MedSurge LEC 2 TERM 3 NEURO MOON OUTLINE I. II. III. IV. MULTIPLE SCLEROSIS MYASTHENIA GRAVIS GUILLAIN-BARRE SYNDROME PARKINSON’S DISEASE MULTIPLE SCLEROSIS Multiple areas of scar and plaque formation in the CNS (brain and spinal cord) SCLEROSIS or hardening RARE: 30 out of 100,000 common among WOMEN 20-40 years old; common in CAUCASIAN First described in 1868 by Jean-Martin Charcot An immune-mediated, chronic, progressive, degenerative disease with periods of remission and exacerbation characterized by randomly scattered patches of demyelination in the brainstem, cerebrum, cerebellum and spinal cord resulting to impaired transmission of nerve impulses Twice as many women are diagnosed with MS as men Cause: remains unknown INFECTIONS (viral) + autoimmunity EBV, Hepatitis and Herpes Zoster Theories: (Predisposing Factors) • Genetics: indicates the presence of a specific cluster (haplotype- DNA variation) • Infections • Environmental factors - geographic • Severe stress • Smoking • Intake of aspartame TYPES & COURSES - RPSP RELAPSING REMITTING MS is characterized by clearly acute attacks with full recovery or with sequelae & residual deficit upon recovery. PRIMARY PROGRESSIVE MS is characterized by disease showing progression of disability from onset, without plateaus & temporary minor improvements SECONDARY PROGRESSIVE MS begins with an initial RR course, followed by progression of variable rate, which may also include occasional relapses & minor remissions PROGRESSIVE RELAPSING MS shows progression from onset but with clear acute relapses with or without recovery. PATHOPHYSIOLOGY Sensitized T&B lymphocytes cross the BBB ↓ In MS, sensitized T cells remain in the CNS ↓ T cells recognize myelin as foreign ↓ Inflammatory processes is triggered Attacks the myelin as if it were an invading virus ↓ Plaques & demyelinated axons occur ↓ Axons begin to degenerate ↓ Permanent & irreversible damage CLINICAL MANIFESTATION During EXACERBATIONS, new symptoms appear & existing ones worsen During REMISSIONS, symptoms decrease or disappear Signs & symptoms are varied & multiple, reflecting the location of lesion or combination of lesions Primary symptoms ▪ Fatigue, weakness ▪ Depression ▪ Numbness ▪ Difficulty in coordination, loss of balance & Pain Visual disturbances (demyelination of CN 2) ▪ Blurring of vision ▪ Diplopia ▪ Patchy blindness- scotoma ▪ Total blindness Heat, Depression Anemia, Deconditioning (weakness) Sensory manifestations ▪ Pain ▪ Paresthesias ▪ Dysesthesias Spasticity of the extremities Behavioral- emotional lability, euphoria, depression 1 MedSurge LEC 2 TERM 3 NEURO Uhthoff – often the first sign of M.S. - Worsening of vision caused by hot temperature (increased temp: slowed/blocked nerve TRANSMISSION LHERMITTE’S SIGN – electric shock like sensation radiating down the spine to the legs and arms when neck is moved Cognitive change ▪ Memory loss ▪ Decreased concentration Impaired cerebellar function (Charcot’s Triad) ▪ Scanning speech ▪ Intention tremors ▪ Nystagmus ▪ Ataxia ▪ Dysarthria (poor speech articulation) Bladder, bowel & sexual dysfunctions "Walking is good exercise", for example, might be pronounced as "Walk (pause) ing is good ex (pause) er (pause) cise" DIAGNOSTIC FINDINGS • MRI- scattered patches of scar/plaque (>5mm) in the CNS • CSF Studies- protein electropheresis., Igs are separated from csf, results (+) oligoclonal bonds • CT Scan • EEG MOON PHARMACOLOGIC THERAPY Disease-Modifying therapies Immunosuppressants • Corticosteroids Prednisone (Deltasone, Liquid Pred, Deltasone, Orasone, Prednicen-M); methylprednisolone (Medrol, Depo-Medrol) Interferons: have the ability to regulate the immune system & play an important role in protecting against intruders including viruses Beta interferons- found to be useful in managing MS Beta 1a: rebig; beta 1b: betasteron (SQ) Glatiramer acetate (Copaxone) – increase suppresor T cells, Admin sq daily (P3000/shot) Symptom Management Baclofen (Lioresal); (GABA agonist)- for spasticity Benzodiazepines (Valium), Tizanidine (Zanaflex) & dantrolene (Dantrium) Fatigue: amantadine (symmetrel), pemoline (Cylert), fluoxetine (prozac) Ataxia: beta adrenergic blockers (Inderal); antiseizure agents (Neurontin) & benzodiazepines (Klonopin) BOWEL AND BLADDER PROB: Anticholinergic (incontinence/frequency) – probanthine; oxybutynin Cholinergic (retention)– Bethanecol; Neostigmine PAIN – gabapentin; carbamazepine; amitriptyline NURSING DIAGNOSES • Impaired physical mobility related to weakness, muscle paresis, spasticity • Risk for injury related to sensory & visual impairment • Impaired urinary & bowel elimination • Disturbed thought process related to cerebral dysfunction • Potential for sexual dysfunction related to lesions or psychological reactions NURSING INTERVENTIONS ✔ Promoting physical mobility MEDICAL MANAGEMENT • No known cure for MS • Goal of the treatments • Attempt to return function after an attack • Prevent new attacks • Prevent disability • Delay the progression of the disease Exercises: Walking Use of assistive device- cane. crutches, walker ✔ Minimizing Spasticity and Contractures 2 MedSurge LEC 2 TERM 3 NEURO Application of warm packs Daily exercises • Stretch- hold- relax routine Swimming and stationary bicycling Giving enough time to do activities ✔ Activity and Rest Very strenuous exercise is not advisable Take frequent short rest periods ✔ Preventing Injury Gait training – widen base of support • Teach patient how to walk with feet apart Weighted bracelets or wrist cuffs – aids in coordination ✔ Enhancing Bladder and Bowel Control The sensation of the need to void must be heeded immediately Voiding time schedule Adequate fluids, dietary fiber and bowel training program MOON RARE: affects 1-2 out of 100,000 Most common cause of rapidly acquired paralysis Usually preceded by mild upper respiratory infection or gastroenteritis After the initial and plateau periods, recovery may take up to a year and in some cases, it can cause residual effects and even death due to complications Major concern is difficulty of breathing Cause: Unknown Predisposing Factors Antecedent viral infection- 2 weeks from onset of symptoms (CAMPYLOBACTER JEJUNI, CYTOMEGALOVIRUS, EPSTEIN BARR VIRUS) Immunization- flu vaccine Autoimmune Disorders ✔ Enhancing Communication and Managing Swallowing Difficulties Speech therapy Availability of suction apparatus, careful feeding and proper positioning for eating ✔ Improving Sensory and Cognitive Function Vision: use of eye patch or a covered eyeglass, prism glasses (for diplopia), free talking book Cognition and emotional responses: providing emotional support; assisting in setting meaningful and realistic goals; provision of hobbies; and structured daily routine ✔ Promoting Sexual Functioning Collaboration among the patient, family, and health care provider Sharing and communication of feelings, planning for sexual activity and exploring alternative methods of sexual expression Complications: Complications of Immobility Blindness GUILLAIN-BARRE SYNDROME An acute infectious neuronitis of the cranial and peripheral nerves caused by overreaction of the immune system to an infection resulting to destruction of myelin sheaths Also called Landry’s Paralyis TYPES OF GBS 1) Acute inflammatory demyelinating polyradiculoneuropathy (AIDP). The most common sign of AIDP is muscle weakness that starts in the lower part of the body and spreads upward. 3 MedSurge LEC 2 TERM 3 NEURO MOON 2) Miller Fisher syndrome (MFS), in which paralysis starts in the eyes. MFS is also associated with unsteady gait. MFS occurs in about 5 percent of people with Guillain-Barre syndrome in the U.S. but is more common in Asia. 3) Acute motor axonal neuropathy (AMAN)and acute motor-sensory axonal neuropathy (AMSAN), which are less common in the U.S. but more frequent in China, Japan and Mexico PATHOPHYSIOLOGY: COMPLICATIONS - Segmental demyelination of peripheral nerves causes inflammation and degeneration in sensory and motor nerve roots - Most clients experience spontaneous and complete recovery although mild deficits may persist. Manifestations • Paresthesia • Generalized muscle weakness (starts from lower extremities) - Ascending paralysis • Muscle weakness of the legs (Dyskinesia) • Persistent pain in the back, calves of the legs and may progress to upper extremities, trunk AUTONOMIC DISTURBANCES • Paralysis of the ocular, facial, glossal and esopharyngeal muscles ( DYSPHAGIA AND DYSARTHRIA) Disturbance of heart rate and rhythm • Transient HPN • Orthostatic Hypotension • Paralytic ileus DIAGNOSTICS: CSF