Neuro disorder part 3

Amyotrophic Lateral Sclerosis (ALS)

• Also known as Lou Gehrig's disease, it is a rare, progressive, and fatal condition with no cure
• It involves the degeneration of motor neurons in the brainstem and spinal cord, disrupting signals to muscles
• Electrical and chemical messages from the brain fail to reach the muscles, impairing muscle function
• Patients remain cognitively intact, fully aware of their physical decline
• The cause is unknown
• Onset typically occurs between ages 55 and 75
• Men are slightly more susceptible than women
• The average survival time from onset to death is two to six years
• Caucasians and Hispanics have an increased risk
• Veterans face a 1.5 times greater risk, potentially due to environmental toxins
• Results in the loss of both upper and lower motor neurons
• Death is commonly caused by respiratory infection due to weakening of respiratory muscles

Primary Signs and Symptoms of ALS

• Weakness in the upper extremities, often beginning in the hands
• Dysarthria (difficulty speaking)
• Dysphagia (difficulty swallowing)
• Muscle wasting due to lack of nerve impulses from the brain
• Fasciculations (muscle twitches) due to denervation
• Denervation refers to the lack of nerve stimulation to muscles

Diagnosis and Treatment of ALS

• Diagnosis involves electromyogram (EMG), nerve conduction study (NCS), MRI, and lab work to rule out other disorders
• Treatment includes riluzole, which can protect motor neurons and extend life by a few months
• Other medications address muscle spasms and excessive oral secretions as needed
• Riluzole decreases the intensity of ALS symptoms
• Therapy (physical, occupational, speech, and respiratory) is essential for ALS care

Supportive Care for ALS Patients

• Nutritional consults are necessary to maximize diet and prevent further weakening
• Moderate-intensity endurance exercises for the trunk and limbs can decrease spasticity
• Communication should be facilitated
• Provide diversional activities
• Emotional support is crucial for patients and their families

Huntington's Disease

• Chronic, progressive, hereditary disease transmitted genetically
• Offspring have a 50% risk of inheriting and developing
• Average onset is between 30 and 50 years of age
• Death typically occurs 10 to 30 years following onset
• Characterized by an overproduction of dopamine, unlike Parkinson's disease
• Ultimately fatal with no cure available
• Diagnosis is based on family history, signs and symptoms, and DNA analysis to identify characteristic genetic patterns
• Symptoms often do not manifest until after individuals have had children, making genetic counseling and family planning challenging

Huntington's Disease

• Many individuals may already have children, a factor to consider regarding the genetic implications
• Involves excessive, involuntary movements known as Korea, characterized by writhing of limbs and facial muscles
• Patients experience difficulties in chewing and swallowing
• Speech impairment is common
• High risk of aspiration and malnutrition
• Intellectual decline will occur
• Gait unsteadiness will progress to severe mobility issues
• Patients will experience a high risk of falls
• Bowel and bladder incontinence may develop
• Patients will express labile emotions like happiness, sadness, crying, or anger
• Psychotic behaviors, including paranoia and hallucinations, may manifest
• Patients will have a ravenous appetite due to the high caloric expenditure from constant muscle movement
• Malnutrition can occur due to difficulties in chewing, swallowing, and the need for high caloric intake
• Treatment focuses on managing signs and symptoms rather than curing the disease
• Medications prescribed include antidepressants, antipsychotics, and anti-Korea drugs like clonazepam
• A diet of 4,000 to 5,000 calories per day, consisting of easily chewed and swallowed foods, is necessary
• Nursing care involves providing a comfortable environment,maintaining ambulation as long as possible, and ensuring a safe environment
• Emotional support is essential
• Driving is not permitted due to the risk of accidents or injuries
• Fall and choking hazards must be addressed
• High suicide risk necessitates careful monitoring and intervention
• Genetic counseling is recommended to assess the risk of being a carrier
• DNA analysis can determine carrier status but cannot predict the expression of signs and symptoms

Cerebrovascular Accident (Stroke)

