PARKINSON’S DISEASE ( Idiopathic Parkinsonism or Paralysis Agitans )

1. PARKINSON’S DISEASE(Idiopathic Parkinsonism or Paralysis Agitans)

2. PARKINSON’S DISEASE • Parkinson's disease (PD or, simply, Parkinson's) is the most common form of Parkinsonism, a group of motor system disorders. • Named after James Parkinson. a British physician who first described the disease in a paper he published as the “shaking palsy” in 1817

3. It is a slowly progressing, degenerative neurologic disease that is usually associated with the following symptoms, • tremor or trembling of the arms, jaw, legs, and face • stiffness or rigidity of the limbs and trunk • bradykinesia (slowness of movement) • postural instability, or impaired balance and coordination

4. The primary symptoms are the results of decreased stimulation of the motor cortex by the basal ganglia, normally caused by the insufficient formation and action of dopamine, which is produced in the dopaminergic neurons of the brain. Secondary symptoms may include high level cognitive dysfunction and subtle language problems. PD is both chronic and progressive. • Dopamine is a substance produced in the body which has many effects, including smooth and coordinated muscle movement.

5. Prevalence and Incidence • 500,000 – currently suffer PD in USA • 50,000- new cases are reported each year • Males are more affected than females • Symptoms begin between 40 and 70 years of age with a peak onset on the 60’s.

6. Risk Factors • The specific causeof PD is unknown; however, medical experts believe the symptoms are related to a chemical imbalance in the brain caused by brain-cell death. • Advancing age. The average age for the onset of PD is 60 years. • Gender. Fifty percent more men are affected than women, according to the National Institute of Neurological Disorders and Stroke. However, the reason for this is unclear. • Family history. Individuals with a parent or sibling who are affected have approximately two times the chance of developing PD.

7. Two genes which are linked to PD a. synuclein- a member of a small family of proteins that are expressed preferentially in the substantia nigra. One of the major components of the Lewy bodies that are found in brain tissues of persons with PD. b. Parkin genes- encoding the protein parkin was linked to an autosomal recessive form of PD.

8. Risk Factors • Environmental causes are being researched and the strong consistent findings are that rural living, exposure to well water, and exposure to agricultural pesticides and herbicides are related to PD. Currently researchers believe that in most individuals the cause of PD is a combination of genetics and environmental exposure. *endotoxin or lipopolysaccharide – a common airborne environmental and occupational contaminate in agriculture and other industries.

9. Parkinson's syndrome, Atypical Parkinson's, or Parkinsonism: • tumors in the brain • repeated head trauma • drug-induced parkinsonism - prolonged use of tranquilizing drugs, such as the phenothiazines, butyrophenones, reserpine, and the commonly used drug, metoclopramide for stomach upset. • toxin-induced parkinsonism - manganese and carbon monoxide poisoning. • postencephalitic parkinsonism - a viral disease that causes "sleeping sickness." • striatonigral degeneration - the substantia nigra of the brain is only mildly affected, while other areas of the brain show more severe damage. • parkinsonism that accompanies other neurological conditions - such as Shy-Drager syndrome (multiple system atrophy), progressive supranuclear palsy, Wilson's disease, Huntington's disease, Hallervorden-Spatz syndrome, Alzheimer's disease, Creutzfeldt-Jakob disease, olivopontocerebellar atrophy,  post-traumatic encephalopathy, and dementia with Lewy bodies.

10. Four primary symptoms of Parkinson The following are the most common symptoms of Parkinson's disease. However, each individual may experience symptoms differently. Symptoms may include: • Muscle rigidity - stiffness when the arm, leg, or neck is moved back and forth. • Resting tremor - tremor (involuntary movement from contracting muscles) that is most prominent at rest. • Bradykinesia - slowness in initiating movement. • Postural instability - poor posture and balance that may cause falls; gait or balance problems.