studies- elevated CHON levels EMG- diminish electrical activity of skeletal muscles Nerve conduction studies progressive deterioration of nerve conduction velocity ❖ ARF ❖ Cardiac dysrhythmia ❖ DVT and pulmonary embolism ❖ Paralysis ❖ Pressure ulcers ❖ Contractures ❖ Muscle wasting ❖ Aspiration DRUGS: • Propanolol for HPN • Atropine – for bradycardia MEDICAL MANAGEMENT • Mechanical ventilation if respiratory problems are present • Plasmapheresisdecrease circulating antibody • Continuous ECG monitoring • Corticosteroidsdepress immune response • Antiarrhythmic agents Nursing Interventions Maintain adequate ventilation - Monitor rate and depth of respirations; serial vital capacities - Observe for ventilatory insufficiency - Maintain mechanical ventilation as needed Check individual muscle groups every 2 hours Assess cranial nerve function - Check gag reflex and swallowing activity Ability to handle secretions - Voice Monitor vital signs and observe for signs of autonomic dysfunction Prevent complications of immobility ROMs, anticoagulants, antiembolism stockings Promote comfort Promote optimum nutrition (TPN/Gastrostomy) Provide psychological support and encouragement to client/significant MYASTHENIA GRAVIS - Literally means "grave muscle weakness“ - An autoimmune neuromuscular disease leading to fluctuating skeletal muscle weakness and fatigability - Results from failure of nerve transmission at the neuromuscular junction due to inadequate release of ACETYLCHOLINE or inadequate response of muscle fibers to acetylcholine 4 MedSurge LEC 2 TERM 3 NEURO MOON - Affects voluntary muscles especially those which are innervated by the cranial nerves 3 TYPES OF MUSCLES AFFECTED IN MG 1. OCULAR – affects only eye and lid muscles 2. BULBAR – affects muscles for breathing, swallowing and speaking 3. GENERALIZED – OCULAR, BULBAR + NECK and LIMB MUSCLES (most common) Exact Cause: UNKNOWN Decrease in Ach secretion by the motor end plate Increased acetylcholinesterase (enzyme that destroys Ach) at the nerve endings Autoimmune diseases (Thymoma) Normally: When impulses travel down the nerve ↓ Nerve endings release a neurotransmitter substance ↓ Ach travels through the neuromuscular junction ↓ Binds to acetylcholine receptors ↓ Activation and generation of muscle contraction CLINICAL MANIFESTATIONS Subjective Objective: • Ptosis • Extreme muscle weakness • Dysphonia- impaired • Fatigue ability to produce voice • Dysphagia • Strabismus • Diplopia- caused by • Mask-like facial weakening of extraocular expression muscles • Myasthenic smile• Dysarthria SNARLING SMILE • Dyspnea • Drooling • Decreasing vital capacity and respiratory failure 1. 2. 3. 4. PATHOPHYSIOLOGY Antibodies block, alter, or destroy the receptors for acetylcholine at the neuromuscular junction ↓ Communication between the nerve & muscle is interrupted ↓ Muscle contraction is prevented ↓ Skeletal muscle weakness & fatigability DIAGNOSTIC TEST Tensilon test - Edrophonium chloride- a fast acting AChE inhibitor, is administered IV to diagnose MG (+) test: immediate improvement in muscle strength after administration of this agent. Ice pack Test - ice is applied to the eyes for 1-2 minutes; (+) if there’s improvement in lid strength MRI – CHECK THYMUS GLAND EMG – delayed/ failed nerve – transmission MEDICAL MANAGEMENT Directed at improving function and reducing and removing circulating antibodies NO CURE: treatment does not stop production of Ach receptor antibodies PHARMACOLOGICAL MANAGEMENT Pyridostigmine Bromide (Mestinon): anticholinesterase medication; provides symptomatic relief Adverse effects: fasciculations, abdominal pain, diarrhea, increased oropharyngeal secretions Immunosuppressive drugs: to reduce the production of the antibody corticosteroid (P------ ), Monitor CBC Azathioprine (Imuran) Intravenous immune globulin: used to treat exacerbations and long- term adjunctive basis 5 MedSurge LEC 2 TERM 3 NEURO MOON PLASMAPHERESIS a technique used to treat exacerbations patient’s plasma and plasma components are removed through a centrally placed largebore double-lumen catheter the blood cells & antibody containing plasma are separated, after which the cells and a plasma substitute are reinfused SURGICAL MANAGEMENT • THYMECTOMY: surgical removal of the thymus gland; after thymus