• Oxygen supply to a brain area is reduced or cut off, leading to cellular death
• Alternate term of Brain Attack
• Risk factors include smoking, high blood pressure, diabetes, and certain birth control methods
• Hypertension is the most modifiable risk factor
• Coronary artery disease is a risk factor
• Atherosclerosis is the most common underlying cause
• Plaque buildup in blood vessels restricts blood flow, potentially leading to permanent brain damage or death
• Hypertension and diabetes accelerate atherosclerosis
• Symptoms often occur during or shortly after sleep, related to circadian rhythm
• Symptoms worsen for the first few hours and peak at 72 hours
• Strokes primarily affect individuals aged 60-90 but can occur at any age
• Risk factors include atrial fibrillation, heart disease, kidney disease, peripheral vascular disease, family history of stroke, high cholesterol, stress, and sedentary lifestyle
• Circadian rhythm influences the timing of strokes due to vital sign increases anticipating waking up (AM surge)

Embolic Stroke

• Embolic strokes typically occur in younger individuals
• Emboli often originate from a thrombus in the endocardial layer of the heart
• Common sources include rheumatic heart disease, mitral stenosis, atrial fibrillation, myocardial infarction, infective endocarditis, valve prosthesis, and atrial septal defect
• Other causes include air emboli, fat emboli, amniotic fluid emboli (after childbirth), and tumors
• These clots can block blood vessels, leading to brain damage

Hemorrhagic Stroke

• Hemorrhagic stroke involves bleeding into the brain or subarachnoid space
• Bleeding destroys and replaces brain tissue
• Peak incidence for aneurysms is between 35 and 60 years of age
• Occurs more frequently in women
• High risk of recurrent rupture 7-10 days after the initial stroke
• Hemorrhage spreads rapidly, causing damage and irritation to cerebral vessels
• Blood irritates cerebral tissue
• It takes three weeks for the blood to be reabsorbed
• Prognosis is generally poor
• Aneurysms, caused by atherosclerosis, hypertension, trauma, or infection, can rupture, leading to rapid signs and symptoms
• Deficits depend on the affected brain area
• Patients are typically unconscious and may experience seizures
• Permanent brain damage or death can result
• Frontal lobe involvement leads to movement problems, parietal lobe to sensory issues, and occipital lobe to vision problems
• Patients will exhibit changes in level of consciousness and sensory deficits like aphasia
• Hemianopsia (loss of part of the visual field) and loss of proprioception may occur
• Motor defects such as hemiplegia (paralysis) or hemiparesis (weakness) can manifest
• Hemorrhage typically presents with a sudden, explosive headache

Right Brain Damage

• Causes paralysis on the left side of the body (hemiplegia)
• Left-sided neglect, where the patient may forget or ignore the affected side
• Spatial perceptual deficits, affecting distance judgment
• Tendency to deny or minimize problems
• Rapid performance and short attention span
• Impulsiveness, leading to safety risks
• Impaired judgment and time concept

Left Brain Damage

• Impaired speech and language abilities (aphasias)
• Impaired right and left discrimination
• Slow and cautious behavior
• Awareness of deficits, leading to depression and anxiety
• Impaired comprehension related to language and math

Stroke Diagnosis and Treatment

• Diagnosis involves CT scans to determine size, location, and type of stroke
• MRI provides detailed information about brain injury
• Early diagnosis leads to better outcomes
• Treatment depends on the cause of the stroke
• Hemorrhagic strokes require stopping the bleed
• Craniotomy
• Embolectomy
• Aneurysm clipping
• Clot removal
• Thrombolytics, like TPA, are used to dissolve clots
• TPA must be administered within three hours (optimally) or up to four and a half hours from the event
• Anticoagulants (Heparin, Lovenox, Coumadin) are used
• Corticosteroids decrease intracranial pressure
• Stool softeners, laxatives, or enemas prevent straining
• Platelet inhibitors (aspirin, Plavix) are administered
• Fluids may be restricted initially to decrease brain edema
• Calcium channel blockers may be used within 96 hours of bleeding, continuing for 21 days to decrease recurrent bleeding risk

