Neurological Stressors III Chronic Neurological Disorders

1. Neurological Stressors IIIChronic Neurological Disorders Joy Borrero, RN, MSN

2. Topics • Multiple Sclerosis • Amyotropic Lateral Sclerosis (ALS) • Parkinson’s Disease • Myasthenia Gravis • Guillain-Barre Syndrome • Huntington’s Disease

3. Chronic Neuromuscular Dysfunction • Some commonalities in adaptations are: • Altered motor activity • Altered coordination • Altered sensory function • Altered urinary/bowel patterns • Altered role/self perception • Altered speech/swallowing • Impaired physical mobility • Risk for injury • Nutrition less than body requirements • Risk for aspiration

4. Multiple Sclerosis • Chronic autoimmune disease affecting the myelin sheath and conduction pathway of the CNS • Characterized by periods of remission and exacerbation • Inflammatory response resulting in random or patchy areas of plaque in the white matter of the CNS • Demyelination of brain and spinal cord, affects conduction pathway of CNS • Two major courses: relapsing and remitting, and chronic and progressive

5. Multiple Sclerosis (MS) • Exact cause unknown, may be defective immune response, early viral infection. • More common in people living in northern temperate climate. • Affects twice as many women than men. • Typically occurs between ages 20-40 • Family hx of MS in 15% of cases • No cure for the disease • Dx- McDonald criteria • http://www.nmss.org/

6. Virus,infectious agent Living in cold climate Physical injury Emotional stress Pregnancy Fatigue Overexertion Temperature extremes Hot shower/bath Trigger Factors

7. MS Primary Symptoms: • Fatigue, weakness, numbness • Difficulty in coordination, loss of balance • Visual disturbances: blurring, diplopia, patchy or total blindness, change in peripheral vision, nystagmus • Speech defects • Dysarthria, dysphagia • Spastic weakness, ataxia, tremors, dysmetria • Emotional lability, depression/euphoria. • Bladder/bowel dysfunction • Tinnitus, vertigo, decreasing hearing acuity • Cognitive changes-memory loss, impaired judgement

8. MS Secondary symptoms- Problems/complications that occur a/r/o primary symptoms • Repeated UTIs • Loss of muscle tone and disuse weakness • Poor posture and control of torso • Decreased bone density • Shallow inefficient breathing • Pressure ulcers from immobility

9. MS • Impaired physical mobility r/t weakness, muscle paresis and spasticity. • Risk for injury r/t sensory, visual and motor impairment. • Altered urinary and bowel elimination r/t SC dysfunction. • Altered thought processes r/t cerebral dysfunction.

10. Pharmacology Biological response modifiers- Inteferon Monoclonal antibody- Natalizumab Synthetic protein-Glatiramer acetate Immunosuppressives-Cyclosporine Corticosteroids-Prednisone Antispasmodic drugs-Dantrolene, Baclofen Anticonvulsants-Dilantin Stool softeners-Colace Anticholinergics-Probanthine Beta-blockers- Inderal (tremors) CAM Therapy

11. Biological Response Modifiers • Interferon –Betaseron- blocks replication of viruses and stimulates host immunoregulatory activities. • Reduce frequency of exacerbations • Dose 0.25 mg sc every other day. • Adverse effects: Dizziness, confusion, anorexia, hypotension, leukopenia, flu like symptoms, depression,suicidal ideation • Thrombocytopenia

12. Amyotrophic Lateral Sclerosis (ALS) • Deteriorating disease of motor neurons in anterior horn of spinal cord and motor nuclei of lower brain stem • AKA Lou Gehrig’s disease • Etiology unknown. • More common men 50-60. • Manifestations: Depends on location of affected motor neurons, atrophy and twitching. • Death occurs within 2-5 years after onset of symptoms.

13. Diagnostic Procedures • Creatine kinase (CK)- increased • Electromyography (EMG)- Muscle fasciculations • http://www.youtube.com/watch?v=k0uSpYd_Ics • Muscle biopsy- Muscle atrophy • Serial muscle testing- Loss of muscle strength • Pulmonary function tests

14. Assessments & Interventions • Assess airway, resp status • Assess speech patterns, skin integrity • No known cure, no treatment, no preventive measures • Riluzole, only drug approved by FDA to extend survival time • Exercise and mobility program • Management of swallowing difficulties

15. Interventions • Teach swallowing technique • Maintain resp. function- assess lung sounds,suction prn, C&DB • Administer Quinine for muscle cramps • Antispasmotics: baclofen, dantrolene, diazepam • www.als.org for pt/family support and education