gland is removed, it may take up to 3 years for the patient to benefit from the procedure, because of the long life of circulating T cells MYASTHENIC CRISES Acute exacerbation of MG Caused by rapid, unrecognized progression of the disease; inadequate amount of medication; infection; fatigue; or stress • Symptoms: respiratory distress, varying degrees of dysphagia, dysarthria, eyelid ptosis, diplopia, and prominent muscle weakness MANAGEMENT Patient is placed in ICU ET intubation and mechanical ventilation Provide ventilator assistance Ongoing assessment of respiratory failure Chest physical therapy Monitor ABG, serum electrolytes, input and output NGT feeding Avoid sedative and tranquilizers CHOLINERGIC CRISIS - Results in depolarization of motor end plates - Caused by overmedication with anticholinesterase NURSING MANAGEMENT • Medication management- 30 mins. before meals MEDICATIONS TO AVOID: Barbiturates Muscle relaxants Morphine Sulfate Neomycin Tranquilizers • Energy conservation Identify the optimal time for rest throughout the day • Minimize the risk of aspiration • Mealtimes should coincide with the peak effects of anticholinesterase medications • Rest before meals • Sit upright during meals • Soft foods- encourage gravy and sauce • Suction should be available at home • Supplemental feedings • Strategies to help with ocular manifestations Tape the eyes closed for short intervals – Regularly instill artificial tears COMPLICATION Respiratory failure Impaired communication Corneal ulceration Myasthenic crisis Cholinergic crisis CLINICAL MANIFESTATIONS Nausea and vomiting, diarrhea and abdominal cramps Pallor Facial muscle twitching Hypotension Intervention: Hold anticholinesterase medication Prepare to administer ANTIDOTE ANTIDOTE – atropine Sulfate (anticholinergic) ET intubation – mechvent 6 MedSurge LEC 2 TERM 3 NEURO MOON PARKINSON’S DISEASE - a.k.a. Parkinson disease, Parkinson's, Idiopathic parkinsonism, Primary parkinsonism, PD, or paralysis agitans - a degenerative disorder of the CNS - a slowly progressing neurologic movement disorder that eventually leads to disability - associated with decreased levels of dopamine - the degenerative or idiopathic form is the most common CAUSE • Unknown for most cases (idiopathic) • Dopamine - acetylcholine disequilibrium RISK FACTORS Age is the largest risk factor (Older than 50-60 years of age) Men are affected about 1.5 to 2 times more often than women. Genetics; A small number of individuals are at increased risk because of a family history of the disorder Head trauma & illness Exposure to environmental toxins (pesticides & herbicides) NORMAL A substance called DOPAMINE ↓ Messenger between 2 brain areas - substantia nigra & the corpus striatum ↓ Produces smooth, controlled movement BASAL GANGLIA is a collection of nuclei which has different parts: • CAUDATE NUCLEUS, d/o: overactive: OCD; Huntington’s, depression • PUTAMEN (perform automatic behaviors: riding a bike, typing in the keyboard, driving) d/o: TOURETTE’S SYNDROME • SUBSTANTIA NIGRA (black substance from neuromelanin) – necessary for smooth muscle movement d/o: PARKINSON’S DISEAES PATHOPHYSIOLOGY Dopaminergic neuronal cells is destroyed ↓ Depletion of dopamine stores ↓ Degeneration of the dopaminergic nigrostriatal pathway ↓ Imbalance of acetylcholine & dopamine neurotransmitters in the corpus striatum ↓ Impairment of extrapyramidal tracts controlling complex body movements ↓ Tremors, Rigidity, Bradykinesia, Postural changes BASAL GANGLIA FUNCTION VOLUNTARY MOVEMENTS; ROUTINE BEHAVIORS, COGNITION, LEARNING, EMOTION, etc. 7 MedSurge LEC 2 TERM 3 NEURO CLINICAL MANIFESTATIONS Early onset- the most obvious symptoms are movement-related include: Shaking Rigidity Slowness of movement Difficulty with walking & gait. Later MOON It may become difficult to start walking & to make turns Individuals may freeze in mid-stride and appear to fall forward while walking. Cognitive & behavioral problems Dementia; occurring in the advanced stages of the disease Sensory, sleep & emotional problem 4 CARDINAL SIGNS OF PD TREMOR - A slow, unilateral resting, trembling in fingers, hands, (TURNING MOTION), arms, feet, legs, jaw, or head - Occur when individual is resting, but not while involved in a task - Tremors worsen; when