Chapter 7 - A. Neuro-assesment Disorder

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NEURO ASSESSMENT DISORDERS

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MAJOR CLASSIFICATION OF NEUROLOGIC DISORDERS 1.CEREBRAL DISORDERS A. EPILEPSY DEFINITION Epilepsy is a common condition that causes repeated seizures. The seizures are caused by bursts of electrical activity in the brain that are not normal. Seizures may cause problems with muscle control, movement, speech, vision, or awareness. They usually don't last very long, but they can be scary. The good news is that treatment usually works to control and reduce seizures. Epilepsy is not a type of mental illness or retardation. It generally does not affect how well you think or learn. You can't catch epilepsy from someone else (like a cold), and they can't catch it from you.

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SYMPTOMS The main symptom of epilepsy is repeated seizures that happen without warning. Without treatment, seizures may continue and even become worse and more frequent over time. There are different kinds of seizures. You may have only one type of seizure. Some people have more than one type. Depending on what kind of seizure you have: Your senses may not work right. For example, you may notice strange smells or sounds. You may lose control of your muscles. You may fall down, and your body may twitch or jerk. You may stare off into space. You may faint (lose consciousness). Not everyone who has seizures has epilepsy. Sometimes seizures happen because of an injury, illness, or another problem. In these cases, the seizures stop when that problem improves or goes away.

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MEDICAL/SURGICAL MANAGEMENT The list of treatments mentioned in various sources for Epilepsy includes the following list. Always seek professional medical advice about any treatment or change in treatment plans. Anticonvulsant medications Phenytoin Primidone Methoin Clonazepam Sodium valproate Carbamazepine Brain surgery Lifestyle changes Avoid alcohol Avoid driving Slide 4 of 68

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B. SEIZURES DEFINITION Seizures are caused by abnormal electrical discharges in the brain. Symptoms may vary depending on the part of the brain that is involved, but seizures often cause unusual sensations, uncontrollable muscle spasms, and loss of consciousness. Some seizures may be the result of a medical problem. Low blood sugar, infection, a head injury, accidental poisoning, or drug overdose can cause a seizure. A seizure may also be due to a brain tumor or other health problem affecting the brain. In addition, anything that results in a sudden lack of oxygen to the brain can cause a seizure. In some cases, the cause of the seizure is never discovered.

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SYMPTOMS Sensory/thought: Black out Confusion Deafness/Sounds Electric Shock Feeling Loss of consciousness Smell Spacing out Out of body experience Visual loss or blurring

Emotional: Fear/Panic •Physical: Chewing movements Convulsion Difficulty talking Drooling Eyelid fluttering Eyes rolling up Falling down Foot stomping Hand waving Inability to move Incontinence

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Lip smacking Making sounds Shaking Staring Stiffening Swallowing Sweating Teeth clenching/grinding Tongue biting Tremors Twitching movements Breathing difficulty Heart racing

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MEDICAL/SURGICAL MANAGEMENT The first step toward getting treatment for seizures is to see a doctor! The doctor may make a diagnosis of epilepsy (the doctor might call it a seizure disorder). This step usually will involve some diagnostic tests. Then comes the treatment. There are many seizure medicines. Each one tends to work better for certain kinds of seizures than for others. The doctor chooses one based on the type of seizure, the person's age, and whether the person has any other medical conditions. The doctor then will usually discuss how to take the medicine, what side effects to look for, and when to come back for a follow-up visit.

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C. BRAIN TUMORS DEFINITION Brain tumor is a growth or tumor that develops in the tissues of the brain in adults. The tumor can be benign or malignant.

SYMPTOMS The symptoms of brain tumors depend on tumor size, type, and location. Symptoms may be caused when a tumor presses on a nerve or damages a certain area of the brain. They also may be caused when the brain swells or fluid builds up within the skull.

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These are the most common symptoms of brain tumors: Headaches (usually worse in the morning) •Nausea or vomiting •Changes in speech, vision, or hearing •Problems balancing or walking •Changes in mood, personality, or ability to concentrate •Problems with memory •Muscle jerking or twitching (seizures or convulsions) •Numbness or tingling in the arms or legs These symptoms are not sure signs of a brain tumor. Other conditions also could cause these problems. Anyone with these symptoms should see a doctor as soon as possible. Only a doctor can diagnose and treat the problem.

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MEDICAL/SURGICAL MANAGEMENT If a person has symptoms that suggest a brain tumor, the doctor may perform one or more of the following procedures: 1. Physical exam - The doctor checks general signs of health. 2. Neurologic exam - The doctor checks for alertness, muscle strength, coordination, reflexes, and response to pain. The doctor also examines the eyes to look for swelling caused by a tumor pressing on the nerve that connects the eye and brain. 3. CT scan - An x-ray machine linked to a computer takes a series of detailed pictures of the head. The patient may receive an injection of a special dye so the brain shows up clearly in the pictures. The pictures can show tumors in the brain. 4. MRI - A powerful magnet linked to a computer makes detailed pictures of areas inside the body. These pictures are viewed on a monitor and can also be printed. Sometimes a special dye is injected to help show differences in the tissues of the brain. The pictures can show a tumor or other problem in the brain. Slide 10 of 68

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D. CEREBROVASCULAR DISEASE INTRODUCTION Cerebrovascular disease is a group of brain dysfunctions related to disease of blood vessels supplying the brain. Hypertension is the most important cause that damages the blood vessel lining endothelium exposing the underlying collagen where platelets aggregate to initiate a repairing process which is not always complete and perfect. Sustained hypertension permanently changes the architecture of the blood vessels making them narrow, stiff, deformed and uneven which are more vulnerable to fluctuations of blood pressure. A fall in blood pressure during sleep can lead to marked reduction in blood flow in the narrowed blood vessels causing ischemic stroke in the morning whereas a sudden rise in blood pressure can cause tearing of the blood vessels causing intracranial hemorrhage during excitation at daytime. Primarily people who are elderly, diabetic, smoker, or have ischemic heart disease, have cerebrovascular disease. All diseases related to artery dysfunction can be classified under a disease as known as Macrovascular disease.

