Degenerative Diseases

1. Degenerative Diseases Multiple Sclerosis Parkinson’s Alzheimer’s Myasthenia Gravis Amiotrophic Lateral Sclerosis Huntington’s Disease Tourette’s Syndrome

2. Degenerative Diseases • “Degenerative Diseases” refers to neurological disorders in which there is a premature aging of nerve cells • Caused by suspected metabolic disturbance or unknown cause

3. Multiple Sclerosis • Multiple Sclerosis • Chronic, progressive • Cause is unknown; genetic factor suspected due to the higher rate among relatives • Something goes wrong and induces the T cells (normally the “field commanders” of the immune system) to attack the body  myelin damage occurs

4. Multiple Sclerosis • A viral infection may be the beginning mechanism, but a defective immune response seems to have an important role in the pathology • People living in temperate climates have an increased risk of the disease

5. Multiple Sclerosis • Pathology: multi-foci of demyelinationare distributed randomly in the white matter of the brainstem, spinal cord, optic nerves, and cerebrum  causes an interruption or distortion of the nerve impulse • There is some evidence of partial healing in areas of degeneration

6. Multiple Sclerosis • Clinical Manifestations: • Onset is insidious and gradual • Vague symptoms that occur intermittently over months or years • Visual problems; urinary incontinence; fatigue, weakness, incoordination of an extremity; swallowing difficulty; sexual problems

7. Multiple Sclerosis • Assessment: • Subjective data: pt. c/o eye problems, weakness or numbness of a part of the body; fatigue, emotional instability, bowel/bladder problems; vertigo; loss of joint sensation; involvement of the cerebellum  ataxia and tremor • Objective data: nystagmus, muscle weakness and spasms; changes in coordination; dysarthria, dysphagia; urinary incontinence; emotional instability

8. Multiple Sclerosis • Medical Management: • No specific treatment • Favorable results with: corticosteroids; prednisone, ACTH; dexamethasone • Valium to treat muscle spasms • Interferon reduces frequency of exacerbations; slows progress of physical disability • Interferon is a low molecular wt. protein that regulates the extent and duration of the immune and inflammatory response

9. Multiple Sclerosis • Nursing Interventions: • Nutrition: well balanced diet; high fiber • Skin Care: frequent turning; pressure-relief devices • Activity: exercise regularly; avoid fatigue; daily rest periods • Control of Environment: air-conditioned surroundings during hot weather; peaceful, relaxed setting

10. Multiple Sclerosis • Patient Teaching: • Environmental control • 24 hr care in late stages of disease; need caregiver • Support • Prognosis: • Average life expectancy after the onset of symptoms is > 25 years

11. Parkinson’s Disease • A syndrome that includes: • A slowing down in the initiation and execution of movement (bradykinesia) • Increased muscle tone (rigidity) • Impaired postural reflexes

12. Parkinson’s Disease • Involves: • Damage or loss of the dopamine-producing cells in the midbrain  depletion of the dopamine that influences the initiation, modulation, and completion of movement

13. Parkinson’s Disease • Disease shows no gender, socioeconomic, or cultural preference • Symptoms commonly occur after 50 years old • Many causes: encephalitis, chemical toxins, drug-induced (Aldomet, Haldol, Thorazine) • Signs/symptoms: tremors, muscle rigidity, slowed movements, impaired balance

14. Parkinson’s Disease • Clinical Manifestations: • Beginning stages: mild tremor, slight limp, decreased arm swing • Later: shuffling gait, arms flexed, loss of postural reflexes; slight changes in speech pattern • Diagnosis based solely on the hx., neurological exam, and clinical features • Dementia occurs in approx. 40% of pts.