excited, tired, or stressed - May manifest a motion of the thumb against the fingers as if rolling a pill between the fingers RIGIDITY Stiff & resistant limbs & trunk that increase during movement Muscle aches & pain Handwriting (micrographia)/ eating difficulty lead-pipe rigidity; cogwheel Asymmetrical in early stages (neck & shoulder muscles prior to the muscles of the face & extremities) With progression, rigidity typically affects the whole body & reduces the ability to move. BRADYKINESIA Slowness of voluntary movement Difficult to initiate/complete movement Bradykinesia together with stiffness affect the facial muscles leading to expressionless, "masklike" appearance Initial manifestations- problems when performing daily tasks which require fine motor control: writing, sewing or getting dressed. Severe form: FREEZING PHENOMENON – transient inability to perform active movement. Walking – “glued” POSTURAL INSTABILITY Impaired or lost reflexes Postural & gait problems---”FALLS” The posture is caused by the forward flexion of the neck, hips, knees & elbows The patient may walk faster & faster, trying to move the feet forward under the body’s center of gravity (shuffling gait) More progressive Parkinson's disease develop a distinctive shuffling walk with a stooped position & a diminished or absent arm swing Other Manifestations • Psychiatric changes- depression, dementia (progressive mental deterioration), delirium, hallucinations • Mental changes- cognitive, perceptual & memory deficits; intellect is not usually affected. • Dysphonia (soft, slurred, low-pitched, less audible speech) • Dysphagia, begins to drool, & at risk for choking & aspiration Autonomic symptoms HYPERHIDROSIS, ORTHOSTATIC HYPOTENSION GASTRIC, URINARY RETENTION; CONSTIPATION SEXUAL DYSFUNCTION COMPLICATIONS - Patients are at risk for: Respiratory problems UTI Skin breakdown Injury from falls DIAGNOSTIC TESTS PET & Single Photon Emission Computed Tomography (SPECT)- FINDINGS: nigrostriatal dysfunction EEG No tests is diagnostic of PD - Most accepted: Pt’s history + at least 2 of 4 cardinal signs 8 MedSurge LEC 2 TERM 3 NEURO MOON MEDICAL MANAGEMENT Treatment are directed at controlling symptoms & maintaining functional independence Care in individualized for each patient based on presenting symptoms & social, occupational, & emotional needs Patient are usually cared for at home & are admitted to the hospital only for complications or to initiate new treatments PHARMACOLOGIC THERAPY - - - Antiparkinsonian medications act by: Increasing striatal dopaminergic activity Restoring a balance between dopaminergic & cholinergic activities Acting on neurotransmitter pathways other than the dopaminergic pathway Levodopa (Larodopa)/ b with levodopa (Sinemet)- the most effective agent & the mainstay treatment Converted to dopamine in the basal ganglia, producing symptom relief Avoid the following when on Levodopa Tyramine rich foods (prevent hypertensive crisis) B6 (pyridoxine) NURSING DIAGNOSES Impaired physical mobility related to muscle rigidity & motor weakness Self-care deficits related to tremor & motor disturbance Constipation related to medication & reduced activity Imbalanced nutrition Impaired verbal communication Ineffective coping NURSING INTERVENTIONS Improving mobility (progressive program of daily exercise) Enhancing self-care activities (independence) Improving bowel elimination Improving nutrition (high residue, high caloric, soft diet) Encouraging the use of assistive devices Improving communication (refer to speech therapy) Supporting coping abilities Promoting Home & community based care • SLEEPING: avoid pillows, use firm mattress, prone position to minimize stooped posture SURGICAL MANAGEMENT • Surgery provides symptom relief in selected patients 1. Stereotactic procedures: To interrupt the nerve pathways & thereby alleviate tremors or rigidity Thalamotomy; a stereotactic electrical stimulator destroys part of the ventrolateral portion of the thalamus in an attempt to reduce tremor Pallidotomy; involves destruction of part of the ventral aspect of the medial globus pallidus through electrical stimulation in patients with advanced disease. DEEP BRAIN STIMULATOR – insertion of a pacemaker like device with electrodes attached to thalamus to relieve tremors 9