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SYMPTOMS A cerebrovascular accident is apparent if the victim displays the following symptoms: dizziness, nausea, vomiting, severe headaches, head pressure, and the numbness within the limbs, slurred speech, vision loss, loss of coordination and the ability to walk. Hemorrhagic strokes may require surgery to relieve the pressure within the brain. Another treatment, endovascular treatment, requires inserting a tube into the major artery.

MEDICAL/SURGICAL MANAGEMENT 1. Physical examination Pulse and blood pressure Cranial nerve examination including visual acuity and visual fields, pupils and eye movements, facial muscles and facial sensation, hearing, gag reflex and tongue Examine gait Examine upper and lower limbs noting tone, power, reflexes, coordination and sensation Listen to carotid arteries for bruits (sign of reduced blood flow to brain) Slide 12 of 68

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2. Blood tests Full blood count to detect polycythaemia, infection or inflammation Electrolytes and renal function tests Blood glucose Syphilis serology to detect possible neurosyphilis Clotting screen if appropriate (antithrombin III deficiency, protein C or S deficiency, lupus anticoagulant) Lupus erythematosus serology e.g. antinuclear antibodies, double stranded DNA, cardiolipin antibodies Blood cultures if any chance of bacterial endocarditis 3. Electrocardiogram - to detect atrial fibrillation. 4. Radiological tests CT Scan Brain MRI Brain can detect changes of stroke within a few hours Chest X-Ray Carotid Doppler Echocardiogram and Cerebral angiogram Slide 13 of 68

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E. BRAIN INFECTIONS DEFINITION Any infection of the brain is usually called encephalitis, though meningitis is closely related.

SYMPTOMS Patients with encephalitis suffer from fever, headache and photophobia with weakness and seizures also common. Less commonly, stiffness of the neck can occur with rare cases of patients also suffering from stiffness of the limbs, slowness in movement and clumsiness depending on which specific part of the brain is involved. The symptoms of encephalitis are caused by the brain's defense mechanisms activating to get rid of the infection.

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MEDICAL/SURGICAL MANAGEMENT Treatment is usually symptomatic. Reliably tested specific antiviral agents are available only for a few viral agents (e.g. acyclovir for herpes simplex virus) and are used with limited success for most infection except herpes simplex encephalitis. In patients who are very sick, supportive treatment, such as mechanical ventilation, is equally important.

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F. HEADACHES DEFINITION A headache is a pain in the head with the pain being above the eyes or the ears, behind the head (occipital), or in the back of the upper neck. Headache, like chest pain or back ache, has many causes.

SYMPTOMS The list of signs and symptoms mentioned in various sources for Headache includes the 4 symptoms listed below: Confusion Memory problems Headaches Vision problems Note that Headache symptoms usually refers to various symptoms known to a patient, but the phrase Headache signs may refer to those signs only noticable by a doctor. Slide 16 of 68

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MEDICAL/SURGICAL MANAGEMENT Not all headaches require medical attention, and many respond with simple analgesia (painkillers) such as paracetamol/acetaminophen or members of the NSAID class (such as aspirin/acetylsalicylic acid or ibuprofen). In recurrent unexplained headaches, healthcare professionals may recommend keeping a "headache diary" with entries on type of headache, associated symptoms, precipitating and aggravating factors. This may reveal specific patterns, such as an association with medication, menstruation or absenteeism or with certain foods. It was reported in March 2007 by two separate teams of researchers that stimulating the brain with implanted electrodes appears to help ease the pain of cluster headaches.

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2. DEGENERATIVE NEUROLOGIC DISORDERS A. DEMENTIA (ALZHEIMERS) DEFINITION Alzheimer's disease (AD) affects the mental abilities including memory, language, and cognition. Progressively it leads to dementia and death. AD usually arises in late middle age or the elderly but there is a rare familial subtype that occurs earlier. Because AD is so well-known, other causes of dementia or memory loss may be overlooked. Other possible diagnoses include normal aging (if very mild symptoms), emotional problems, fatigue, depression, and certain medical conditions such as thyroid disease, brain tumors, multi-infarct disease, or Huntington's disease. In its early stages, a correct diagnosis of AD can also be overlooked itself and misdiagnosed as other conditions such as depression, dementia, simple forgetfulness, or senility.

