Autoimmune Neurological Disorders PDF

Autoimmune Neurological Disorders  Multiple sclerosis  Myasthenia gravis  Guillain-Barré syndrome  Multiple Sclerosis (MS)  A progressive immune-related demyelination of the white matter of the CNS and damage to the nerve fibers and their targets  Demyelina...

Autoimmune Neurological Disorders  Multiple sclerosis  Myasthenia gravis  Guillain-Barré syndrome  Multiple Sclerosis (MS)  A progressive immune-related demyelination of the white matter of the CNS and damage to the nerve fibers and their targets  Demyelination refers to the destruction of the myelin – the fatty and protein materials that surrounds nerve fibers in the brain and spinal cord  …it results in impaired transmission of nerve impulses  Multiple Sclerosis (MS) Causes:  …exact causes is unknown  but is related to slow-acting or latent viral infection and autoimmune response  Other theories suggests environmental and genetic factors may also be linked to MS  Emotional stress, overwork, fatigue, pregnancy, and acute respiratory infection may precede the onset of illness Process of Demyelination  Multiple Sclerosis Pathophysiology:  Sensitized T cells typically crosses the blood-brain barriers  main function is to check the CNS for antigen and then leave  However, in MS the T cells remains and promotes the infiltration of other agents that damage the immune system  Immune system initiates an inflammatory response that attacks the myelin  Multiple Sclerosis Complication:  Injuries from fall  UTI  constipation  Joint contracture  Pressure ulcers  Rectal distention  Pneumonia  Multiple Sclerosis: Signs and Symptoms  …depends on the site of myelin destruction  Visual problems and sensory impairment such as numbness and tingling sensation (paresthesia)  Ocular disturbances (optic neuritis, diplopia, opthalmoplegia, blurred vision, and nystagmus)  Multiple Sclerosis: Signs and Symptoms  Muscle dysfunction (weakness, paralysis ranging from monoplegia to quadriplegia, spasticity, hyperreflexia, intention tremor, and gait ataxia  Urinary disturbances (incontinence, urgency, frequency and frequent infection  Emotional lability (mood swings, irritability, euphoria, and depression)  Often, the disease relapses and remits, exacerbates, and symptoms recur including fatigue, weakness, numbness, difficulty in coordination, loss of balance, pain, and visual disturbances Types and Courses of Multiple Sclerosis  Multiple Sclerosis: Diagnosis  MRI – detects MS lesions  ECG  LP – shows elevated gamma globulin fractions in the CSF  WBC count may rise  Characteristics of relapse and remission of the disease or physical signs and symptoms Multiple Sclerosis CT Scan and MRI  Multiple Sclerosis: Treatment  Medical management › Disease-modifying therapies: interferon -1a and interferon -1b, glatiramer acetate (Copaxone), and IV methylprednisolone › Symptom management of muscle spasms, fatigue, ataxia, and bowel and bladder control  The aim of treatment is to shorten exacerbation and relieve neurologic deficits  Multiple Sclerosis: Treatment  Those with relapse-remitting courses are placed on immune modulating therapy, with interferons or glatiramer acetate.  Steroids are used to reduce the associated edema of the myelin sheaths during exacerbation  Other drugs: baclofen, tizadine, or diazepam to relieve muscles spasticity  Cholinergic agents to relieve urine retention and minimize frequency and urgency  Amantadine to relieve fatigue  Multiple Sclerosis: Treatment  Anti-depressants – for mood swings and behavioral symptoms  During exacerbation: supportive measures includes bed rest, comfort measures, massage, prevention of fatigue, pressure ulcers, bowel and bladder training,  Antibiotics for bladder infection  Physical and speech therapy  Counseling  Exercise program to maintain muscle tone Nursing Process—Assessment of the Patient With MS  Neurologic deficits  Secondary complications  Impact of disease on physical, social, and emotional function and on lifestyle  Patient and family coping Nursing Process—Diagnosis of the Patient With Multiple Sclerosis  Impaired physical mobility  Risk for injury  Impaired bowel and bladder function  Impaired verbal communication  Disturbed thought processes  Ineffective coping  Impaired home maintenance  Potential sexual dysfunction  Nursing Process—Planning the Care of the Patient With Multiple Sclerosis  Major goals may include: › Promotion of physical mobility › Avoidance of injury › Achievement of bowel and bladder continence › Promotion of speech and swallowing mechanisms › Improvement in cognitive function › Development of coping strengths › Improved home maintenance › Adaptation to sexual function  Myasthenia Gravis  Autoimmune disorder affecting the myoneural junction  A disorder of transmission at the neuromuscular junction that affects communication between the motoneuron and the innervated muscle cells  Affects women than men, peaks at ages 20 and 30 years old  Antibodies directed at acetylcholine at the myoneural junction impair transmission of impulses, although the exact mechanism that triggers the autoimmune response is still unclear, it is thought that