Myasthenia Gravis autoimmune disorder affecting the myoneural junction voluntary muscles weakness 60,000 women than men

20-40 early onset 60-70 for men purely motor disorder (no effect on sensationa & coordination)

CM Initial 1. diplopia (double vision) 2. ptosis (drooping of the eyelids) other 1. face (bland expression) and throat muscle weakness (bulbar symptoms) 2. generalized weakness(extremities & intercostals muscles) 3. dysphonia (voice impairment) 4 risk for choking and aspiration 5. decrease vital & respiratory failure Assessment and diagnostic findings

Pathophysiology thymic hyperplasia or thymic tumor

offending Antibodies directed at acetylcholine receptor sites

1. acetylcholinesterase inhibitor test ( used to diag. MG) acetylcholinesterase inhibitor -stops breakdown acetylcholine = inc. availability Edrophonium chloride (Tensilon) -fast-acting acetylcholinesterase inhibitor(via IV) - 30 sec. after inject. facial muscle weakness and ptosis (resolve5 mins) atropine - control SE of edrophonium (bradycardia, sweating, and cramping) 2. MRI 3. EMG (electromyography) detects a delay or failure of neuromuscular transmission, 99% confirming MG Med. Mgt

impaired transmission of impulse across the myoneural junction

fewer receptors are available for stimulation

weakness in voluntary muscle 1. anticholinesterase 2. immunosuppressive therapy 3. plasmapheresis 4. thymectomy No cure, treatments do not stop the production of the acetylcholine receptor antibodies

Pharmacologic Therapy 1. Pyridostigmine bromide (Mestinon) -anticholinesterase -1st line of therapy -inhibiting break of acetyl and inc. availability at NJ -dose gradual inc. to a daily maximum (qid) AE 1.fasciculations 2. abdominal pain 3. diarrhrea 4. inc. oropharyngeal secretions fewer SE compare to other anticholinesterase med. 2. Immunomodulating drugs - if Mestinon not effective -reduce production of antibody corticosteroids - suppress immune response prednisone initially given when symptoms improve

Plasmapheresis - plasma exchange (treat exacerbation) - pt plasma & plasma components removal - daily or alternate day - 75% improvements but last only few weeks Surgical Mgt. 1. Thymectomy - transsternal surgical(to removed entire gland) - 3yrs before it can benefit from procedure Complications 1. Respiratory failure Nsg. Mgt 1. pt and family teaching 2. educational topic (self-care,med mgt, E conservation, help ocular manifestation, prevention & mgt of complications) Note: anticholinesterase med must be administered on time

3. cytotoxic med - if steroids not effective Azathioprine (Imuran) - inhibits T cells & reduces acetylcholine receptor antibody levels Therapeutic Effect may not be evident for 3 12 months Serious AE 1. leukopenia 2. hepatotoxicity monthly evaluation of liver enzymes & WBC is necessary 4. Intravenous immune globulin (IVIG) - treat exacerbation/longterm adjunctive basis -easy/pooled human gamma-globulin -improve in few days Procaine (Novocain) - avoid / informed dentist

Myathenic crisis - repi. distress - dysphagia - dysarthia - ptosis - diplopia - muscle weakness

Alzheimers Disease Factors 1. inc. age 2. environmental 3. dietary 4. inflammatory factor 5. genetics 6. neurotransmitter changes 7. vascular abnormalities 8. stress hormones 9. circadian changes 10. head trauma 11. seizure disorders irreversible, degenerative neurologic disease insidiously, losses of cognitive fxn & distru. in behavior & affect 40, uncommon before 65 not normal part of aging

pathophysiology neuropathologic changes biochemical changes

neurofibrillary tangles & neuritic plaques neural damaged occur cerebral cortex decreased brain size affects acetylcholine production

impaired memory processing

S&SX AD CM

Types early stage 1. familial or early onset AD - rare -<10% of all cases -associated with genetic mutations -middle aged adults 2. sporadic or late-onset AD 1. forgetfulness 2. subtle memory loss 3. may experience small diff. in work & social act. bt adequate cognitive fxn further progression 1. agnosia 2. amnesia 3. disoriented 4. aphasia 5. personality changes 6. wandering at night 7. dysphagia 8. incontinence terminal stage

1. immobile 2. death (pneumonia, malnutrition, or dehydration) Assessment and Diagnostic findings - autopsy - history , PE - CBC, chemistry profile & vit. 12, thyroid hormone levels - electroencephalography, CT - MRI - examination of the CSF Depression - mimic early-stage - MMSE test (chpter 12-2) Med mgt Drugs 1. cholinesterase inhibitors (CEIs) 2. donepezil hydrochloride (Aricept) & memantine (Namenda) 3. rivastigmine tartrate (Exelon) 4. galantamine hydrobromide (Razadyne) 5. tacrine (cognex) - enhance acetylcholine uptake in the brain - used for mild to moderate symptoms - Aricept & memantine (receptor gonist, moderate to severe) - improve. 6 to 12 m but cessation of med. results in disease progression - recommend continuing at least in moderate stage - CEI with memantine for mild to moderate cognitive symptoms behavioral and psychosocial therapies agitation psychosis manage cognitive & behavioral symptoms no cure and no way to slow the progression

depression

Nsg. Mgt promoting function and independence physical safety self care reducing anxiety agitation improving communication socialization adequate nutrition balanced activity & rest family education

patho

Multiple Sclerosis - is an immune-mediated - progressive demyelinating disease of the CNS - impaired transmission of nerve impulses - any age but typically 20-40 years of age - women - Europe, new Zealand, southern Canada factors environmental factors genetic predisposition virus