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SYMPTOMS Early symptoms Forgetfulness Loss of concentration Forgetting names Progressing symptoms Memory loss Forgetting how to do everyday tasks Thinking difficulty Difficulty speaking Difficulty reading Language deterioration Impaired visual skills Impaired spatial skills Poor judgment Confusion Disorientation

Later symptoms Indifferent attitude Anxiety Apathy Cognition disintegration Personality disintegration Suspicion Hostility Aggression Inability to function Loss of speech Difficulty swallowing Drooling Incontinence

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Depression Aggression Wandering Normal motor function - AD affects the brain but not the body

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MEDICAL/SURGICAL MANAGEMENT Neuropsychological screening tests, such as the mini-mental state examination (MMSE), are widely used to evaluate the cognitive impairments needed for diagnosis. More comprehensive test arrays are necessary for high reliability of results, particularly in the earliest stages of the disease. Neurological examination in early AD will usually provide normal results, except for obvious cognitive impairment, which may not differ from standard dementia. Further neurological examinations are crucial in the differential diagnosis of AD and other diseases. Interviews with family members are also utilised in the assessment of the disease. Caregivers can supply important information on the daily living abilities, as well as on the decrease, over time, of the person's mental function. A caregiver's viewpoint is particularly important, since a person with AD is commonly unaware of his own deficits. Many times, families also have difficulties in the detection of initial dementia symptoms and may not communicate accurate information to a physician. Supplemental testing provides extra information on some features of the disease or is used to rule out other diagnoses. Blood tests can identify other causes for dementia than AD—causes which may, in rare cases, be reversible. Psychological tests for depression are employed, since depression can either be concurrent with AD or be the cause of cognitive impairment. Slide 20 of 68

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B. PAKINSON’S DISEASE DEFINITION Parkinson's disease (also known as Parkinson disease or PD) is a degenerative disorder of the central nervous system that often impairs the sufferer's motor skills, speech, and other functions. Parkinson's disease belongs to a group of conditions called movement disorders. It is characterized by muscle rigidity, tremor, a slowing of physical movement (bradykinesia) and, in extreme cases, a loss of physical movement (akinesia). The primary symptoms are the results of decreased stimulation of the motor cortex by the basal ganglia, normally caused by the insufficient formation and action of dopamine, which is produced in the dopaminergic neurons of the brain. Secondary symptoms may include high level cognitive dysfunction and subtle language problems. PD is both chronic and progressive.

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SYMPTOMS Fixed facial expression • Lack of blinking • Tremor or trembling - various uncontrolled movements typically occuring in fingers, hands, arms, legs, jaw, and face Tremor in one little finger - a common early symptom Hand tremor Tremor in one hand Tremor in one leg Tremor in one arm Tremor in one side of the body Involuntary head nodding

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Walking symptoms Difficulty walking Stooping posture Loss of postural reflexes Rigid back Shuffling gait Postural instability Impaired balance Impaired coordination Falls Falling forwards Falling backwards

Rigidity - stiffness of the limbs and trunk • Bradykinesia - slowness of movement • Difficulty swallowing • Speech changes Difficulty talking Voice changes Softer voice Monotonous voice Stammering

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Facial symptoms Facial expression changes Fixed mask-like expression Tight facial muscles Facial muscle spasm Intellectual symptoms - usually late in the progression of symptoms; most people suffer physical symptoms without early mental changes.

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MEDICAL/SURGICAL MANAGEMENT Typically, the diagnosis is based on medical history and neurological examination conducted by interviewing and observing the patient in person using the Unified Parkinson's Disease Rating Scale. A radiotracer for SPECT scanning machines called DaTSCAN and made by General Electric is specialized for diagnosing Parkinson's disease, but it is only marketed in Europe. Due to this, the disease can be difficult to diagnose accurately, especially in its early stages. Due to symptom overlap with other diseases, only 75% of clinical diagnoses of PD are confirmed to be idiopathic PD at autopsy. Early signs and symptoms of PD may sometimes be dismissed as the effects of normal aging. The physician may need to observe the person for some time until it is apparent that the symptoms are consistently present. Usually doctors look for shuffling of feet and lack of swing in the arms. Doctors may sometimes request brain scans or laboratory tests in order to rule out other diseases. However, CT and MRI brain scans of people with PD usually appear normal.

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C. CREUTZFELDT-JAKOB DISEASE DEFINITION Creutzfeldt-Jakob disease (CJD) is the dementing form of the human prion diseases (also known as spongiform transmissible encephalopathies or infectious amyloidoses). A prion is an unconventional, transmissible agent (not a virus or a bacterium). Prions are special proteins that can be transmitted from animal to animal to cause a group of degenerative diseases of the nervous system. These diseases can be manifest as sporadic, infectious, or inherited disorders. CJD is characterized by forgetfulness and nervousness; jerky, trembling hand movements; unsteady gait; myoclonus; chronic dementia; severe balance disturbance; and muscular rigidity. Patients can have characteristic brain electrical changes referred to as periodic electroencephalogram (EEG) complexes. Brain biopsy (pathology under the microscope) includes typical tissue changes referred to as status spongiosus, diffuse nerve cell degeneration, and glial proliferation. There is no known treatment or cure for CJD. Bovine spongiform encephalopathy (BSE) represents another, similar disease of cattle. Slide 26 of 68

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SYMPTOMS The first symptom of CJD is rapidly progressive dementia, leading to memory loss, personality changes and hallucinations. This is accompanied by physical problems such as speech impairment, jerky movements (myoclonus), balance and coordination dysfunction (ataxia), changes in gait, rigid posture, and seizures. The duration of the disease varies greatly, but sporadic (non-inherited) CJD can be fatal within months or even weeks (Johnson, 1998). In some people, the symptoms can continue for years. In most patients, these symptoms are followed by involuntary movements and the appearance of a typical diagnostic electroencephalogram tracing. The symptoms of CJD are caused by the progressive death of the brain's nerve cells, which is associated with the build-up of abnormal prion proteins. When brain tissue from a CJD patient is examined under a microscope, many tiny holes can be seen where whole areas of nerve cells have died. The word 'spongiform' in 'transmissible spongiform encephalopathies' refers to the 'spongy' appearance of the brain tissue.

