The document discusses several neurodegenerative disorders including Parkinson's disease, Huntington's disease, Alzheimer's disease, amyotrophic lateral sclerosis, muscular dystrophies, herniation of intervertebral disks, and degenerative disk disease. The causes, signs and symptoms, pathophysiology, assessments, and management strategies are described for each condition. Neurodegenerative disorders are characterized by the slow deterioration of cells in the nervous system leading to progressive loss of function.

1. D i D s e a s e s e g e n e r a i t e v

2. Disorders of the central and peripheral nervous system that are neurodegenerative characterized by slow onset of signs and symptoms. Signs and symptoms that leads to the deterioration of normal cells or function of the nervous system. Patients are mainly managed at home for as long as possible and are admitted to the acute care setting for exacerbations, treatments, and surgical interventions as needed. Degenerative Disorders

3. Parkinson’s Disease (PD) Huntington’s Disease (HD) Alzheimer’s Disease (AD) Amyotrophic Lateral Sclerosis (ALS) Muscular Dystrophies Degenerative Disk Disease (DDD) Herniation of a Cervical Intervertebral Disk Herniation of a Lumbar Disk The Disorders Include:

4. Parkinson’s Disease (PD)

5. Parkinson's disease is a disorder that affects nerve cells in the part of the brain controlling muscle movement. People with Parkinson's disease often experience trembling, muscle rigidity, difficulty walking, problems with balance and slowed movements. Symptoms usually develop after age 60, although some people affected by Parkinson's disease are younger than age 50. Although Parkinson's disease may eventually be disabling, the disease often progresses gradually, and most people have many years of productive living after a diagnosis. Parkinson’s Disease

6. Most people with Parkinson's disease are described as having idiopathic PD (having no specific cause). There are far less common causes of PD including genetic, toxins, head trauma, cerebral anoxia, and drug-induced PD. Causes

7. Past episodes of head trauma are reported more frequently by sufferers than by others in the population. A methodologically strong recent study found that those who have experienced a head injury are four times more likely to develop Parkinson’s disease than those who have never suffered a head injury. Head Trauma

8. Antipsychotics, which are used to treat schizophrenia and psychosis, can induce the symptoms of Parkinson's disease (or parkinsonism) by lowering dopaminergic activity. Drug Induced PD

9. The symptoms of Parkinson's disease result from the loss of pigmented dopamine-secreting (dopaminergic) cells, secreted by the same cells, in the pars compacta region of the substantia nigra These neurons project to the striatum and their loss leads to alterations in the activity of the neural circuits within the basal ganglia that regulate movement, in essence an inhibition of the direct pathway and excitation of the indirect pathway Pathophysiology of PD

10. The direct pathway facilitates movement and the indirect pathway inhibits movement, thus the loss of these cells leads to a hypokinetic movement disorder. The lack of dopamine results in increased inhibition of the ventral lateral nucleus of the thalamus, which sends excitatory projections to the motor cortex, thus leading to hypokinesia. Pathophysiology of PD

11. Clinical Manifestations Tremors Bradykinesia Rigid Muscles Impaired Balance Dementia Difficulty Swallowing Impaired Speech Loss of Automatic Movements

12. Medications Anticholinergics Cathechol – o – Methyltransferase Selegiline Eldepryl Dopamine Agonist Levodopa

13. Surgical Management Pallidotomy Deep Brain Stimulation Thalatomy

14. Physical Exercise and Speech Therapies

15. Difficulty chewing and swallowing. Urinary problems. Constipation Sleep problems Sexual dysfunction Complications

16. Provide information and support regarding Parkinson’s Disease. Health teaching, diet, high in fiber, increase oral fluid intake to prevent constipation. Promote relaxation exercises to promote proper sleep. Improving mobility. Enhancing self-care activities. Improving bowel elimination Improving nutrition. Enhancing swallowing. Nursing Management

17. Huntington’s Disease (HD)

18. Huntington’s disease is a chronic, progressive, hereditary disease of the nervous system that results in progressive involuntary choreinform movement and dementia. It is transmitted as an autosomal dominant genetic disorder. Also called Huntington's chorea, “chorea” comes from the greek word meaning “to dance” and refers to the incessant quick, jerky, involuntary movements that are characteristic of this condition. Each child of a parent with Huntington’s disease has a 50% risk of inheriting the illness. It affects men and women. Huntington’s Disease