15. Parkinson’s Disease • Assessment: • Subjective data: c/o fatigue, incoordination of movement, judgment defects, emotional instability, heat intolerance • Objective data: presence of tremor (pill-rolling style), bradykinesia, muscle rigidity; mask-like facial appearance, drooling; swallowing may be abnormal

16. Parkinson’s Disease • Diagnostic Tests: • No specific dx. test for Parkinson’s Firm dx. can be made when 2 of the 3 classic triad of symptomspresent: tremor, rigidity, bradykinesia • Dx. Confirmed with clinical exam and history • CT scan may show cerebral atrophy Ultimate confirmation of dx. is a positive response to antiparkinsoniandrugs

17. Parkinson’s Disease • Medical management: • Goal: to ease the s/sx of the disease • Medications: esp. Carbid-levo (Sinemet) • Surgery: • Ablation surgery: destroying portions of the brain that control the rigidity or tremor • Deep brain stimulation: electrode placement connected to a generator implanted in the chest. A specific current is delivered to the targeted brain location  improved motor performance and gait

18. Parkinson’s Disease • Nursing Interventions: • Activity needs: pay special attention to posture • Ambulation: when “freezes up”, usually need prn medication; can also teach techniques such as stepping over pretend or real lines on the floor; avoid hurrying the patient • Nutrition: easy to chew and swallow foods; cut into bite-size pieces; 6 small meals vs 3 large; aspiration precautions; don’t hurry.

19. Parkinson’s Disease • Nursing Interventions: • Elimination: toileting schedule and prn sense of urgency; may also experience urinary hesitancy • Chronic constipation: high fiber diet; encourage oral fluid intake; stool softeners, prune juice; mild cathartics/laxatives • Patient Teaching: Medication schedule, skin care, keeping active; safe ambulation and positioning; proper feeding techniques

20. Alzheimer’s Disease • Chronic, progressive, degenerative disorder that affects the cells of the brain and causes impaired intellectual functioning • Possible genetic link • Changes in the brain include “plaques” in the cortex and “neurofibrillary tangles” ( a tangled mass of non-functioning neurons)  decrease in brain size

21. Alzheimer’s Disease • Clinical manifestations: • 4 stages: • Early: mild memory lapses; difficulty using the correct word; decreased attention span; disinterest in surroundings • 2nd stage: increased memory lapses – esp. short-term memory; disorientation; “loses” personal belongings; confabulation; loss of ability to recognize familiar faces; loss of impulse control  agitation

22. Alzheimer’s Disease • 3rd stage: total disorientation, motor problems; unable to recognize objects; difficulty carrying out ADLs; wandering • Terminal Stage: severe mental and physical deterioration; total incontinence

23. Alzheimer’s Disease • Assessment: usually characterized by: • Memory loss • Inability to carry out normal activities • Diagnostic Tests: CT or PET scan, EEG, MRI may be used to rule out other pathologic conditions. Family hx. of Alzheimer’s is significant

24. Alzheimer’s Disease • Medical Management • Options are limited • To treat agitation: small doses of Lorazepam (Ativan) or haloperidol (Haldol) • Mild cognitive impairment may respond to Donepezil (Aricept) reducing the progression of AD; Memantine (Namenda)slows symptoms of moderate to severe AD

25. Alzheimer’s Disease • Nursing Interventions/Patient Teaching • Maintaining adequate nutrition/hydration • Safety • Support with ADL • Education usually directed to the family

26. Amyotrophic Lateral Sclerosis

27. Amyotrophic Lateral Sclerosis • Rare, progressive neurological disease  death in 2-6 years • Also known as Lou Gehrig’s disease • Motor neurons in the brainstem and spinal cord gradually degenerate Chemical and electrical messages originating in the brain do not reach the muscles to activate them • Primary symptoms: weakness of the UE, dysarthria, and dyphagia

28. Amyotrophic Lateral Sclerosis • Death usually results from respiratory infection secondary to compromised respiratory function • No cure • Medication: Rilutek slows the progression • Support and teaching provided by OT, ST, PT, RD, RN, and psychological support • Nursing care is to support patient’s cognitive function, provide diversional activities, help pt. and family do future planning and anticipatory grieving

29. Huntington’s Disease

30. Huntington’s Disease • A genetically transmitted autosomal dominant disorder • Affects men and women of all races • Offspring have 50% chance of inheriting it (diagnosis often occurs after the affected individual has had children)

31. Huntington’s Disease • Pathology: affects the basal ganglia and extrapyramidal motor system • Overactivity of the dopamine pathway • Clinical Manifestations: abnormal and excessive involuntary movements (chorea) • Writhing, twisting movements of the face, limbs, and body. • Speech, chewing, and swallowing are affected  malnutrition and aspiration