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MEDICAL/SURGICAL MANAGEMENT There is currently no cure for CJD, a fatal disease, and the search for viable treatments continues. An experimental treatment was given to a Northern Irish teenager, Jonathan Simms, beginning in January 2003. The medication, called pentosan polysulphate (PPS) and used to treat interstitial cystitis, is infused into the patient's lateral ventricle within the brain. PPS does not seem to stop the disease from progressing, and both brain function and tissue continue to be lost. However, the treatment is alleged to slow the progression of the otherwise untreatable disease, and may have contributed to the longer than expected survival of the seven patients that were studied. The CJD Therapy Advisory Group to the UK Health Departments advises that data are not sufficient to support claims that pentosan polysulphate is an effective treatment and suggests that further research in animal models is appropriate. A 2007 review of the treatment of 26 patients with PPS finds no proof of efficacy because of the lack of accepted objective criteria.

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D. HUNTINGTON’S DISEASE DEFINITION Huntington's disease, also called Huntington's chorea, chorea major, or HD, is a genetic neurological disorder characterized after onset by uncoordinated, jerky body movements called chorea and a decline in some mental abilities, which can lead to affected aspects of behavior. As the disorder progresses, these symptoms can cause complications that significantly reduce life expectancy.

SYMPTOMS Physical symptoms are usually the first to cause problems and to be noticed, but at this point they are usually accompanied by cognitive and psychiatric ones that are often not recognized. Almost everyone with Huntington's disease eventually exhibits all physical symptoms, but cognitive and psychiatric symptoms vary significantly between individuals.

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MEDICAL/SURGICAL MANAGEMENT The list of treatments mentioned in various sources for Huntington's Disease includes the following list. Prenatal chorionic villus sampling Medications Anti-chorea medications Anti-agitation medications Physiotherapy Exercise Always seek professional medical advice about any treatment or change in treatment plans.

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E. MULTIPLE SCLEROSIS DEFINITION Multiple sclerosis (MS) is a disease in which the nerves of the central nervous system (brain and spinal cord) degenerate. Myelin, which provides a covering or insulation for nerves, improves the conduction of impulses along the nerves and also is important for maintaining the health of the nerves. In multiple sclerosis, inflammation causes the myelin to eventually disappear. Consequently, the electrical impulses that travel along the nerves decelerate, that is, become slower. In addition, the nerves themselves are damaged. As more and more nerves are affected, a patient experiences a progressive interference with functions that are controlled by the nervous system such as vision, speech, walking, writing, and memory.

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SYMPTOMS Symptoms of MS usually appear in episodic acute periods of worsening (relapses, exacerbations, bouts or attacks), in a gradually-progressive deterioration of neurologic function, or in a combination of both. The most common presentation of MS is the clinically isolated syndrome (CIS). In CIS, a patient has an attack suggestive of demyelination, but does not fulfill the criteria for multiple sclerosis.Only 30 to 70% of persons experiencing CIS later develop MS. The disease usually presents with sensorial (46% of cases), visual (33%), cerebellar (30%) and motor (26%) symptoms. Many rare initial symptoms have also been reported, including aphasia, psychosis and epilepsy. Patients first seeking medical attention commonly present with multiple symptoms. The initial signs and symptoms of MS are often transient, mild, and self-limited. These signs and symptoms often do not prompt a person to seek medical attention and are sometimes identified only retrospectively once the diagnosis of MS has been made. Cases of MS are sometimes incidentally identified during neurological examinations performed for other causes. Such cases are referred to as subclinical MS.

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The person with MS can suffer almost any neurological symptom or sign, including changes in sensation (hypoesthesias and paraesthesias), muscle weakness, muscle spasms, or difficulty in moving; difficulties with coordination and balance (ataxia); problems in speech (dysarthria) or swallowing (dysphagia), visual problems (nystagmus, optic neuritis, or diplopia), fatigue, acute or chronic pain, and bladder and bowel difficulties. Cognitive impairment of varying degrees and emotional symptoms of depression or unstable mood are also common. The main clinical measure of disability progression and symptom severity is the Expanded Disability Status Scale or EDSS.

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MEDICAL/SURGICAL MANAGEMENT There are many issues for the patient and physician to consider in treating multiple sclerosis. Goals may include reducing the number of attacks, improving recovery from attacks, and attempting to slow further progression of the disease (treatment with disease-modifying drugs). An additional goal is relief from complications due to the loss of function of affected organs (treatment with drugs aimed at specific symptoms). Most neurologists will consider treatment with disease-modifying drugs once the diagnosis of multiple sclerosis is established. Many will begin treatment at the time of the first multiple sclerosis attack, since clinical trials have suggested that patients in whom treatment is delayed may not benefit as much as patients who are treated early. Finally, utilizing support groups or counseling may be helpful for patients and their families whose lives may directly be affected by multiple sclerosis.

