dd-2015 - Degenerative Diseases Movement Disorders Parkinsons Disease Multiple Sclerosis Myasthenia Gravis Guillain-Barre Syndrome ALS Huntington

dd-2015 - Degenerative Diseases Movement Disorders...

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Unformatted text preview: Degenerative Diseases/ Movement Disorders Parkinson’s Disease Multiple Sclerosis Myasthenia Gravis Guillain-Barre Syndrome ALS Huntington Disease Parkinson Patients Parkinson’s Disease Definition: a disorder of the central nervous system, involving primarily a degeneration of certain nerve cells in deep parts of the brain called the basal ganglia, and in particular a loss of nerve cells (or neurons) in a part of the brainstem called the substantia nigra. These cells make the neurochemical messenger dopamine, which is partly responsible for starting a circuit of messages that coordinate normal movement Parkinson’s Disease Occurrence: Worldwide estimated to be 4 million In U.S. 60,000 new cases diagnosed yearly Prevalence is slightly higher in men Average age of onset is @ 60 Prevalence is slightly higher in Caucasian people Dopamine and Parkinson’s Disease Symptoms of Parkinson’s Tremors at rest Increase rigidity Shuffling gait No arm/hand swing Mask like face Drooling Weight loss Constipation Emotional instabilitydepression Parkinson’s Disease Primary Symptoms: Tremor Tremor at rest is the characteristic feature of PD Rest tremor occurs rarely in any other condition Slow and rhythmic Usually begins in one hand and spreads to involve the other side Feet or legs may occasionally exhibit tremor Lips and jaw may also shake Parkinson’s Disease Signs and symptoms: Initial symptoms Weakness Difficulty getting started initiating movement Symptoms begin on one side and remain unilateral for some time. Shaking or trembling, usually of the hands, may occur very early in the condition. The shaking is generally at rest Dragging of one leg is also a common complaint early on Shuffling Gait Classic Triad of PD Resting tremors- “pill-rolling” Rigidity- “cog wheeling” Bradykinesia- slow and deliberate ? v=ECkPVTZlfP8&feature=relat ed Parkinson’s Disease Speech: Voice may become softer May be a loss of the normal variation in volume and emotion Speaking rapidly with the words crowded together Hoarseness In more advanced stages, a type of stuttering, which makes the patient much more difficult to be understood Parkinson’s Disease Swallowing: problems with swallowing (dysphagia), when they occur in PD, happen late in the course of the disease Drooling: experienced with swallowing, in that saliva pools in the back of the throat Seborrheic dermatitis: a common skin disorder in many people, excessive oily secretions, particularly on the forehead and scalp, may be a problem Ankle swelling: (edema) of the feet and ankles occur frequently Parkinson’s Disease Visual problems: may complain of some mild double vision or problems with the eyes ‘bouncing’ around. Weight loss: loss of weight, sometimes considerable, is a common occurrence in PD. It may result from a decreased appetite in PD, swallowing difficulties, or other gastrointestinal disturbances. Constipation and other GI problems: PD may slow the bowels down. Abdominal distention or bloating may occur in PD. Nausea and vomiting may occur in untreated PD. Parkinson’s Disease Depression, anxiety, and panic attacks: occurs in as many as 50% of PD patients. They may be among the first symptoms of PD, and in a small number of patients, the initial symptom may be frank panic attacks, with full-blown palpitations, hyperventilation, sweating, pallor, and a feeling of impending doom. Parkinson’s Disease Disturbances of sleep: patients fall asleep with no problem, but wake up frequently during the night. Dementia, memory loss, and confusion: problems with memory, thinking, wordfinding, and other features of cognitive function (dementia) may occur in up to 4050% of PD patients, especially late in the disease Treatment Medications: levodopa Sinemet (L-Dopa and Carbidopa) Cogentin Surgery: Sterotactic DBS Rehab Gait training ROM Medications for Parkinson’s Disease: Levodopa: the most important and most effective drug to treat the symptoms of PD. Levodopa restores the amount of dopamine in the substantia nigra and striatum to near-normal levels and thereby reduces the symptoms and signs of the disease. Levodopa/carbidopa: it is usually taken in combination with carbidopa (trade name: Sinemet) Sterotactic Surgery Surgery Deep Brain Stimulation Surgery ? q=deep+brain+stimulation+parkinson %27s&FORM=HDRSC3#view=detail&mid= A568C44C1189FF004703A568C44C1189 FF004703 Multiple Sclerosis Multiple Sclerosis Pathophysiology Abnormal immune system response causes myelin sheath surrounding axons to become