19. Huntington's disease is an inherited condition caused by a single abnormal gene. Because only one copy of the defective gene, inherited from either parent, is necessary to produce the disease. Because signs and symptoms typically first appear in middle age, some parents may not know they carry the gene until they've already had children and possibly passed on the trait. Cause of HD

20. Pathophysiology involves premature death of cells in the stiatum (caudate and putamen of the basal ganglia, which involve the control movement. There is also loss of cells in the cortex, it is the region of the brain associated with thinking, memory, perception, and judgement and also the cerebellum that coordinates voluntary muscle activity. Building block for protein called gluthamine abnormally collects in the nucleus, causing cell death. The cell’s destruction results in a lack of the neurotransmitters gamma-aminobutyric acid. This is usually occurs in ages between 35 to 45 years. Pathophysiology of HD

21. CT scan and MRI may show atrophy of the Caudate Nuclei once the disease is well established. A genetic marker for Huntington’s disease has been identified through the use of recombinant DNA technology. As a result, researchers can now identify presymptomatic individual who will develop this disease. Assessment and Diagnosis

22. Abnormal involuntary movements (chorea), intellectual decline, and emotional disturbances. Constant writhing and uncontrollable movements of the entire body occur as this disease progresses. Facial movements produce ticks and grimaces; speech becomes slurred, hesitant, open explosive, and then eventually unintelligible. Chewing and swallowing are difficult, and aspiration and choking are dangers. Gait becomes disorganized, and ambulation is eventually impossible; patient is eventually confined to a wheelchair. Clinical Manifestations

23. Medications Tranquilizers such as clonazepam (Klonopin) and antipsychotic drugs such as haloperidol (Haldol) and clozapine (Clozaril) can help control movements, violent outbursts and hallucinations. While these medications can be helpful, a common side effect is sedation, and in some cases, these medications may cause additional stiffness and rigidity. Various medications, including fluoxetine (Prozac, Sarafem), sertraline (Zoloft) and nortriptyline (Aventyl, Pamelor), can help control depression and the obsessive-compulsive rituals that some people with Huntington's disease develop. Medications such as lithium (Eskalith, Lithobid) can help control extreme emotions and mood swings. Management

24. Physical therapy can help keep muscles stronger and more flexible, which helps maintain balance and may lessen the risk of falling. Occupational therapy can help make your home safer and give you strategies for coping with memory and concentration problems. Later in the disease, occupational therapy can assist you with eating, dressing and hygiene challenges. Physical and Occupational Therapy

25. Though the signs and symptoms vary from person to person, vital functions, such as swallowing, eating, speaking and walking, usually degenerate over time. Many people with Huntington's disease develop depression, and some are at risk of suicide. However, death generally occurs as a result of complications of the disease, such as an infection or a fall. Complications

26. Reinforcement the understanding that Huntington’s disease takes emotional, physical, social, and financial tolls on every member of the patient’s family. Encourage genetic counseling, long term psychological counseling, marriage counseling, and financial and legal support. Teach patient and family about the medication, including signs indicating the need for change in dosage or medication. Nursing Management

27. Address strategies to manage symptoms (chorea, swallowing problems, ambulation problems, or altered bowel or bladder function). Emphasize the need for regular follow-up. Refer for home care nursing assistance, respite care, day care centers, and eventually skilled long-term care to assist patient and family cope. Provide information about the Huntington’s Disease Foundation of America, which gives information, referrals, education, and support for reaserch. Nursing Management

28. Alzheimer’s Disease (AD)

29. Alzheimer's disease is the most common cause of dementia, which is the loss of intellectual and social abilities severe enough to interfere with daily functioning. Dementia occurs in people with Alzheimer's disease because healthy brain tissue degenerates, causing a steady decline in memory and mental abilities. Alzheimer’s Disease

