Nội dung text NCM 116 LEC SEMI FINALS
SEMI FINALS COVERAGE Multiple Sclerosis Myasthenia Gravis Guillain Barre Syndrome CN Disorders Trigeminal neuralgia Bell’s Palsy Peripheral Neuropathies Parkinson's, Huntington’s Alzheimer’s ALS Muscular Dystrophies Degenerative Disk Disease Herniation of cervical intervertebral Disk General Degenerative disorders GOOGLE DRIVE LINK (printable): SEMI FINALS COVERAGE 0 Multiple Sclerosis 1 Myasthenia Gravis 4 Guillain Barre Syndrome (Acute Inflammatory Demyelinating Polyradiculoneuropathy; AIDP) 5 CN Disorders 7 Trigeminal neuralgia 7 Bell’s Palsy (Facial Paralysis) 9 Peripheral Neuropathies 10 Parkinson's Disease 10 Huntington’s Disease (Huntington’s Chorea) 12 Alzheimer’s Disease 14 ALS (AMYOTROPHIC LATERAL SCLEROSIS; Lou Gehrig’s disease) 15 Muscular Dystrophies 16 Degenerative Disk Disease 17 Herniation of Cervical and Intervertebral Disk 17
NCM 116 LEC SEMI-FINALS by TONS and MADS and JANNY ➢ Cerebellar Sign: Nystagmus, Ataxia, Dysarthria, Dysphagia ➢ Motor: ■ Weakness or paralysis of limbs, trunk or head ■ Diplopia ■ Scanning speech ■ Spasticity of muscles that are chronically affected ➢ Sensory ■ Numbness, tingling and other paresthesias ■ Patchy blindness (scotomas) ■ Blurred vision ■ Vertigo, tinnitus, decreases ➢ The may assume many different patterns. In some, benign course and symptoms are so mild ➢ Between 80-85% have relapsing-remitting (RR) ■ With each relapse, recovery is usually complete; however, residual deficits may occur and accumulate over time, contributing to functional decline. ■ Fifty percent of those with RR course of MS progress to secondary progressive course, in which disease progression occurs with or without relapses. ➢ 10% of patients have a primary progressive course: disabling symptoms steadily increase, with rare plateaus and temporary improvement. ➢ Primary progressive MS may result in quadriparesis, cognitive dysfunction, visual loss, and brain stem syndromes. ➢ Least common presentation (about 5% of cases) is progressive relapsing course. It is characterized by relapses with continuous disabling progression between exacerbations ➢ The signs and symptoms of MS are varied and multiple, reflecting location of lesion (plaque) or combination of lesions. ➢ The primary symptoms most commonly reported are fatigue, depression, weakness, numbness, difficulty in coordination, loss of balance, pain ➢ Visual disturbances due to lesions in optic nerves ■ blurring of vision, diplopia (double vision), ■ patchy blindness (scotoma), ■ total blindness. ➢ Fatigue affects most people with MS and is often most disabling symptom. Heat, depression, anemia, deconditioning, and medication may contribute to fatigue. ➢ Avoiding hot temperatures, effective treatment of depression and anemia, and occupational and physical therapies may help control fatigue. ➢ Additional strategies include a balance of rest and activities, good nutrition, and healthy lifestyle including avoidance of alcohol and cigarette smoking ➢ Pain is another common symptom of MS that can contribute to social isolation. Lesions on sensory pathways cause pain. Additional sensory manifestations include paresthesias, dysesthesias, and proprioception loss ➢ Many people with MS need daily analgesic medications. In some cases, pain is managed with opioids, anti seizure medications, or antidepressants. Rarely, surgery may be needed to interrupt pain pathways. Among perimenopausal women, those with MS are more likely to have pain related to osteoporosis. In addition to estrogen loss, immobility and corticosteroid therapy play role in development of osteoporosis among women with MS. Bone mineral density testing is recommended for this high-risk group ➢ Spasticity (muscle hypertonicity) of the extremities and loss of the abdominal reflexes result from involvement of the main motor pathways (pyramidal tracts) of the spinal cord. ➢ Cognitive and psychosocial problems may reflect frontal or parietal lobe involvement. Some degree of cognitive change (eg, memory loss, decreased concentration) occurs in about half of patients, but severe cognitive changes with dementia (progressive organic mental disorder) are rare. ➢ Involvement of the cerebellum or basal ganglia can produce ataxia (impaired coordination of movements) and tremor. ➢ Loss of the control connections between the cortex and basal ganglia may occur and cause emotional lability and euphoria. Bladder, bowel, and sexual dysfunctions are common. ➢ Secondary complications of MS include urinary tract infections, constipation, pressure ulcers, contracture deformities, dependent pedal edema, pneumonia, reactive depression, and osteoporosis. Emotional, social, marital, economic, and vocational problems ➢ During exacerbations, new symptoms appear and existing ones worsen; during remissions, symptoms decrease or disappear. Relapses may be associated with emotional and physical stress. ❖ Types of MS ➢ Relapsing Remitting (RR) - characterized by clearly acute attacks with full recovery or with sequelae & residual deficit upon recovery. Periods between disease relapses are characterized by lack of disease progression (recovery is usually complete); occurs 80-85% in MS cases ➢ Primary Progressive (PP) - neurologic function worsens or disability accumulates as soon as symptoms appear, w/o early relapses or remissions. ➢ Secondary Progressive (SP) - follows the initial relapsing-remitting course. Some people eventually go on to have a secondary progressive course, in which neurologic function worsens progressively or disability accumulates over time. ➢ Progressive Relapsing (PR) - rare form that causes steady damage to nerves when symptoms first appear and continues to cause progressive worsening. Relapses (disease flare-ups) occur, followed by full or partial recovery, but nerve damage continues and symptoms become increasingly disabling ❖ Gerontologic Considerations Page 1959 ❖ Diagnostics ➢ CT & MRI - patches of destroyed myelin & multiple plaques in CNS confirms; diagnosis based on the 2