Nursing Interventions for Stroke

• Performing neuro checks, including Glasgow Coma Scale and Four Score Coma Scale assessments
• Assessing level of consciousness, pupillary response (PERRLA), aphasia, dysarthria, and vital signs
• Monitoring movement, weakness, strength, and signs of increased intracranial pressure
• Repositioning every two hours and performing passive range of motion exercises every four hours
• Assessing functional abilities and encouraging independence
• Providing intensive therapy and rehab, including physical, occupational, speech, and recreational therapy
• Implementing bladder training after catheter removal
• Ensuring safety with side rails and assistance with ambulation
• Implementing fall prevention strategies
• Monitoring for unilateral neglect
• Approaching patients with hemianopsia from the unaffected side
• Using communication boards, writing, or specialized computers to aid communication for patients with aphasia
• Avoiding straws for patients with dysphagia
• Elevating the head during meals
• Tilting head to the unaffected side to facilitate swallowing
• Managing agnosia, the loss of ability to recognize people or objects
• Providing feeding assistance, possibly with peripheral IV, NG tube, or G-tube as needed
• Teaching ways to prevent aspiration
• Assisting with dressing and feeding

Transient Ischemic Attack (TIA)

• Cardiovascular insufficiency with temporary episodes of neurological dysfunction lasting less than 24 hours, often resolving within three hours
• Caused by microembolisms
• Symptoms include weakness, dysphagia, numbness, paresthesia, loss of vision, ataxia, inability to speak, tinnitus, vertigo, blurred vision, diplopia, and eyelid ptosis
• TIA serves as a warning sign for an impending stroke, typically within two to five years
• Medications include aspirin Plavix, or Coumadin
• Patients with atrial fibrillation may receive Coumadin, Pradaxa, Xarelto, or Eliquis
• Often occurs with coronary artery stenosis, reducing blood flow to the brain
• Patients are neurologically normal between attacks
• Immediate medical evaluation is necessary
• Surgery may be performed for 70-99% blockage in the coronary artery
• Carotid endarterectomy
• Percutaneous transluminal angioplasty
• There is a risk of emboli release and subsequent stroke during these procedures

Trigeminal Neuralgia

• Peripheral nerve problem caused by pressure on or degeneration of the trigeminal nerve
• Typically seen in middle to late adulthood, more common in women
• Characterized by excruciating, knife-like stabbing pain to the lips, gums, cheek, forehead, and side of the nose
• Attacks are brief, lasting two to three minutes, and usually occur on one side
• Trigger points along the nerve can initiate pain with slight stimulation, such as chewing, brushing teeth, exposure to hot or cold air, washing the face, yawning, or talking
• Treated with anti-seizure medications, such as carbamazepine and phenytoin
• Can also be treated with anti-spasmodic muscle relaxers like baclofen
• Absolute alcohol injections into the peripheral branches can provide relief for weeks or months
• Other strategies include biofeedback, acupuncture, and mega vitamins
• Surgery can provide permanent relief of the pain
• Nursing care involves providing comfort measures, hygiene, nourishment, and monitoring for suicide risk

Bell's Palsy

• Inflammatory condition of the facial nerve, often due to herpes simplex, herpes zoster, Epstein-Barr, or adenovirus infections
• Viruses cause inflammation, edema, ischemia, and demyelination of the facial nerve
• Affects any age, more common between 15 and 45
• Abrupt onset with numbness, stiffness, and a drawing sensation of the face
• Unilateral weakness of facial muscles, causing inability to wrinkle forehead, close eyes, pucker lips, or retract mouth on one side
• Facial asymmetry with drooping mouth or cheek
• Loss of taste, altered chewing, decreased saliva, pain behind the ear, ringing in the ear, and hearing loss
• Electrical stimulation or warm, moist heat along the nerve can help
• Treatment includes steroids (prednisone)
• Acyclovir is used if herpes simplex is the cause
• During sleep, protect eye with an eye shield
• Massage affected areas
• Perform facial exercises, including wrinkling brow and forehead, closing eyes tightly, and puffing out cheeks, for five minutes, three times a day
• Resolution typically occurs in three to six months, up to a year
• Return of taste is the first sign of improvement
• Steroids must be tapered down if used for more than two weeks