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Once goals have been set, initial therapy may include medications to manage attacks, symptoms, or both. An understanding of the potential side effects of drugs is critical for the patient because sometimes side effects alone deter patients from drug therapy. Patients may choose to avoid drugs altogether or choose an alternative drug that may offer relief with fewer side effects. A continuous dialogue between the patient and physician about the medications is important in determining the needs for treatment. Drugs known to affect the immune system have become the primary focus for managing multiple sclerosis. Initially, corticosteroids, such as prednisone (Deltasone, Liquid Pred, Deltasone, Orasone, Prednicen-M) or methylprednisolone (Medrol, Depo-Medrol), were widely used. However, since their effect on the immune system is non-specific and their use may cause numerous side effects, corticosteroids now tend to be used to manage only sudden, severe multiple sclerosis attacks.

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F. GUILLAIN BARRE SYNDROME DEFINITION Guillain-Barré syndrome (often misspelled Guillain-Barre) is a disorder in which the body's immune system attacks part of the peripheral nervous system. The first symptoms of this disorder include varying degrees of weakness or tingling sensations in the legs. In many instances the weakness and abnormal sensations spread to the arms and upper body. These symptoms can increase in intensity until certain muscles cannot be used at all and, when severe, the patient is almost totally paralyzed. In these cases the disorder is life threatening potentially interfering with breathing and, at times, with blood pressure or heart rate - and is considered a medical emergency. Such a patient is often put on a respirator to assist with breathing and is watched closely for problems such as an abnormal heart beat, infections, blood clots, and high or low blood pressure. Most patients, however, recover from even the most severe cases of Guillain-Barré syndrome, although some continue to have a certain degree of weakness.

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SYMPTOMS Symptoms of Guillain-Barré Syndrome include weakness, typically beginning in the legs and progressing upward. The weakness is accompanied by decreased feeling (paresthesia). Reflexes are lost, for example, the hammer to the front of the knee will not induce a kick. In severe cases breathing can be affected enough to require a ventilator and rarely the heart can be affected. The maximal degree of weakness usually occurs within the first 2-3 weeks.

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MEDICAL/SURGICAL MANAGEMENT There is no known cure for Guillain-Barré syndrome. However, there are therapies that lessen the severity of the illness and accelerate the recovery in most patients. There are also a number of ways to treat the complications of the disease. Currently, plasma exchange (sometimes called plasmapheresis) and highdose immunoglobulin therapy are used. Both of them are equally effective, but immunoglobulin is easier to administer. Plasma exchange is a method by which whole blood is removed from the body and processed so that the red and white blood cells are separated from the plasma, or liquid portion of the blood. The blood cells are then returned to the patient without the plasma, which the body quickly replaces. Scientists still don't know exactly why plasma exchange works, but the technique seems to reduce the severity and duration of the Guillain-Barré episode. This may be because the plasma portion of the blood contains elements of the immune system that may be toxic to the myelin.

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In high-dose immunoglobulin therapy, doctors give intravenous injections of the proteins that, in small quantities, the immune system uses naturally to attack invading organisms. Investigators have found that giving high doses of these immunoglobulins, derived from a pool of thousands of normal donors, to Guillain-Barré patients can lessen the immune attack on the nervous system. Investigators don't know why or how this works, although several hypotheses have been proposed. The use of steroid hormones has also been tried as a way to reduce the severity of Guillain-Barré, but controlled clinical trials have demonstrated that this treatment not only is not effective but may even have a deleterious effect on the disease. The most critical part of the treatment for this syndrome consists of keeping the patient's body functioning during recovery of the nervous system. This can sometimes require placing the patient on a respirator, a heart monitor, or other machines that assist body function. The need for this sophisticated machinery is one reason why Guillain-Barré syndrome patients are usually treated in hospitals, often in an intensive care ward. In the hospital, doctors can also look for and treat the many problems that can afflict any paralyzed patient complications such as pneumonia or bed sores. Slide 39 of 68

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G. MYASTHENIA GRAVIS DEFINITION Myasthenia gravis is a chronic autoimmune neuromuscular disease characterized by varying degrees of weakness of the skeletal (voluntary) muscles of the body. The name myasthenia gravis, which is Latin and Greek in origin, literally means "grave muscle weakness." With current therapies, however, most cases of myasthenia gravis are not as "grave" as the name implies. In fact, for the majority of individuals with myasthenia gravis, life expectancy is not lessened by the disorder.

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SYMPTOMS Although myasthenia gravis may affect any voluntary muscle, muscles that control eye and eyelid movement, facial expression, and swallowing are most frequently affected. The onset of the disorder may be sudden. Symptoms often are not immediately recognized as myasthenia gravis. In most cases, the first noticeable symptom is weakness of the eye muscles. In others, difficulty in swallowing and slurred speech may be the first signs. The degree of muscle weakness involved in myasthenia gravis varies greatly among patients, ranging from a localized form, limited to eye muscles (ocular myasthenia), to a severe or generalized form in which many muscles sometimes including those that control breathing - are affected. Symptoms, which vary in type and severity, may include a drooping of one or both eyelids (ptosis), blurred or double vision (diplopia) due to weakness of the muscles that control eye movements, unstable or waddling gait, weakness in arms, hands, fingers, legs, and neck, a change in facial expression, difficulty in swallowing and shortness of breath, and impaired speech (dysarthria).