16. Parkinson’s disease Cause unknown-progressively debilitating disease that affects gross motor function. Degeneration of the nerves that control voluntary movement. Occurs in the part of the brain called the substantia nigra Neurons here communicate with other neurons in the brain using a neurotransmitter called dopamine. When these neurons that make dopamine die, signaling between neurons is severely hampered, causing a loss of controlled movement • Risk factors • Dx Procedures

17. Manifestations • Manifestations: • Postural instability- • Resting tremors • Akinesia, Bradykinesia • Muscle Rigidity • ANS changes • Dementia

18. Parkinson’s disease • Impaired physical mobility r/t muscle rigidity and weakness. • Self care deficit r/t to tremor and motor disturbance. • Risk for falls • Risk for imbalanced nutrition: Less than body requirements, r/t tremor, slowness in eating, difficulty in chewing and swallowing.

19. Parkinson’s DiseaseNursing Interventions • Assess/monitor the patient • Mobility- progressive, daily exercise. • Nutrition/Hydration – wt. loss is common, teach swallowing sequence. • Speech – encourage to speak slowly • Promote patient communication. • Provide for a safe environment • Administer prescribed meds

20. Drug Therapy for Parkinson’sGoal: Increase patient’s functional abilities

21. Parkinson’s disease levodopa (Dopar, Laradopa)- antiparkinson agent, precursor of dopamine that can cross the blood brain barrier, then is transformed to dopamine. levodopa and carbidopa (Sinemet)- GOLD STANDARD,antiparkinsonian combo med-dosage is adjusted to patient’s symptoms • Adverse effects – involuntary movements, ataxia, increased tremor, anorexia

22. Parkinson’s disease • amantadine hydrochloride (Symmetrel) • Antiviral and Antiparkinsonian • Therapeutic actions – thought to increase dopamine release, helpful in treating bradykinesia, rigidity and tremor. • Dose – 100 mg po bid (up to 400 mg) • Adverse- dizziness, insomnia, dry mouth, confusion, constipation, urinary retention, orthostatic hypotension.

23. Parkinson’s Disease Complications: • Aspiration pneumonia • Altered cognition, dementia Additional therapeutic options: Thalamotomy Stereotactic Pallidotomy Deep Brain Stimulation http://www.parkinsons.org

24. Myasthenia Gravis Progressive autoimmune disease resulting in severe muscle weakness. Women 15-35 or men over 40. Exacerbations and remissions Manifestations: Extreme muscle weakness and fatiguability. Diplopia and ptosis are early signs Sleepy, mask like expression, dysphonia. Problems chewing and swallowing – aspiration Progressive weakness of diaphragm- resp. distress.

25. Myasthenia Gravis • Diagnostic tests – pt. history and exam, Tensilon testing • IV tensilon – relieves symptoms within 30 seconds. Positive result. • AChR-Acetylcholine receptor antibodies found serum of 90%. • EMG- shows neuromuscular transmission characteristics.

26. Myasthenia Gravis Nursing diagnoses: • Ineffective breathing pattern r/t respiratory muscle weakness. • Impaired physical mobility due to muscle weakness. • Risk for aspiration • Self care deficit

27. Therapeutic Procedures • Plasmapheresis • Thymectomy • http://www.myasthenia.org

28. Myasthenia Gravis Nursing Interventions • Teach patient about exacerbation triggers • Promote respiratory function • Increase physical mobility • Avoid exposure to heat and cold. • Provide eye care • Prevent aspiration, assess swallowing • Assess for complications • I&O, small frequent hi caloric meals • Consults: OT, PT, Speech/Language therapy • Medi alert bracelet

29. Medications Anticholinesterase meds- pyridostigmine (Mestinon) and ambenonium (Mytelase) • Administer with small amount of food to avoid GI upset and eat within 45 minutes. • Must be administerd on time and same time each day. • Potential SE is cholinergic crisis Cotricosteroids Immunosuppressants

30. Complications

31. Guillian Barre Syndrome • Acute inflammatory disease of unknown origin • Usually preceeded by a viral infection (URI or GI) 1-4 weeks • Degeneration of the myelin sheath of peripheral nerves • Manifestations: paresthesias, ascending weakness in legs progresses to paralysis,autonomic dysfunction • Diagnosis by H&P, CSF exam.