30. The causes of Alzheimer's are poorly understood, but its effect on brain tissue has been demonstrated clearly. Alzheimer's damages and kills brain cells. Studies of plaques and tangles from the brains of people who have died of Alzheimer's suggest several possible roles these structures might play in the disease. Cause of AD

31. Plaques are made up of a normally harmless protein called beta-amyloid. Although the ultimate cause of neuron death in Alzheimer's isn't known, mounting evidence suggests that a form of beta-amyloid protein may be the culprit. Neural Plaques and AD

32. Medical History Basic Medical Test (e.g. blood tests) Mental Status Evaluation Neuropsycological Testing Brain Scans Assessment and Diagnosis

33. Stage 1. Mild memory lapses Stage 2. Obvious short- term memory lapses Stage 3. Disintegrations of personality Stage 4. Terminal Stage – physical and mental deterioration AD’s Different Stages

34. Clinical Manifestations Disorientation Loss of Judgement Difficulty Finding The Right Word Difficulty Performing Familiar Tasks Difficulty with Abstract Thinking Personality Changes Increasing and Persistent Forgetfulness

35. Currently, there is no cure for AD. Medications are prescribed to offer relatively small symptomatic relief. Management of AD

36. This group of medications, which includes donepezil (Aricept), rivastigmine (Exelon) and galantamine (Reminyl) works by improving the levels of neurotransmitters in the brain. Side effects include diarrhea, nausea, and vomiting. Cholinesterase Inhibitors

37. The first drug approved to treat moderate to severe stages of Alzheimer's, memantine (Namenda), protects brain cells from damage caused by the chemical messenger glutamate. It sometimes is used in combination with a cholinesterase inhibitor. Most common side effect is dizziness, and may increase agitation and delusional behavior. Memantine (Namenda)

38. Amyotrophic Lateral Sclerosis (ALS)

39. Amyotrophic lateral sclerosis (ALS) is a serious neurological disease that attacks the nerve cells that control voluntary muscles. It may begin with muscle twitching or weakness in an arm or leg, or with slurring of speech. Eventually, amyotrophic lateral sclerosis affects your ability to control the muscles needed to move, speak, eat and breathe. Amyotrophic Lateral Sclerosis

40. Amyotrophic lateral sclerosis is a disease that involves death of motor neurons, the nerve cells that control voluntary muscles. In ALS, both upper motor neurons, located in your brain, and lower motor neurons, located in your spinal cord, gradually die. ALS is an inherited disease. The other type of ALS appears to occur randomly with no identifiable cause and no obvious risk factors. Cause of ALS

41. Difficulty lifting the front part of your foot (footdrop) Weakness in your leg, feet or ankles Hand weakness or clumsiness Slurring of speech or trouble swallowing Muscle cramps and twitching in your arms, shoulders and tongue Fatigue in combination with the above signs and symptoms Eventually, amyotrophic lateral sclerosis paralyzes the muscles needed to breathe. Most people with ALS die of respiratory failure, usually within three to five years after symptoms begin. Clinical Manifestations

42. Currently, there is no treatment for ALS. Treatment of amyotrophic lateral sclerosis typically focuses on efforts to relieve symptoms and maintain quality of life in the years after diagnosis. May need tube feedings Activity as tolerated Dantrolene (Dantrium) or baclofen (Lioresal) for muscle spasticity I.V. or intrathecal administration of thyrotropin-releasing hormone Riluzole (Rilutek) to delay progression of disease Treatment and Management

43. Muscular Dystrophies

44. Muscular dystrophy (MD) is a group of inherited muscle diseases in which muscle fibers are unusually susceptible to damage. Muscles, primarily voluntary muscles, become progressively weaker. In the late stages of muscular dystrophy, fat and connective tissue often replace muscle fibers. Some types of muscular dystrophy affect heart muscles, other involuntary muscles and other organs. Muscular Dystrophies

45. Muscular dystrophy is a general term for a group of inherited diseases involving a defective gene. Each form of muscular dystrophy is caused by a genetic mutation that's particular to that type of the disease. The most common types of muscular dystrophy appear to be due to a genetic deficiency of the muscle protein dystrophin. Causes of Muscular Dystrophies