11. Other symptoms of Parkinson's disease (PD): • Symptoms of Parkinson's disease vary from patient to patient. The symptoms may appear slowly and in no particular order. Early symptoms may be subtle and may progress over many years before reaching a point where they interfere with normal daily activities. • The four cardinal symptoms of PD are listed above. Other symptoms are divided into motor (movement related) and nonmotor symptoms.

12. Gait and posture disturbances • Shuffling gait • Decreased arm-swing. • Turning "en bloc" • Stooped, forward-flexed posture • Festination • Gait freezing • Dystonia (in about 20% of cases) Speech and swallowing disturbances • Hypophonia • Monotonic speech. • Festinating speech • Drooling • Dysphagia

13. Other motor symptoms: • Fatigue (up to 50% of cases); • Masked faces (a mask-like face also known as hypomimia), with infrequent blinking; • Difficulty rolling in bed or rising from a seated position; • Micrographia (small, cramped handwriting); • Impaired fine motor dexterity and motor coordination; • Impaired gross motor coordination; • Akathisia, the inability to sit still.

14. Cognitive disturbances include: • Slowed reaction time; both voluntary and involuntary motor responses are significantly slowed. • Executive dysfunction • Dementia • Hallucinations, delusions and paranoia may develop. • Short term memory loss; procedural memory is more impaired than declarative memory. Prompting elicits improved recall. • Non-motor causes of speech/language disturbance in both expressive and receptive language: these include decreased verbal fluency and cognitive disturbance especially related to comprehension of emotional content of speech and of facial expression. • Medication effects: some of the above cognitive disturbances are improved by dopaminergic medications, while others are actually worsened.

15. Sleep • Excessive daytime somnolence • Initial, intermediate, and terminal insomnia • Disturbances in REM sleep: disturbingly vivid dreams, and rapid eye movement behavior disorder, characterized by acting out of dream content — can occur years prior to diagnosis

16. Perception • Impaired visual contrast sensitivity, spatial reasoning, colour discrimination, convergence insufficiency (characterized by double vision) and oculomotor control • Dizziness and fainting; usually attributable orthostatic hypotension, a failure of the autonomic nervous system to adjust blood pressure in response to changes in body position • Impaired proprioception (the awareness of bodily position in three-dimensional space) • Reduction or loss of sense of smell (hyposmia or anosmia) pain: neuropathic, muscle, joints, and tendons, attributable to tension, dystonia, rigidity, joint stiffness, and injuries associated with attempts at accommodation

17. Autonomic • Oily skin and seborrheic dermatitis • Urinary incontinence • Nocturia— up to 60% of cases • Constipation and gastric dysmotility • Altered sexual function • Weight loss, which is significant over a period of ten years.

18. As the disease progresses, walking may become affected, causing the patient to stop in mid-stride or "freeze" in place, and maybe even fall over. Patients also may begin walking with a series of quick, small steps as if hurrying forward to keep balance, a practice known as festination.

19. Risk/Related Factors Age, Genetics, Drugs, Toxins,Headtrauma Destruction of dopaminergic neuronal cells in the substantia nigra in the basal ganglia Depletion of dopamine stores Degeneration of the Dopaminergic nigrostriatal pathway Imbalance of Excitatory (acethylcholine) and inhibiting (dopamine) neurotransmitters in the corpus striatum Impairment of extre pyramidal tracts controlling complex body movements Tremors, Rigidity, Bradykinesia

20. Diagnosis

21. Neurological examination (including evaluation of symptoms and their severity) • Trial test of drugs - when symptoms are significant, a trial test of drugs (primarily levodopa [L-dopa]) may be used to further diagnose the presence of PD. If a patient fails to benefit from levodopa, a diagnosis of Parkinson's disease may be questionable. • Computed tomography scan (Also called a CT or CAT scan.) • Magnetic resonance imaging (MRI)