damaged Plaques form along nerve fibers in the brain and spinal cord Electrical conduction interrupted Trigger may be viral or environmental Multiple Sclerosis Epidemiology 250-350 Americans affected Prevalence higher among Caucasians Affects prime of life adults, women 3 times as often as men Jack Osbourne Signs and Symptoms of MS patient Primary symptoms vary with lesion location: Fatigue (also called MS lassitude to differentiate it from tiredness resulting from other causes) Problems with walking Bowel and or bladder disturbances Visual problems Signs and Symptoms of MS patient Changes in cognitive function, including problems with memory, attention, and problem-solving Abnormal sensations such as numbness or "pins and needles" Changes in sexual function Pain Depression and/or mood swings Clinical Patterns of MS Multiple Sclerosis Diagnostic findings: MRI: Detects abnormalities, but non-specific for MS Evoked potentials: Identify delays in nerve conduction Visual, Brainstem Auditory, or somatosensory CSF: Antibodies (Oligoclonal bands) “Kappa light chains” MRI of MS Multiple Sclerosis Treatment goals: Prevent secondary symptoms Decrease and control specific symptoms Alter long-term disease activity Support psych-social concerns Multiple Sclerosis Advances in Treatment: High-dose IV corticosteroid (methylrednisolone) Low dose methotrexate (immunosuppressant) Monoclonal antibodies Immunomodulators Interferon beta-1b (Betaseron) Interferon beta-1a (Avonex) Glatiramer acetate (Copaxone) Myasthenia Gravis Normal ACh receptor site ACh receptor site in myasthenia gravis Myasthenia Gravis Ptosis. Ophthalmoplegia. May present as only ocular myasthenia gravis ~50% of the time. Systemic manifestations include: shortness of breath, dysphagia, hoarseness, and fatigue. May be associated with Grave's in ~5% of cases. May mimic other cranial nerve palsies, e.g., 6th nerve palsy, 4th nerve palsy, internuclear ophthalmoplegia, etc... May be associated with thymoma. The Thymus and MG Diagnosis Before and after Tensilon Medical Treatment of MG May follow patient closely if patient has mild symptoms. For symptomatic patients, (Mestinon) pyridostigmine bromide 60 mg PO 4X/day with dosage adjustment according to response. Dosing of over 120 mg PO Q3H is rarely helpful Systemic steroids may be needed for severe cases. Other immunosuppressive therapy may be indicated. Removal of thymoma. Plasmapheresis Plasmapheresis Plasmapheresis is a procedure in which blood is separated into cells and plasma (liquid). The plasma is removed and replaced with fresh frozen plasma, a blood product called albumin and/or a plasma substitute. The procedure is often referred to as plasma exchange. Myasthenia Crisis Occurs when weakness affects the muscles that control breathing Respiratory assist may be necessary Plasma exchange may be needed Myasthenic /Cholinergic Crisis Result of disease exacerbation or precipitating event, most commonly a respiratory infection. Severe generalized muscle weakness with respiratory and bulbar weakness. Patient may develop respiratory compromise failure. Caused by overmedication with cholinesterase inhibitors Severe muscle weakness with respiratory and bulbar weakness Patent may develop respiratory compromise and failure Management of Myasthenic Crisis Patient instruction in signs and symptoms of myasthenic crisis and cholinergic crisis. Assuring adequate ventilation; intubation and mechanical ventilation may be needed. Assessment and supportive measures include: Measures to ensure airway and respiratory support ABGS, serum electrolytes, I&O, and daily weight If patient cannot swallow, nasogastric feeding may be required Avoid sedatives and tranquilizers Guillain-Barré Syndrome: A diffuse Inflammatory Response Occurring in the Peripheral Nervous System Theories of Etiology Preceded by febrile illness (infective or viral) Autoimmune response Tissue hypersensitivity reaction (altered cell-medicated immunity) Associated with immunosupression Guillain-Barré Syndrome: Pathophysiology Demyelination of peripheral nerve with minimal axonomal damage Perivascular inflammation results in compression of nerve roots Serum proteins enter SAS and CSF Demyelination alters nerve conduction Guillain-Barré Syndrome: Motor losses are symmetrical and may ascend to any level of the PNS or CNS Restoration of function follows regeneration of myelin sheath Motor function returns in descending order Recovery may take 636 months Signs and Symptoms of GB Difficulty with eye movement, facial movement, speaking, chewing or swallowing Severe pain in the lower back Difficulty with bladder control or intestinal functions Very slow heart rate or low blood pressure Difficulty breathing Signs and Symptoms History of recent