46. Muscle weakness Apparent lack of coordination Progressive crippling, resulting in fixations (contractures) of the muscles around your joints and loss of mobility Weakening of voluntary muscles that control your arms and legs, usually beginning with the limb muscles farthest from the torso — the muscles of the feet, hands, lower legs and forearms. Clinical Manifestation

47. There are currently no cures for any forms of muscular dystrophies. Treatment and management are aimed to decrease the severity of symptoms and reduce deformities brought about by the disease. Physical Therapy Assistive Devices Surgery, to release contractures that may develop and that can position joints in painful ways. Anti-inflammatory corticosteroids Medications to manage muscle spasms Treatment and Management

48. Herniation of A Intervebral Disk

49. Low back pain is a significant public health disorder. Acute low back pain lasts less than 3 months, whereas chronic or degenerative disk disease lasts 3 months or longer. Cervical disc herniations occur in the neck, most often between the sixth and seventh cervical vertebral bodies. Symptoms can affect the back of the skull, the neck, shoulder girdle, scapula, shoulder, arm, and hand. The nerves of the cervical plexus and brachial plexus can be affected. Herniation of an Intervertebral Disk

50. Lumbar disk herniations occur in the lower back, most often between the fourth and fifth lumbar vertebral bodies or between the fifth and the sacrum. Symptoms can affect the lower back, buttocks, thigh, and may radiate into the foot and/or toe. The sciatic nerve is the most commonly affected nerve, causing symptoms of sciatica. Herniation of an Intervertebral Disk

51. The intervertebral disk is a cartilaginous plate that forms a cushion between the vertebral bodies. This tough, fibrous material is incorporated in a capsule. A ball like cushion in the center of the disk is called the nucleus pulposus . In herniation, the nucleus of the disk protrudes into the annulus, with subsequent nerve compression. Pathophysiology

52. Protrusion or rupture of the nucleus pulposus usually is preceeded by degenerative changes that occur with changing. Loss of protein polysaccharides in the disk decreases the water content of the nucleus pulposus. The development of radiating cracks in the annulus weakens resistance to nucleus heriation. Pathophysiology

53. Chief complaint is leg pain greater than back pain. Intense pain in the hip joint from a lumbar disk herniation. Unrelenting neck or lower back pain. Numbness Tingling Muscular weakness Paralysis Paresthesia Affection of reflexes Sciatica Clinical Manifestations

54. Majority of herniated disks will heal themselves in about six weeks and do not require surgery. If pain due to disc herniation, protrusion, bulge, or disc tear is due to chemical radiculitis pain, then prior to surgery it may make sense to try an anti-inflammatory approach. Often first attempted with NSAIDs. Epidural steroid injection for short term relief of pain Bad rest and lumbo-sacral support belt Physical therapy Osteopathic / chiropractic manipulations Massage therapy Oral steroids (for pain) Treatment and Management

55. Anterior cervical discectomy and fusion Laminectomy Hemilaminectomy Lumbar fusions Disk arthroplasty Artificial disk replacement Surgical Options

56. Degenerative Disk Disease (DDD)

57. Degeneration of the intervertebral disk, which is often called " degenerative disk disease " (DDD) of the spine, DDD is in fact a condition that can be painful and can greatly affect the quality of one's life. While disk degeneration is not a normal part of aging and for most people is not a problem, for certain individuals a degenerated disk can cause severe constant chronic pain. Degenerative Disk Disease (DDD)

58. Trauma micro or macro as a simple lifting accident is the most common cause of disc degeneration. After trauma the discs in the spine dehydrate, or dry out, and lose their ability to act as shock absorbers between the vertebrae. There is minimal blood supply to the discs so they lack the ability to heal or repair themselves. Causes of DDD

59. Most DDD can be successfully treated without surgery. One or combinations of physical therapy, osteopathy, anti-inflammatory medications, chiropractic treatments, or spinal injections. Anterior cervical discectomy and fusion Cervical corpectomy Facetectomy Foraminotomy Laminoplasty Laminotomy Micro-discectomy Spinal laminectomy Treatment and Management

60. Thanks For Listening…

61. DR. ANTHONY TOLEDO