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MEDICAL/SURGICAL MANAGEMENT Immunosuppressive drugs such as prednisone, cyclosporine, and azathioprine may also be used. These medications improve muscle strength by suppressing the production of abnormal antibodies. They must be used with careful medical followup because they may cause major side effects. Thymectomy, the surgical removal of the thymus gland (which often is abnormal in myasthenia gravis patients), reduces symptoms in more than 70 percent of patients without thymoma and may cure some individuals, possibly by re-balancing the immune system. Other therapies used to treat myasthenia gravis include plasmapheresis, a procedure in which abnormal antibodies are removed from the blood, and high-dose intravenous immune globulin, which temporarily modifies the immune system and provides the body with normal antibodies from donated blood. These therapies may be used to help individuals during especially difficult periods of weakness. A neurologist will determine which treatment option is best for each individual depending on the severity of the weakness, which muscles are affected, and the individual's age and other associated medical problems.

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H. AMYOTROPHIC LATERAL SCLEROSIS DEFINITION Amyotrophic lateral sclerosis (also called ALS or "Lou Gehrig's disease") is a classic motor neuron disease. Motor neuron diseases are progressive chronic diseases of the nerves that come from the spinal cord responsible for supplying electrical stimulation to the muscles. This stimulation is necessary for the movement of body parts.

SYMPTOMS All forms of ALS cause progressive muscle weakness and wasting. Spontaneous tiny local areas of muscle twitching, called fasciculations, are characteristic in most patients. These may be sensed by the patient as muscle cramping. Lower extremity muscle wasting (atrophy) and weakness generally follows wasting of the arms, hands, and shoulders. Spastic muscles can be present. Other muscles diseases are considered in the evaluation of patients. Blood testing and muscle electrical testing with electromyography (EMG) and nerve conduction velocities (NCV) can be helpful for a diagnosis. Slide 43 of 68

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MEDICAL/SURGICAL MANAGEMENT Treatment measures in patients with motor neuron diseases are largely supportive, treating the complications, such as infections and general health. The treatment of ALS is also directed toward suppressing the immune inflammation felt to play a role in the degeneration of the nervous system of these patients. While there is no cure for ALS or a proven therapy that will prevent or reverse the course of the disorder, the U.S. Food and Drug Administration (FDA) has approved riluzole (RILUTEK), the first drug that has been shown to prolong the survival of ALS patients. Patients may also receive supportive treatments that address the symptoms of the disease.

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3. PERIPHERAL NERVOUS SYSTEM DISORDERS A. LOWER BACK PAIN DEFINITION Low back pain (sometimes referred to generally as lumbago) is a common musculoskeletal disorder causing back pain in the lumbar vertebrae. It can be either acute, subacute or chronic in its clinical presentation. Typically, the symptoms of low back pain do show significant improvement within two to three months from its onset. In a significant number of individuals, low back pain tends to be recurrent in nature with a waxing and waning quality to it. In a small proportion of sufferers this condition can become chronic. Population studies show that back pain affects most adults at some stage in their life and accounts for more sick leave and disability than any other single medical condition.

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CAUSES Common causes of low back pain include lumbar strain, nerve irritation, lumbar radiculopathy, bony encroachment, and conditions of the bone and joints.

MEDICAL/SURGICAL MANAGEMENT The McKenzie Method or Mechanical Diagnosis and Treatment (MDT) of the pain. This is based on a set of repeated movements or statically held postures to determine if symptoms can be changed (better or worse). Treatment is then based on avoiding postures or movements that aggravate symptoms, as well as performing or adhering to postures to assist in symptom reduction. Robin McKenzie, Founder of the McKenzie Institute, originally based his premise for treatment on a intervertebral disc model. Hence, he believes this type of assessment and treatment is beneficial for degenerative disc disease including disc herniation. The McKenzie Institute, along with Robin, expanded it's scope of treatment to any mechanical origin of spine pain. Health care professionals such as Doctors, Physical Therapists and Chiropractors can follow a postgraduate study in the McKenzie Method.

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Analgesics (pain medications), such as NSAIDs or acetaminophen. •Arch support •The Alexander Technique was shown in a UK clinical trial to have long term benefits for patients with chronic back pain. •Spinal manipulation for acute or chronic pain. A clinical prediction rule can guide who is most likely to respond to manipulation. •Muscle relaxants for acute or chronic pain. •Antidepressants for chronic low back pain. •Stay physically active. •Exercise for chronic pain. •Intensive multidisciplinary treatment programs may help subacute or chronic low back pain. •Behavioral therapy •Acupuncture may help chronic pain; however, a more recent randomized controlled trial suggested insignificant difference between real and sham acupuncture Slide 47 of 68

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B. TRIGEMINAL NEURALGIA DEFINITION Trigeminal neuralgia (TN), or Tic Doloureux, (also known as prosopalgia) is a neuropathic disorder of the trigeminal nerve that causes episodes of intense pain in the eyes, lips, nose, scalp, forehead, and jaw. It is estimated that 1 in 15,000 people suffer from trigeminal neuralgia, although those numbers may be significantly higher due to frequent misdiagnosis. TN usually develops after the age of 50, more commonly in females, although there have been cases with patients being as young as three years of age.