Guillain-Barré Syndrome

• Acute inflammatory polyradiculopathy or post-infectious polyneuritis
• Causes widespread demyelination in the peripheral nervous system
• Potentially fatal
• Antibodies attack Schwan cells, leading to demyelination and impaired nerve conduction
• May occur after a respiratory or gastrointestinal viral infection
• Symptoms appear in the lower extremities first and ascend upward
• Progression can stop at any point
• Recovery occurs in reverse order, from top to bottom
• Most patients have a non-specific infection 10-14 days before onset
• Weakness, tingling, and numbness
• Symmetrical muscle weakness and lower motor neuron paralysis
• Diagnosis is made by excluding other conditions, based on signs, symptoms, and muscle weakness
• It is self-limiting
• Patients will need hospitalization
• Steroids are often used to manage these symptoms
• Plasmapheresis decreases severity and length if started within the first two weeks of Guillain-Barré
• IV Immune globulin is an alternative
• Ensure adequate hydration and renal function (BUN and creatinine levels) before administration
• Mechanical ventilation may be required
• Tracheostomy may be considered to support prolonged ventilation
• Cognition is unaffected
• Ensure emotional support and communication
• An NG tube may be needed for nourishment
• Complication prevention is the priority
• Respiratory status monitoring is essential
• Prevent contractures and decubitus ulcers with range of motion exercises and regular turning
• Vital sign monitoring and strength assessments are important

Meningitis

• Meningitis; acute infection of the meninges, causing irritation
• Bacterial (septic) meningitis requires respiratory isolation
• Aseptic (viral) meningitis is less severe
• Hyperemia in meningeal vessels and edema of brain tissue
• Inflammatory reaction with white blood cells in subarachnoid spaces
• Prevented by childhood vaccinations
• Increased risk in fall and winter months
• Kernig's sign: inability to extend legs completely without extreme pain,
• Brudzinski's sign: Flexion of hips and knees when neck is flexed shows meningeal irritation
• Sudden onset includes: severe headache, nuchal rigidity (stiff neck), irritability, malaise, restlessness, nausea, vomiting, delirium, confusion, elevated temperature, chills, elevated pulse and respirations, and photophobia (light sensitivity)
• CT scan rules out increased ICP, but lumbar puncture with CSF analysis confirms organism
• Antibiotics can not treat the viral kind
• Position is lateral recumbent (fetal position) or sitting leaning forward
• Medical management includes massive doses of IV antibiotics for bacterial
• Ampicillin, penicillin, vancomycin, piperacillin, and third-generation cephalosporins (Rocephin) are the medications of choices, because they can cross the blood-brain barrier
• IV steroids can be given to decrease ICP
• Anticonvulsants can prevent seizures, fluid restriction, and Tylenol for fever
• Respiratory isolation for 24 hours after antibiotics begin
• Dark room, decreased noise to decrease the risk of seizures
• Closely monitor repiratory status, administer oxygen, and assist with ventilation
• Elevate head of bed to 30 degrees, but leave head in neutral position
• Handle patient gently, keep fever under control
• Cooling blankets may be required
• Vaccinations needed: pneumococcal after 65, Hib vaccine prevents haemophilus influenza type B
• Meningococcal shots given between 11 and 12, with a booster at 16
• Encephalitis is an acute inflammation of the brain
• Usually caused by a virus
• Can be caused by ticks and mosquitoes
• Can be a complication of measles, mumps, and chickenpox
• Highest mortality rate if caused by herpes simplex virus

Signs and Symptoms

• Gradual onset
• Severe headache
• High fever
• Seizures due to irritability of the brain
• Change in level of consciousness
• Nausea and vomiting

Long-Term Impact

• Memory impairment
• Epilepsy
• Anomia
• Dysphagia
• Personality changes

Diagnosis

• MRI or PET scan
• Analysis of cerebral spinal fluid

Treatment

• Diuretics such as Mannitol to decrease cerebral edema
• Corticosteroids for cerebral edema
• Acyclovir or Vira-A if herpes simplex is the cause