32. Facts • Most people reach the stage of greatest weakness within 2 weeks after symptoms appear, and by the 3rd week 90% of all patients are at their weakest. • Recovery may be from a few weeks to a few years • 30% will have residual weakness after 3yrs • 3% may suffer a relapse

33. Guillian Barre - Interventions • Monitor CV status • Respiratory- Chest PT, elevate HOB, mechanical Ventilator and/or trach. • Nutrition – NG or oral feedings, assess bowel sounds, TPN. • Communication: lip reading, picture cards, eye blinks. • Physiologic support – pt. and family teaching, relaxation exercises, involve pt. and family in planning and diversional activities. • Prevent deformities, ROM exervises • Skin care • Plasmaphoresis • Consider steroids

34. Huntington’s Disease • Formerly called Huntington’s Chorea • Hereditary disease of the basal ganglia and cerebral cortex • Appears in adult life • Almost 50% of offspring inherit disorder • Usual onset 4th or 5th decade of life • Slightly higher incidence in males • Abnormal gene found on chromosome 4 • Usually die within 15 years

35. Huntington’s Disease • Unexplained atrophy of basal ganglia and cerebral cortex • Chronic, progressive chorea and mental deterioration that results in dementia • Emotional disturbances, mental deterioration • Choreiform movement- jerky movement affecting the entire body • Speech slurred, often explosive and eventually unintelligible • Chewing and swallowing difficulty and danger of choking.

36. Symptoms • In early stages emotional changes fits of anger, profound depression, apathy or euphoria • Judgement and memory impaired • Eventually pt becomes totally demented, incontinent and completely helpless • Succumb to Pn, heart failure or infection • Usually die as a result of choking, infection or suicide

37. MANAGEMENT Dx: made on basis of symptoms and family hx • Genetic counseling and testing Tx: • Haldol and Navane • Riluzole • Antidepressants

38. NCLEX TIME • Mr. Cohen is being evaluated for signs associated with either a myasthenia crisis or a cholinergic crisis. Which symptoms would confirm that this client is experiencing a cholinergic crisis? • A.Increased pulse, anoxia, and decreased urine output • B.Abdominal cramps, blurred vision, and facial muscle twitching • C.Pupillary miosis, cyanosis, and anoxia • D.Bowel and bladder incontinence, pallor, and cyanosis

39. NCLEX TIME Once Mr. Cohen's crisis has resolved, his nurse teaches him about proper medication administration. Which statement, if made by the client, demonstrates the need for further teaching? A.“I can still take over-the-counter medications without a problem.” B.“I should keep an extra supply of medication in my car.” C.“I should wear a watch with an alarm to remind me to take my medications.” D.“I can post my medicine schedule at home, so my family knows my schedule.”

40. NCLEX TIME Mr. Montgomery is a 64-year-old man admitted with an exacerbation of Guillain-Barré syndrome. He presents with facial weakness, dysphagia, and difficulty speaking. Which nursing diagnosis would be most applicable to this client? A.Acute Pain related to paresthesia B.Imbalanced Nutrition: Less than Body Requirements C.Disturbed Body Image related to loss of body function D.Risk for Impaired Skin Integrity

41. NCLEX TIME Mr.Montgomery is having trouble breathing. Which nursing intervention should his nurse perform first? • A.Auscultate breath sounds. • B.Instruct the client on how to cough effectively. • C.Perform chest physiotherapy as appropriate. • D.Remove secretions by suctioning the client.

42. NCLEX TIME Sarah is a senior nursing student doing a clinical rotation on a neuro unit. She is studying the differences between Guillain-Barré syndrome and myasthenia gravis. Which statement, if made by Sarah, demonstrates a good understanding of the two? A.“Myasthenia gravis in an acute autoimmune disorder characterized by varying degrees of motor weakness and paralysis.” B.“Guillain-Barré syndrome is a chronic disease characterized by fatigue and weakness primarily in muscles innervated by the cranial nerves.” C.“Key features of Guillain-Barré syndrome include labile blood pressure, tachycardia, and cramping pain.” D.“Key features of myasthenia gravis include decreased or absent deep tendon reflexes, cardiac dysrhythmias, and diplopia.”

43. NCLEX TIME • Charles' wife decides to attend a local teaching session on Parkinson's disease. Which intervention would be appropriate in caring for a client with Parkinson's disease? • A.Complete activities of daily living for the client. • B.Schedule appointments early in the morning to avoid the client being tired for them. • C.Provide high-calorie, high-carbohydrate foods to maintain the client’s weight. • D.Administer medications promptly on schedule to maintain therapeutic drug levels.

44. Think like a Nurse A patient is undergoing test to confirm a dx of Myesthenia Gravis. The nurse explains that MG is established if muscle function improves after the patient receives an IV injection of a medication. What is the brand name of this medication?

45. NCLEX Time Which of the following is not a typical clinical manifestation of multiple sclerosis? • Double vision • Sudden bursts of energy • Weakness in the extremities • Muscle tremors

46. NCLEX Time During the neuro assessment of a patient with a tentative Guillain-Barre syndrome the nurse would expect that the patient will manifest: • Hallucinations • Increased muscular weakness • Pronounced muscular atrophy • Impairment in cognitive reasoning