficulty hearing when s/he speaks. •I have difficulty understanding his or her speech. •S/he does not talk as much as in the past. •S/he does not attend social functions as frequently as in the past. •S/he often asks me to make phone calls or order from a menu for him or her. •S/he clears his or her throat often. •S/he often sounds as if s/he is running out of breath when speaking. •S/he suspects that I need a hearing aid. •S/he thinks I ignore what s/he has to say. If you checked more than one box, your family member or friend probably has problems with speech and communicating. Perceptually, speech and voice in people with PD are characterized by • reduced loudness, • Collectively, these • monopitch, speech symptoms are • monoloudness, called hypokinetic • reduced stress, • breathy, dysarthria • hoarse voice quality, • Voice problems are • imprecise articulation, • short rushes of speech, typically the first to • and hesitant and occur, with other dysfluent speech problems, such as prosody, articulation and fluency, gradually appearing as the speech disorder progresses TREATMENT Lee slivervoice treatment One therapy, that has proven effective in treating patients with neurological disorders, specifically Parkinson’s Disease, is the Lee Silverman Voice Treatment (LSVT®). Based upon nearly 15 years of research data, this treatment offers the opportunity to consistently improve speech and voice production in individuals with neurological disorders and significantly improve their quality of life. (https://www.rainbowrehab.com/RainbowVisions/article_dow nloads/articles/Art-THPY-LSVT.pdf) LSVT is the first and only documented efficacious speech treatment for individuals with Parkinson’s disease – over 400 have been treated and included in efficacy research studies. Ninety percent of patients studied showed improvements in vocal intensity from pre- to post-treatment. Approximately 80 percent maintained improvements in volume for 12-24 months post-treatment. Learn appropriate breathing techniques •As breath is the energy behind your voice , weak breath support weak voice •Figure out where your diaphragm is below your lungs, when you breath your torso comes out LSVT Sustained phonation\say a In front of mirror or recording •Open your mouth nice and big say loud and long as you can •Great strengthener, forces your vocal folds stay together in a phonation position and work in muscles Highest and lowest pitch Start at a normal pitch and then glide as high as you can and then as low as you can Rationale With Pd because of the stiffness of muscles, the range of your muscle starts to get narrow and as you get monotone. Good exercise to increase your range super high and super low. To improve Vocal Quality Relaxation exercises •Yawn sigh (reduce the strain) 5 times Expressive phase Facial muscles are stiffening List of expressive words e.g. wow! Exaggerate the expression Take ten sentences or phrases that u use every day / name of people family members practices these activities •Good breath support •Loud voice with •good quality https://www.parkinsons.va.gov/NorthWest/Documents/Pt_ed_handout s/Handout_Swallowing_and_PD_2-8-13.pdf Use it or loss it Inactivity may accelerate deficit Continuous activity may slow disease progression http://www.parkinson.org/sites/default/files/Swallow %20%26%20Dental.pdf Saliva management Saliva is necessary for digestion. It lubricates the throat to make swallowing easier and contains chemicals that break down food. Some people with Parkinson’s develop problems controlling their saliva, which can lead to drooling or dribbling. The medical term for this is sialorrhea. Research shows that this can affect more than half of people with Parkinson’s. • When you have Parkinson’s the natural tendency to swallow slows down. If you swallow less, saliva can pool in your mouth and, instead of being swallowed, it can overflow from the corners of your mouth PARKINSONISM Also called atypical Parkinson's disease Parkinsonism is an umbrella term for patients who have sign and symptoms suggesting that they have got • stiffness in the limbs the limbs that we call rigidity • Bradykinesia- slowness of moment • Shaking or tremors particularly in the hands • That combination of signs and symptoms that we call parkinsonism Causes Lot of different causes for that • Parkinson disease is one of the common cause of the combination of these symptoms. But there are other causes • Certain medication that block dopamine in the brain. • Vascular diseases, or blockage of blood vessels in the brain e.g. after stroke • Combination of other rare degenerative disease • Corticobasal degeneration • Dementia with Lewy bodies • Multiple system atrophy • Progressive supranuclear palsy Differences • Tend to progress more rapidly then pd. • Present with additional symptoms such as early falling, dementia, hallucinations • Not responding to PD drugs levodopa therapy. • Examples • Example 1LBD ( earliest sign memory loss ) • Early retirement no longer manages his meetings and other task other wise multitask • 5 years ago dignosed with dementia • 3 years ago diagnosed with PD because of stiffness in limbs and slowness • Did not response well to medication • Currently hallucinations • Physically looks like he had mild pd walks talks and sustain conversation. • Example 2 PSP • At the age of 60 started falling backwards • Year ago start having double vision • Six months ago diagnosed with pd but does not respond well to medication • Now cannot stand up without falling • Example 3 FTD ( earliest symptom b change) • Year ago 57 started embarrassing his wife by saying odd things at party saying inappropriate things. • When his brother died suddenly he didn’t seem to care which was quite disturbing for the family. • About 6 months ago noted tremor in right hand ( diagnosed with pd) but didn’t response well to medicine L • Friends donot like to talk to him any more / became unfriendly and unappropriated • Currently his wife has to tell him when to bath, eat etc • Extravagant vs lack of motivation Example 4 MAS ( multiple system Atrophy) early S Fainting •Age 52 started having dizziness •see urologist for bladder control issues •1 year ago started fainting and Due to rigidity and stiffness diagnosed with PD •Now on wheel chair due to extreme low BP •Intellect was completely intact( fully functioning in this regard). •L do not work . •Prognosis poor Sources • Aminoff, M. (2003). Parkinson Primer: Overview of Parkinson’s Disease. Retrieved November 16, 2005, from http://www.parkinson.org/site/pp.asp?c=9dJFJLPNB&b=71354. • This source provided me with the most of the background information necessary in explaining the foundation of the disease. This source was especially helpful in determining the characteristic symptoms of the disorder as well as statistics. • Freed, C.R., Green, P.E., Breeze, R.E., Tsai, W., DuMouchel, W., Kao, R., Dillon, S., et al. (1994). Transplantation of Embryonic Dopamine Neurons for Severe Parkinson’s Disease. New England Journal of Medicine, 344, (7), 710-719. • This source played a large part in writing the actual paper. In this article was information on the background of stem cells, implications in stem cell research, and most beneficial, the actual experimental procedure itself. • Lieberman, A. (2004). What is Parkinson’s Disease? Retrieved November 14, 2005, from http://www.pdcaregiver.org/WhatIsParkinsons.html. • This source didn’t help much background information on the disease, but did help in providing an comprehendable version of the substantia nigra and its role in development of Parkinson’s disease. Also beneficial were the figures associated with this source. • Dr Georgia lea lectures.