T cells have a key role  Myasthenia Gravis  75% of people with MG also have thymic abnormalities such as thymoma (thymus tumor) or thymic hyperplasia (increased thymus cells)  Lambert-Eaton myasthenic syndrome is a special type of MG that develops in association with neoplasm particularly small cell carcinoma of the lungs  Neonatal myasthenia gravis is caused by placental transfer of Ach receptors antibody  Myasthenia Gravis Normal ACh receptor site ACh receptor site in myasthenia gravis  Myasthenia Gravis: Signs and Symptoms  Progressive skeletal muscle weakness  Fatigability  Weak eye closure  Ptosis  Diplopia  Blank and expressionless faces and nasal vocal voices impairment (dysphonia)  Frequent nasal regurgitation and difficulty chewing and swallowing  Weakened respiratory muscles with decreased tidal volume and vital capacity  Respiratory infection  Myasthenia Gravis: Diagnosis  History and physical examination  Anticholinesterase test – uses drugs that inhibit acetylcholinesterase (e,g. Tensilon test – edrophonium chloride)  Nerve stimulation studies  Immunoassay test – detects the presence of Ach receptor antibodies in the blood  Single – fiber electromography – detects delayed or failed neuromuscular transmission in muscle fibers supplied  Medical Management  Pharmacologic therapy › Immunosuppressive (corticosteroids, azathioprine and cyclosporine) › Pyridostigmine and neostigmine – inhibits breakdown of Ach at the neuromuscular junction by acytelcholinesterase  Plasmapheresis  Thymectomy  Intravenous immunoglobulin therapy  Note: Aminoglycoside antibiotic should be avoided due to exacerbation of the disease  Plasmapheresis  Is the removal of antibodies from the circulation and provides short term clinical improvement  …used to stabilize the condition of persons in myesthenic crises or for short-term treatment in under going thymectomy Myasthenic Crisis Cholinergic Crisis  Result of disease  Caused by exacerbation or a overmedication with precipitating event, most cholinesterase inhibitors commonly a respiratory  Severe muscle weakness infection with respiratory and  Severe generalized bulbar weakness muscle weakness with  Patent may develop respiratory and bulbar respiratory compromise weakness and failure  Patient may develop respiratory compromise failure  Management of Myasthenic Crisis  Patient instruction in signs and symptoms of myasthenic crisis and cholinergic crisis  Ensuring adequate ventilation; intubation and mechanical ventilation may be needed  Assessment and supportive measures include: › Ensure airway and respiratory support › Take ABGs, serum electrolytes, I&O, and daily weight › If patient cannot swallow, nasogastric feeding may be required › Avoid sedatives and tranquilizers  Nursing Process—The Care of the Patient With Myasthenia Gravis  Focus on patient and family teaching  Provide medication teaching and management  Implement energy conservation measures  Implement strategies to help with ocular manifestations  Prevent and/or manage complications and avoid crisis  Implement measures to reduce risk of aspiration  Avoid stress, infections, vigorous physical activity, some medications, and high environmental temperatures  Interventions  Utilize a collaborative approach  Coordinate and refer as needed to health care services, social services, speech therapy, physical therapy, counseling services, home care services  Activity and rest › Implement program of activity and daily exercise › Implement relaxation, coordination, and muscle stretching exercises and walking › Avoid very strenuous activity and extreme fatigue  Interventions (cont.)  Bowel and bladder control › Instruction or administration of prescribed medications › Voiding schedule › Bowel training program › Adequate fluid and fiber to prevent constipation  Reinforce and encourage swallowing instructions  Strategies to reduce risk of aspiration  Memory aids, structured environment, and daily routine to enhance cognitive function  Interventions (cont.)  Implement measures to minimize stress  Maintain temperate environment: air conditioning to avoid excessive heat and avoidance of exposure to extreme cold  Use assistive devices and modify home care management and independence in ADLs  Support coping ability  Guillain-Barré Syndrome  Aka: infectious polyneuritis, Lady – Guillain –Barré syndrome  Autoimmune disorder with acute attack of peripheral nerve myelin  …is an acute, rapidly progressive, and potentially fatal form of polyneuritis that causes muscle weakness and mild distal sensory loss  Guillain-Barré Syndrome: Causes  The precise cause is unknown but it may be a cell-mediated immune response with attack on the peripheral nerves in response to a virus (cytomegalovirus and Epstein – Barr virus  Campylobacter jejuni  Precipitating factors: minor febrile illness, respiratory tract infection, gastroenteritis, surgery, rabies, systemic lupus erythematosus, vaccinations, and viruses  Guillain-Barré Syndrome: Types  Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)  Miller Fisher syndrome (MFS)  Acute motor axonal neuropathy (AMAN)and acute motor-sensory axonal neuropathy (AMSAN)  Guillain-Barré Syndrome: Signs and Symptoms  Muscle weakness: › Dyskinesia (inability to execute voluntary movement › Hyporeflexia › Paresthesia (numbness)  Manifestations are variable and include weakness, paralysis, paresthesias, pain, diminished or absent reflexes starting with the lower extremities and progressing upward, bulbar weakness, cranial nerve symptoms, tachycardia, bradycardia, hypertension, or hypotension  Three-Phase Course: Guillain- Barré Syndrome  Initial Phase – begins when the first definitive symptom develops; ends 1 to 3 weeks later, when no further deterioration noted  Plateau phase – lasts for several days to 2 weeks  Recovery phase – remyelination and axonal process regrowth, lasts for 6 months to two years.  