32. Huntington’s Disease • Clinical Manifestations cont. • Gait deteriorates  ambulation difficulties  wheelchair bound • Mental function declines • Emotional lability • Psychotic behavior • Death usually occurs 10-20 yrs after the onset

33. Huntington’s Disease • No cure • Therapeutic Management is palliative • Medications: antipsychotic, antidepressant, and antichorea

34. Huntington’s Disease • Nursing Interventions: • Goal: provide the most comfortable environment possible for the patient and the family • Maintain physical safety • Treat physical symptoms • Provide emotional and psychological support • Nutritional / caloric needs • Genetic counseling for the family

35. Tourettes Syndrome

36. Tourettes Syndrome • A neurological disorder characterized by repetitive, stereotyped, involuntary movements and vocalizations called tics. • Cause is unknown • Early Symptoms: • Noticed first in childhood • Males affected 3-4x more than females

37. Tourettes Syndrome • Symptoms: simple or complex • Simple motor tics: sudden, brief, repetitive movements that involve a limited number of muscle groups • E.g. eye blinking, facial grimacing, shoulder shrugging • Complex tics: involve several muscle groups • E.g. facial grimacing combined with a head twist and a shoulder shrug

38. Tourettes Syndrome • Characteristics: • Tics are often worse with excitement; better during calm, focused activities • Tics come and go over time and vary in type, frequency, location and severity • Tics peak in severity before mid-teen years; improvement for the majority of patients in late teen and early adulthood

39. Tourettes Syndrome • Diagnosis: made after verifying that the patient has had both motor and vocal tics for at least 1 year. • Neuroimaging tests and lab work may be used to rule out other conditions that might be confused with TS

40. Tourettes Syndrome • Treatment: • Medication for those whose tics interfere with functioning – e.g. neuroleptics (e.g.Thorazine) • Psychotherapy • Prognosis: no cure; tics tend to decrease with age; some become symptom-free for a period of time. Neurobehavioral disorders may hamper normal functioning in adulthood.

41. Miscellaneous Slides

42. Myasthenia Gravis • An autoimmune disease of the neuromuscular junction • Characterized by fluctuating weakness of certain skeletal muscle groups

43. Myasthenia Gravis Nerve impulses fail to pass at the myoneural junction muscle weakness • Caused by an autoimmune process a decreased number of ACh receptor sites interference with impulse transmission to the muscle

44. Myasthenia Gravis • Note: About 25% of pts. with MG have been found to have a thymoma and about 80% have changes in the cellular structure of the thymus gland • Clinical Manifestations: • Ocular MG: drooping of eyelid, double vision • Generalized MG: Skeletal weakness of the extremities, neck, shoulder, hands, diaphragm, vocal cords

45. Myasthenia Gravis Ocular MG General MG Skeletal weakness of the: Extremities Neck Shoulders Hands Diaphragm Vocal cords • Drooping of eyelid • Double vision

46. Myasthenia Gravis • Assessment: • Subjective: pt. understanding of the disease; c/o weakness, double vision; difficulty chewing or swallowing ; presence of bowel or bladder incontinence • Objective: documented muscle weakness on neurological assessment; nasal-sounding speech; drooping eyelids; weight loss with swallowing problems

47. Myasthenia Gravis • Diagnostic Tests: • Have the pt. look upward for 2-3 min. MG confirmed if increased droop of eyelids so that the person can barely keep the eyes open • EMG • Lab tests: serum testing for antibodies to acetylcholine receptors • IV infusion of a short-acting cholinesterase

48. Myasthenia Gravis • Medical Management • Use of medications: • Anticholinesterase drugs: Prostigmine, Mestonin • Corticosteroids • Immunosuppressive medications: e.g. Imuran, Cytoxan • Plasmapheresis - Short term

49. Myasthenia Gravis • Medical Management • Thymectomy – indicated for almost all patients.  improvement in all patients • Administration of IV immune globulin to reduce the production of acetylcholine antibodies

50. Myasthenia Gravis • Nursing Interventions/Patient Teaching • Priority: Facilitate effective airway exchange. Respiratory problems frequently occur in MG patients • Teach patient airway protective techniques • Pace daily activities • ROM exercises • Medication management based on sx.