illnesses, immunizations Minimal sensory disturbances occur first Motor disturbances Rapid onset of symmetrical weakness Flaccid paralysis may follow Involves lower limbs, ascends to trunk, upper extremities, cranial nerves Incontinence Muscle twitching Paralysis of diaphragm and intercostal muscles Treatment for Guillain-Barre Nursing Process: The Care of the Patient with Guillain–Barré Syndrome— Assessment Ongoing assessment for with emphasis on early detection of life-threatening complications of respiratory failure, cardiac dysrhythmias, and deep vein thrombosis Monitor for changes in vital capacity and negative inspiratory force Assess VS frequently/continuously including continuous monitoring of ECG Patient and family coping Characteristics of the Syndrome Latent period from infection to symptom Abrupt onset Cardinal features Paralysis/paresthesia Albuminocytologic dissociation in CSF Progression: 1-48 days, average 10 days Duration of maximum symptoms: 10 days Characteristics of the Syndrome Recovery period averages 2 weeks to 24 months Relapse: infrequent Prognosis Mortality: 4-5% Complete recovery: 75% Children fare better than adults Outcome dependent on nursing care Nerve Degeneration in Guillain-Barré Syndrome In Guillain-Barré syndrome, the myelin sheath of peripheral nerves and of anterior and posterior roots at spinal segmental levels demyelinates and degenerates. Early in the disease, inflammation and edema occur. Signs of both sensory and motor impairment appear. These include paresthesias, such as numbness, tingling, and burning, as well as motor neuron paralysis Diagnostic Findings Lumbar puncture for cell count Elevation in CSF protein level Minimal sensory findings Nerve conduction Slowing occurs Initial conduction Reported conduction Interventions Symptomatic Aggressive Steroids Plasmaphoresis Removal of auto antibodies Complications Clotting factors Fluid and electrolyte imbalance Hemolysis ALSAmyotrophic lateral sclerosis ? q=ALS+Disease+Stories&Form=VQFRVP# view=detail&mid=089A899DC758C86BA2 8D089A899DC758C86BA28D Researchers are studying several possible causes of ALS, including: 1.Gene mutation. Various genetic mutations can lead to inherited ALS, which appears nearly identical to the noninherited form. 2.Chemical imbalance. People with ALS generally have higher than normal levels of glutamate, a chemical messenger in the brain, around the nerve cells in their spinal fluid. Too much glutamate is known to be toxic to some nerve cells. 3.Disorganized immune response. Sometimes a person's immune system begins attacking some of his or her body's own normal cells, which may lead to the death of nerve cells Established risk factors for ALS include: 1.Heredity. Five to 10 percent of the people with ALS inherited it (familial ALS). In most people with familial ALS, their children have a 50-50 chance of developing the disease. 2.Age. ALS most commonly occurs in people between the ages of 40 and 60. 3.Sex. Before the age of 65, slightly more men than women develop ALS. This sex difference disappears after age 70. Early signs and symptoms of ALS include: 1.Difficulty walking, tripping or difficulty doing your normal daily activities 2.Weakness in your leg, feet or ankles 3.Hand weakness or clumsiness 4.Slurring of speech or trouble swallowing 5.Muscle cramps and twitching in your arms, shoulders and tongue 6.Difficulty holding your head up or keeping a good posture Medications The drug riluzole (Rilutek) is the only medication approved by the Food and Drug Administration for ALS. The drug appears to slow the disease's progression in some people, perhaps by reducing levels of a chemical messenger in the brain (glutamate) that's often present in higher levels in people with ALS. Riluzole may cause side effects such as dizziness, gastrointestinal conditions and liver function changes. Your doctor may also prescribe medications to provide relief from other symptoms, including: Muscle cramps and spasms Spasticity Constipation Fatigue Excessive salivation Excessive phlegm Pain Depression Sleep problems Uncontrolled outbursts of laughing or crying Woody Guthrie Huntington’s Disease A chronic progressive hereditary disease that results in choreoform movement and dementia Transmitted as an autosomal dominant trait Pathology involves the loss of cells in the striatum of the basal ganglia and the cortex Huntington’s Disease Nursing Intervention for Degenerative Diseases Nursing assessment Nursing diagnoses Planning Nursing implementation Evaluation Treatment for Degenerative Diseases Supportive care Rehabilitation Research ...
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  • Summer '15
  • CatherineBoccanfuso
  • Myasthenia gravis

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