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SYMPTOMS The episodes of pain may occur paroxysmally. To describe the pain sensation, patients describe a trigger area on the face, so sensitive that touching or even air currents can trigger an episode of pain. It affects lifestyle as it can be triggered by common activities in a patient's daily life, such as eating, talking, shaving and toothbrushing. The attacks are said to feel like stabbing electric shocks, burning, pressing or shooting pain that becomes intractable. Individual attacks affect one side of the face at a time, last several seconds, hours or longer, and repeat up to hundreds of times throughout the day. The pain also tends to occur in cycles with complete remissions lasting months or even years. 10-12% of cases are bilateral, or occurring on both sides. This normally indicates problems with both trigeminal nerves since one serves strictly the left side of the face and the other serves the right side. Pain attacks typically worsen in frequency or severity over time. A great deal of patients develop the pain in one branch, then over years the pain will travel through the other nerve branches.

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MEDICAL/SURGICAL MANAGEMENT There is no cure for trigeminal neuralgia, but most people find relief from medication, from one of the five surgical options or sometimes from one of the many complementary or alternative therapies. Atypical trigeminal neuralgia, which involves a more constant and burning pain, is more difficult to treat, both with medications and surgery. Surgery may result in varying degrees of numbness to the patient and lead occasionally to "anesthesia dolorosa," which is numbness with intense pain. However, some people do find dramatic relief with minimal side effects from the various surgeries that are now available. There are some things that a patient can do to minimize the frequency and intensity of TN attacks: Apply ice packs or any readily available source of cold to the area of pain. Cold often numbs the area and will reduce the pain. •Warming packs, wrapped in a towel to protect one's skin, can also provide relief as they stimulate blood flow to the area they are placed upon.

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Get adequate rest in normal rest cycles. •Manage your stress well and keep stress levels low. When you feel a TN attack coming on, try to relax immediately. •Practice healthy living principles such as diet and exercise. •Avoid foods that may act as nerve stimulants, such as coffee, tea, and foods that are high in sugar. •Maintain adequate hydration and electrolyte levels at all time.

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C. BELL’S PALSY DEFINITION Bell's palsy is a paralysis of the facial nerve resulting in inability to control facial muscles on the affected side. Several conditions can cause a facial paralysis, e.g., brain tumor, stroke, and Lyme disease. However, if no specific cause can be identified, the condition is known as Bell's Palsy. Named after Scottish anatomist Charles Bell, who first described it, Bell's palsy is the most common acute mononeuropathy (disease involving only one nerve), and is the most common cause of acute facial nerve paralysis. Bell's palsy is defined as an idiopathic unilateral facial nerve paralysis, usually self-limiting. The trademark is rapid onset of partial or complete palsy, usually in a single day.

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SYMPTOMS Although defined as a mononeuritis (involving only one nerve), patients diagnosed with Bell’s palsy may have "myriad neurological symptoms" including "facial tingling, moderate or severe headache/neck pain, memory problems, balance problems, ipsilateral limb paresthesias, ipsilateral limb weakness, and a sense of clumsiness" that are "unexplained by facial nerve dysfunction". This is yet an enigmatic facet of this condition.

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MEDICAL/SURGICAL MANAGEMENT Treatment of Bell's palsy is a matter of controversy. Two Cochrane reviews from 2004 underlined the need for larger, properly designed clinical trials to evaluate antiviral drugs or corticosteroids for Bell's palsy. The effect of treatment is difficult to evaluate experimentally because spontaneous recovery (without any treatment) is common. In patients presenting with incomplete facial palsy, where the prognosis for recovery is very good, treatment may be unnecessary. Patients presenting with complete paralysis, marked by an inability to close the eyes and mouth on the involved side, are usually treated. Early treatment (within 3 days after the onset) seems to be necessary for therapy to be effective. Prednisolone, a corticosteroid, if used early in treatment of Bell's palsy, significantly improves the chances of complete recovery at 3 and 9 months when compared to treatment with the anti-viral drug acyclovir or no treatment at all.

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D. VASCULAR SPINAL CORD LESIONS E. DISORDERS OF THE PERIPHERAL NERVES

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4. NEUROLOGIC TRAUMA A. SPINAL CORD INJURY DEFINITION Spinal cord injury causes myelopathy or damage to white matter or myelinated fiber tracts that carry sensation and motor signals to and from the brain. It also damages gray matter in the central part of the spinal, causing segmental losses of interneurons and motorneurons. Spinal cord injury can occur from many causes, including: Trauma such as automobile crashes, falls, gunshots, diving accidents, war injuries, etc. • Tumor such as meningiomas, ependymomas, astrocytomas, and metastatic cancer. • Ischemia resulting from occlusion of spinal blood vessels, including dissecting aortic aneurysms, emboli, arteriosclerosis.

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Developmental disorders, such as spina bifida, meningomyolcoele, and other. •Neurodegenerative diseases, spinocerebellar ataxia, etc.

such

as

Friedreich's

ataxia,

•Demyelinative diseases, such as Multiple Sclerosis. Transverse myelitis, resulting inflammation, or other causes.

from

spinal

cord

stroke,

Vascular malformations, such as arteriovenous malformation (AVM), dural arteriovenous fistula (AVF), spinal hemangioma, cavernous angioma and aneurysm.