NCM 116 LEC SEMI-FINALS by TONS and MADS and JANNY presence of multiple plaques in CNS observed with MRI ➢ Electrophoresis of CSF identifies presence of oligoclonal banding (several bands of IgG bonded together, indicating an immune system abnormality). ➢ Underlying bladder dysfunction is diagnosed by urodynamic studies. Neuropsychological testing may be indicated to assess cognitive impairment. A sexual history helps identify changes in sexual function. ➢ Brain Stem Evoked Potential - Defines extent of disease, monitor changes & how long it takes for a nerve impulse. ❖ Medical Management (Treatment): ➢ Interferon B-1a & 1b, glatiramer acetate, IV methylprednisolone ➢ No Cure ➢ Retrieve and harvest stem cells ➢ Immunosuppression (chemotherapy) ➢ Stem cell transplantation ➢ Pharmacology: Medications prescribed for MS include those for disease modification and those for symptom management. The disease-modifying therapies: immunomodulating therapies and immunosuppressive agents ■ Disease modifying therapies: ● Interferon beta-1a (Rebif), Interferon beta-1b (Betaseron), Interferon beta-1a (Avonex): ◆ These medications are administered via injection and help reduce the frequency, duration, and size of relapses in multiple sclerosis (MS). ◆ Side effects include flu-like symptoms, liver damage, fetal abnormalities, and depression. ◆ Optimal disability control requires early initiation of these medications. ● Immunomodulators - helps regulate immune system: ◆ Avonex - Interferon beta 1a IM 1x/week ◆ Betaseron - Interferon beta-2a SQ q other day ◆ Glatiramer acetate (Copaxone): ➢ Administered SQ daily, Copaxone reduces relapse rates and increases time between relapses in relapsing-remitting MS. ➢ Side effects are minimal and manageable, with increased antigen-specific suppressor T cell activity. ◆ Mitoxantrone (Novantrone): ➢ Administered via IV infusion every 3 months, Novantrone reduces relapse frequency in secondary-progressive or worsening relapsing-remitting MS. ➢ Patients require close monitoring for cardiac toxicity due to potential side effects. ■ Corticosteroids: Methylprednisolone IV ● Used in treating acute relapses in relapsing-remitting MS, shortens duration of relapses by exerting anti-inflammatory effects. ● Side effects include mood swings, weight gain, and electrolyte imbalances. ➢ Symptom Management: ■ Muscle Relaxants: Baclofen (Lioresal), Dantrolene (Dantrium), Diazepam (Valium) ■ Immunosuppressants ● Azathioprine (Imuran) ● Cyclophosphamide (Cytoxan) ■ Spasticity: Baclofen (Lioresal), benzodiazepines (Valium), tizanidine (Zanaflex), and dantrolene (Dantrium) are used to treat spasticity. Severe cases may require intrathecal baclofen injection or surgical intervention. ■ Fatigue: Medications such as amantadine (Symmetrel), pemoline (Cylert), or fluoxetine (Prozac) may be prescribed to manage fatigue interfering with activities of daily living. ■ Ataxia: Ataxia is challenging to treat, and medications like beta-adrenergic blockers (Inderal), antiseizure agents (Neurontin), and benzodiazepines (Klonopin) may be used. ■ Bladder and Bowel Problems: Medications such as anticholinergic agents, alpha-adrenergic blockers, and antispasmodic agents help manage bladder and bowel problems. Nonpharmacologic strategies are also employed for effective bowel and bladder elimination. ■ Urinary Tract Infection (UTI): Ascorbic acid (vitamin C) may be prescribed to acidify urine and reduce bacterial growth. Antibiotics are used when necessary to treat UTIs. ❖ An individual treatment program is indicated to relieve patient’s symptoms and provide continuing support, particularly for patients with cognitive changes, who may need more structure and support. ❖ The goals of treatment are to delay the progression of disease, manage chronic symptoms, and treat acute exacerbations. ❖ Many patients with MS have stable disease course and require only intermittent treatment, whereas others experience steady progression of their disease. ❖ Symptoms requiring intervention include spasticity, fatigue, bladder dysfunction, and ataxia. ❖ Management strategies target the various motor and sensory symptoms and effects of immobility that can occur. ❖ NURSING MANAGEMENT ➢ Fatigue ■ Limit activities & stop when fatigue occurs ■ Take rest periods several times a day & 8-9 hours uninterrupted sleep per night ■ Meds that reduce the relapse may control fatigue; ■ Avoiding hot temperatures, ■ Treatment of depression & anemia & pt may control fatigue ■ Arrange daily activities to include rest periods ➢ Impaired Bed & physical Mobility related to weakness, muscle paresis & spasticity ➢ Risk for Injury related to sensory & visual impairment ➢ Impaired Urinary/Bowel Elimination (urgency, frequence, incontinence & constipation) ➢ Support Group: National Multiple Sclerosis Societynn ❖ UPDATES: Women who have multiple sclerosis may reduce their risk of relapses after pregnancy if they breastfeed their babies, as per a research study 3