22. Other Tests to Roll out Secondary Causes • Drug Sreening – to determine the presence of medications or toxins • Upper GI Series – shows delayed emptying, distention and possibly megacolon with severe constipation • CBC – shows low hemoglobin and hematocrit due to anemia • Chemistry Profile – reflect low protein and albumin levels related to the clients inability to buy and prepare meals

23. Medication for Parkinson's disease:

24. a. OLDER DRUGS • Anti-cholinergic • Dopamine Agonist • Levadopa Combinations (levadopa and carbidopa ) • Amantadine (Symmetrel) b.NEWER DRUGS 1. Cathechol o-methyltranferase (COM) inhibitor 1.1. Entacapone (Contan)-used in combination of levadopa and carbidopa. 1.2 Stavelo – a combination of levodopa,cabidopa and entacapone. 1.3 Tolcapone (Tasmar) 2. Monoamine oxidase (MAO) inhibitors Selegiline(Eldepryl) - may be prescribed to confer mild symptomatic benefit before initiating dopaminergics in the treatment of PD. Ex. Carbex, Eldepryl, Novo-Selegiline - reduce metabolic breakdown of dopamine c. INVESTIGATIONAL DRUGS 1. Coenzyme Q10 – may slow the rate of functional declines 2. Dextromethorphan glial – derived neurotrophic factor --- administered directly into the brain.

25. B. Surgical Treatment • Lesion surgery (burning of tissue) • Deep brain stimulation (DBS) • Neural grafting or tissue transplants • Pallidotomy and thalamotomy are rarely done anymore. They involve the precise destruction of very small areas in the deep part of the brain that cause symptoms. • Neurotransplantationsurgery • Fetal Tissue Transplantation is an experimental and highly controversial procedure. Fetal substantia nigra tissue either human or pig is transplanted into the caudate nucleus of the brain.

26. 1. Impaired Physical Mobility related to neuromuscular impairment a. Daily exercise increases muscle strength, improve coordination and dexterity, reduce muscular rigidity and prevent contractures. b. Stretching and ROM exercise to promote joint flexibility. c. Postural exercises are important to counter the tendency of the head and neck to be drawn forward and down. d. Warm baths and massage help relax the muscle and relieve painful muscle spasms that accompany rigidity.

27. 2. Self- Care Deficit related to tremor and motor disturbance • a. Encourage, teach and support the patient during ADL’s to promote self care. • b. Assist and encourage good grooming to enhance independence and self esteem.

28. 3. Impaired Verbal Communication related to neuromuscular impairment a. Instruct client to speak softly and clearly and to pause and take a deep breath at appropriate intervals during each sentence. b. Eliminate unnecessary environmental noise to maximize the listener’s ability to hear and understand the client. c. Asked client to repeat words that the listener does not understand and the listener watches the client’s lips and non-verbal expressions for cues as to the meaning of conversation. d. Instruct client to organize his/her thoughts before speaking and to use facial expression and gestures if possible to assist with communication. e. Collaborate with the speech language pathologist, health care team, client and family in developing a communication plan. f. If client cannot communicate verbally, the use of alternative methods of communication such as communication board or computer must be encouraged.

29. 3. Imbalanced Nutrition less than body requirements related to inability to ingest food due to biologic factors a. Smaller, more frequent meals or commercial powder added to liquids may assist the client who has difficulty swallowing. b. Position client with head elevated to facilitate swallowing and prevent aspiration. d. The client should be weighed once a week and adjustment should be made on diet as indicated. e. Record food intake daily or as indicated. f. As disease progresses and swallowing becomes more of a problem, supplemental feedings become the main source of nutrition to maintain weight. g. Coordinate with a registered dietician to evaluate the client’s food intake. h. If the client has difficulty swallowing coordinate with speech language pathology to conduct an extensive swallowing evaluation in order to develop an individualized dietary plan.

30. 4. Constipation related to medication and decreased activity a. Encourage a patient to follow regular mealtime pattern to establish regular bowel routine. b. Increase fluid intake. c. Eat foods with a moderate fiber content.