T cells remain in CNS promote infiltration of other agents

damaged immune system immune system attacks demyelination

interrupts flow of nerve impulse

optic nerves, chiasm, tracts; cerebrum; brain stem, cerebellum spinal cord S&Sx MS

CM benign course symptoms are mild does not seek treatment

MS type & course 1. Relapsing-remitting (RR) course 80 85 % acute attacks with full recovery with sequelae & residual deficit upon recovery 50 % progress to secondary progressive course

2. depression 3. weakness 4. numbness 5. difficulty in coordination 6. loss of balance 7. pain 8.visual disturbances 9.blurring of vision 10. diplopia 11. patchy blindness and total blindness 12. spasticity 13. ataxia Gerontologic Considerations secondary progressive disease - average 35 years after onset Assesment and diagnostic findings 1. MRI observe plaques in CNS 2. Electrophoresis of CSF - identifies presence of oligoclonal banding 3. evoked potential studies - help define the extent of the disease process and monitor changes 5. urodynamic studies - bladder dysfunction 6.neuropsychological testing - assess cognitive impairement 7. sexual history - identify changes in sexual function Med mgt - no cure Goals - to delay the progression, manage chronic symptoms, treat acute exacerbations

2. primary progressive course 10% disabling symptoms steadily increase rare plateaus and temporary improvement quadriparesis, cognitive dysfunction, visual loss brain stem syndromes

3. secondary progressive - MS begins with an initial RR course, followed by progression of variable rate, which may also include occasional relapses and minor remissions. 4. progressive-relapsing (PR) - least common 5% - MS shows progression from onset but with clear acute relapses with or without recovery. Primary symptoms 1. fatigue

Pharmacologic therapy 1. disease-modifying therapies - reduce frequency, duration of relapse - number & size of plaques - all med. require injection interferon beta-1a (Rebif) & interferon beta-1b (Betaseron) - administered subcutaneously Avonex - intramuscularly once a week. Side effects 1. flulike symptoms (managed w/ acetaminophen & ibuprofen) 2. potential liver damage, fetal abnormalities, and depression NOTE: for optimal control should be started early in the course of the disease Glatiramer acetate (Copaxone) - reduces the rate of relapse in RR course - decrease # plaques - subcutaneously daily - it acts by increasing the antigen-specific suppressor T cells Side effects 1. minimal and manageable Note: it takes 6 months for evidence of an immune response to appear

3. electrolyte imbalance Mitoxantrone (Novantrone) - via IV infusion every 3 months - reduce frequency of relapse w/ secondary-progressive or worseining relapsing-remitting MS SE - cardiac toxicity symptom mgt spasticity 1. Baclofen 2. GABA agonis disabling spasm and contractures 1. nerve blocks or surgical intervention fatigue 1. amantadine (Symmetrel) 2. pemoline (Cylert) 3. fluxetine (Prozac) ataxia 1. beta adrenergic blocker (Inderal) 2. antiseizure agents (Neurontin) 3. benzodiazepines (Klonopin) bladder and bowel problems

IV methylprednisolone - key agent treating acute relapse in RR course - shorten the duration of relapse - 1 gram IV daily for 3days, ff by oral taper of prednisone SE 1. mood swing 2. weight gain

1. anticholinergic agents 2. alpha-adrenergic blockers 3. antispasmodic agents UTI 1. ascorbic acid ( vit. C)

Guillain-Barre syndrome causes autoimmune attack on the peripheral nerve myelin acute, rapid segmental demyelination of peripheral nerves and cranial nerves dyskinesia,hyporeflexia,paresthesias 1 2 cases per 100,000 males between 16 and 25 and between 45 and 60 years of age 60% - 75% recover completely 20% - 25% residual deficits (rapid disease progression) does not affect cognitive function or LOC

CM classical features 1. areflexia 2. ascending weakness 3. sensory symptoms 4. Miller-fisher varian ( paralysis of ocular muscle ataxia, and arefexia) other 1. muscle weakness 2. diminished reflexes of the lower extremities 3. hyporeflexia and weakness may progress to tetraplegia 3. neuromuscular respiratory failure 4. paresthesias Assement and diag findings 1. history of viral illness 2. changes in vital capacity and negative inspiratory force 3. Evoked potential studies

viral infection - campylobacter jejuni - cytomegalovirus - Epstein-barr virus - mycoplasma pneumonia - H. influenza and HIV Patho cell-mediated and humural immune attack on peripheral nerve myelin proteins (ganglioside GM1b) inflammatory demyelination interrupted nerve conduction and axonal loss S&sx

medical management - require intensive care 1. respiratory therapy or mechanical ventilation 2. elective intubation 3. anticoagulant agent 4. anti-embolism stocking or sequential compression boot to prevent thrombosis and pulmonary emboli 5. plasmapheresis and IVIG ( IVIG DOC) 6. ECG

Bells Palsy facial paralysis caused by unilateral inflammation of the 7th cranial nerve unknown 45 year of age pressure paralysis

causes CM 1. tearing 2.painful sensations in face, ear, eye 3. speech difficutles 4.unable to eat on affected side Med management to maintain muscle tone oof the face and to prevent or minimize denervation 3 5 weeks recovery vascular ischemia viral disease autoimmune disease

corticosteroid therapy (prednisone) - reduce inflammation and edema - reduces vascular compression and permits restoration of blodd circulation to the nerve. analgesic controlled facial pain