Complications: Guillain-Barré Syndrome  Mechanical ventilation failure  Aspiration  Pneumonia  Sepsis  Joint contractures  Deep vein thrombosis  Tachycardia  Loss of bowel/sphincter control  Guillain-Barré Syndrome: Diagnostics  History of preceding febrile illness  Increased protein level and WBC of the CSF due inflammatory disease – more definitive diagnosis of condition  Symmetric body weakness  Diminished reflexes noted  Guillain-Barré Syndrome: Treatment  Pallative  Endotracheal tube or tracheotomy for patient with difficulty clearing out secretions  A trial dose of prednisone on the initial phase of the disease, and is discontinued if no effect.  Plasmapheresis is also useful during initial phase  Guillain-Barré Syndrome (cont.)  Medical management › Requires intensive care management with continuous monitoring and respiratory support › Plasmapheresis and IVIG are used to reduce circulating antibodies  Recovery rates vary but most patients recover completely  Nursing Process—Assessment of the Patient With Guillain-Barré Syndrome  Conduct ongoing assessment 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  Encourage patient and family coping  Nursing Process—Planning the Care of the Patient With Guillain- Barré Syndrome  Major goals include: › Improved respiratory function › Increased mobility › Improved nutritional status › Effective communication › Decreased fear and anxiety › Effective patient and family coping › Absence of complications  Interventions  Enhance physical mobility and prevent DVT › Support limbs in a functional position › Perform passive ROM at least twice daily › Initiate position changes at least every 2 hours › Provide elastic compression hose and/or sequential compression boots › Provide adequate hydration  Administer IV and parenteral nutrition as prescribed  Carefully assess swallowing and gag reflex and take measures to prevent aspiration  Interventions (cont.)  Develop a plan for communication individualized to patient needs  Decrease fear and anxiety › Provide information and support › Provide referral to support group › Implement relaxation measures › Maintain positive attitude and atmosphere to promote a sense of well-being › Implement diversional activities  Cranial Nerve Disorders  Trigeminal neuralgia (tic douloureux)  Bell’s palsy  Trigeminal Neuralgia (Tic Douloureux)  Condition of the 5th cranial nerve characterized by paroxysms of pain › Most commonly occurs in the 2nd and 3rd branches of this nerve; vascular compression and pressure is the probable cause  Occurs more often in persons in their 50s and 60s, in women, and in persons with multiple sclerosis  Pain can occur with any stimulation such as washing face, brushing teeth, eating, or a draft of air  Patients may avoid eating, neglect hygiene, and may even isolate themselves to prevent attacks  Distribution of the Trigeminal Nerve Branches  Medical Management  Antiseizure medications such as carbamazepine (Tegretol), gabapentin (Neurontin), phenytoin, or the antispasmodic medication baclofen (Lioresal)  Surgical treatment › Microvascualr decompression of the trigeminal nerve › Radiofrequency thermal coagulation › Percutaneous balloon microcompression  Nursing Interventions  Patient teaching related to pain prevention and treatment regimen  Measures to reduce and prevent pain; avoidance of triggers  Care of the patient experiencing chronic pain  Measures to maintain hygiene: washing face, oral care  Strategies to ensure nutrition: soft food, chew on unaffected side, and avoid hot and cold food  Recognize and provide interventions to address anxiety, depression, and insomnia  Bell’s Palsy  Facial paralysis due to unilateral inflammation of the 7th cranial nerve  Manifestations: unilateral facial muscle weakness or paralysis with facial distortion, increased lacrimation, painful sensations in the face, and possible difficulty with speech and eating  Most patients recover completely in 3 to 5 weeks, and the disorder rarely recurs  Management  Medical › Corticosteroid therapy may be used to reduce inflammation and diminish severity of the disorder  Nursing › Provide and reinforce information and reassure patient that a stroke has not occurred › Protect the eye from injury, cover the eye with a shield at night, and instruct the patient to close the eyelid, use eye ointment, and wear sunglasses › Implement facial exercises and massage to maintain muscle tone