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SYMPTOMS Paralysis Loss of sensation Loss of reflex function Loss of autonomic activity Breathing difficulty Loss of bowel control • Pain • Sensitivity to stimuli • Muscle spasms • Sexual dysfunction • Loss of bladder control

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MEDICAL/SURGICAL MANAGEMENT Treatment for acute traumatic spinal cord injuries have consisted of giving a high dose methylprednisolone if the injury occurred within 8 hours. The recommendation is primarily based on the National Acute Spinal Cord Injury Studies (NASCIS) II and III. Some of the claims of the studies have been challenged as being from faulty interpretation of the data.

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B. HEAD INJURY DEFINITION Head injury is a trauma to the head, that may or may not include injury to the brain (see also brain injury). However, 'brain injury' and 'head injury' are often used interchangeably in the medical literature. The incidence (number of new cases) of head injury is 300 per 100,000 per year (0.3% of the population), with a mortality of 25 per 100,000 in North America and 9 per 100,000 in Britain. Head trauma is a common cause of childhood hospitalization.

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SYMPTOMS Leaking cerebrospinal fluid (a clear fluid drainage from nose, mouth or ear) may be and is strongly indicative of basilar skull fracture and the tearing of sheaths surrounding the brain, which can lead to secondary brain infection. •Visible deformity or depression in the head or face; for example a sunken eye can indicate a maxillar fracture •An eye that cannot move or is deviated to one side can indicate that a broken facial bone is pinching a nerve that innervates eye muscles •Wounds or bruises on the scalp or face. •Basilar skull fractures, those that occur at the base of the skull, are associated with Battle's sign, a subcutaneous bleed over the mastoid, hemotympanum, and cerebrospinal fluid rhinorrhea and otorrhea.

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MEDICAL/SURGICAL MANAGEMENT Unfortunately, once the brain has been damaged by trauma, there is no quick fix. However, there are some steps that can be taken to prevent secondary damage. If left untreated many patients with head injury will rapidly develop complications which may lead to death or permanent disability. Prompt medical treatment may prevent the worsening of symptoms and lead to a better outcome. Medical treatment should begin at the scene of the trauma. Paramedics will generally immobilize the patient to ensure no further damage to the spine or nervous system, insert an airway to ensure uninterrupted breathing and perform endotracheal intubation if indicated. One or more IVs will be inserted to maintain perfusion status.

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In some cases medications may be administered to sedate or paralyze the patient to prevent additional movement which may worsen the brain injury. The patient should be delivered promptly to a hospital with neurosurgical capabilities. The management of brain injury requires the involvement of subspecialists who are generally available only at larger hospitals. Primary treatment involves controlling elevated intracranial pressure. This can include sedation, paralytics, cerebrospinal fluid diversion. Second line alternatives include decompressive craniectomy (Jagannathan et al. found a net 65% favorable outcomes rate in pediatric patients), barbiturate coma, hypertonic saline and hypothermia. Although all of these methods have potential benefits, there has been no randomized study that has shown unequivocal benefit.

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CEREBRAL VASCULAR DISEASE DEFINITION It is an umbrella term that refers to any functional abnormality of the CNS that occurs when the normal blood supply to the brain is disrupted.

MODIFIABLE RISK FACTORS INCLUDES hypertension cardiovascular disease High cholesterol obesity elevated hematocrit diabetes mellitus Oral contraceptive use smoking Drug abuse excessive alcohol consumption

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TWO MAJOR CATEGORIES • Ischemic - vascular occlusion and significant hypoperfusion occur • Hemorrhagic - there is extravasion of blood in the brain.

CLASSIFIED USING THE TIME COURSE IN THE FOLLOWING MANNER • Transient ischemic attack (TIA) - temporary episode of neurologic dysfunction manifested by a sudden loss of motor, sensory or visual function. • Reversible ischemic neurologic deficit - signs and symptoms are consistent with but more pronounced than a TIA and last more then 24 hours. • Stroke in evolution - worsening of neurologic signs and symptoms over several minutes or hours. • Complete stroke - stabilization of the neurologic signs and symptoms.

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CLINICAL FINDING OF CVD 1. Subjective: syncope Headache changes in level of consciousness transient paresthesias (with TIAs) mood swings 2. Objective: Convulsions Hemiplegia aphasia 3. Expressive (motor or broca’s)aphasia 4. Receptive (sensory or wernike’s)aphasia

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5. Global aphasia CSF is bloody if cerebral or subarachnoid hemorrhage is present Abnormal EEG, CT scan ,MRI Cerebral angiography may reveal vascular abnormalities such as aneurysms, narrowing or occlusions. Signs of increased intracranial pressure Dysphagia Sensory changes:hemianopia Alterations in reflexes Altered bladder and bowel function

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SURGICAL INTERVENTIONS •

To relieved pressure control bleeding if hemorrhage is present.

•

Carotid endarterectomy to improve cerebral blood flow when carotid arteries are narrowed by arteriosclerotic

THERAPUETIC INTERVENTIONS FOR CVD: •

Complete bed rest with sedation as needed.

•

maintenance of oxygenation by oxygen therapy or mechanical ventilation

•

Maintenance of nutrition by parenteral route or nasogastric feedings if the client is unable to swallow.

•

Anticoagulant therapy if thrombus or embolus is present; anti platelet therapy.

•

Antihypertensives and anticonvulsants if indicated.

•

Glucocorticoids may be used to reduce cerebral edema and intracranial pressure. Slide 68 of 68