Neurological Disorders PDF

Melody Hope Gallamore 16 Neurological Disorders C H A P T E R KEY POINTS A growi...

Melody Hope Gallamore 16 Neurological Disorders C H A P T E R KEY POINTS A growing body of evidence supports that symptoms associated with severe stroke and chronic neurological disorders are amenable to palliative treatment and that quality of life is increased from multidisciplinary approaches to care. Common problems faced by patients with these disorders include impairments in cogni- tion, communication, sleep, swallowing, breathing, and mobility, as well as pain, fatigue, and depression. To improve treatment choices and end-of-life (EOL) decision making, research is needed to disentangle some difficulties with prognostic criteria for receiving hospice benefits and the accuracy of newer technologies in determining irreversible brain death. CASE STUDY Mr. M.J. is a 79-year-old male who lives in an assisted living facility. He was found on the bathroom floor and taken to the hospital. Physicians discovered that he had a left middle cerebral artery (MCA) stroke. He has lost movement to his right upper and lower extremity. He has decreased sensation in his right upper extremity and slightly more sensation in his right lower extremity. He is exhibiting the symptoms of expressive aphasia and difficulty swallowing. His medical team is contemplating sending him to a long-term care facility in a few days. Currently, he is resistant to physical and occupational therapy. He yells when his right extremities are moved, as if in pain. He does not seem to have a desire to interact with nurses or support staff. What methods could the interdisciplinary team employ to attempt to enhance Mr. M.J.’s overall quality of life? What components should be included in Mr. M.J.’s assessment? What interventions would you recommend to treat Mr. M.J.? T his chapter focuses on management of people with stroke, chronic neurological disorders (CNDs), coma, and brain death. Stroke and CNDs, while unique and from the event, people with CNDs often exhibit unre- sponsiveness or only slight or temporary responsiveness to curative treatments. Symptoms associated with severe individualized in many respects, share a cluster of common stroke and CNDs are amenable to palliative treatment, symptoms and treatment needs. While some people who and a growing body of evidence supports that quality of suffer a stroke recover completely or nearly completely life (QOL) is increased from interdisciplinary palliative Copyright Springer Publishing Company. All Rights Reserved. From: Palliative Care Nursing: Quality Care to the End of Life, Fifth Edition DOI: 10.1891/9780826127198.0016 Matzo_27129_PTR_16_373-398_05-24-18.indd 373 5/25/18 2:55 PM  374 III. PHYSICAL HEALTH: LIFE-THREATENING DISEASES care (PC) approaches to symptom management (Bede with stroke will survive the initial illness. As a result, et al., 2011; Blackhall, 2012; Blacquiere, Gubitz, D ­ upere, there are an estimated 6.8 million stroke survivors in the McLeod, & Phillips, 2009; Burton & Payne, 2012; United States with a projection of an additional 4 million Campbell, Jones, & Merrills, 2010; Higginson et al., by the year 2030 (Go et al., 2013). Stroke accounts for 2009; Kuhn & Forrest, 2012; Lökk, 2011; Martin & more than 130,000 deaths in the United States each year, Sabbagh, 2011; Miller et al., 2009; Miyasaki et al., 2012). which is 1 in every 20 deaths (AHA, 2017). This chapter presents a description of common There are two types of stroke: ischemic and hemor- symptoms experienced from stroke and CNDs, includ- rhagic. Each type of stroke results in the brain being ing Alzheimer’s disease, Parkinson’s disease, multiple deprived of oxygen-rich blood leading to tissue hypoxia sclerosis (MS), and amyotrophic lateral sclerosis (ALS). or death (C. Davis & Lockhart, 2016). An ischemic Comorbid conditions that frequently ­accompany the stroke results in a disruption of blood to a portion of the latter stages of these illnesses are described as well as brain due to an occlusion of the cerebral artery by blood interventions aimed to provide symptom management. clots (emboli) or plaque and fatty deposits (thrombi). Due to the unique assessment and management issues The majority of occlusions occur from the formation of that accompany coma and brain death, these problems a thrombus. In a thrombic event, atherosclerotic blood and their management are presented in separate sections vessels cause complete or partial blockage of blood flow of the chapter. Issues unique to pediatric coma and brain to a local area in the brain. In an embolic event, a clot death are also discussed. All disorders discussed in this forms elsewhere in the body, such as in the heart; breaks chapter except MS are more likely to occur in older off; and travels through the arterial system and lodges adulthood; thus, gerontological issues are incorporated in a cerebral vessel, blocking blood flow. Atheroscle- throughout the chapter. rotic plaques tend to occur at the arterial bifurcations. Common sites for plaque formation include the internal carotid and vertebral arteries, and the junctions of the PREVALENCE, DISEASE TRAJECTORY, basilar and vertebral arteries (Book, 2009). AND PATHOGENESIS Hemorrhagic stroke occurs when a weakened blood vessel bursts, leaking blood into the brain. A stroke can Stroke cause a sudden onset of a focal or global neurological deficit that lasts longer than 24 hours and is caused In the United States, stroke is the fifth leading cause of by disrupted cerebral vascular circulation (Veerbeek death and the leading cause of severe long-term disability et al., 2014). Ruptured intracerebral vessels occur as (C. Davis & Lockhart, 2016), with someone having a a result of hypertension, aneurysm, trauma, erosion of stroke every 40 seconds and someone dying of a stroke, vessels by tumors, arteriovenous malformations, blood on average, every 4 minutes (American Heart ­Association coagulation disorders, vasculitis, or drugs. The bleed- [AHA], 2017). ing that occurs within the brain tissue causes increased It is estimated that each year 795,000 people will pressure within the skull, resulting in brain cell death. ­experience either a new stroke (77%) or a recurrent stroke Hemorrhagic stroke can progress rapidly, resulting in (23%). Eighty-seven percent of strokes are i­ schemic, 10% coma and frequently death (Book, 2009). are hemorrhagic, and 3% are a subarachnoid hemor- Signs of impairment may be perceptual, motor, cog- rhage (AHA, 2017). The prevalence of stroke is higher nitive, or speech related. Risk factors include hyperten- in older adults, African Americans, American Indians/ sion, disorders of heart rhythm, high blood cholesterol Alaska N ­ atives, persons with lower levels of education, and other lipids, diabetes mellitus, physical inactivity, and persons living in the southeastern United States (Go family history and genetics, chronic kidney disease, and et al., 2013). Racial and ethnic disparities in stroke care smoking (AHA, 2017). The effects of an acute stroke continue to be a major challenge for healthcare providers, are dependent on the site and the extent of the brain with the burden of stroke remaining consistently higher damaged. Some of the effects include paralysis, cogni- among ethnic minority groups (Qian et al., 2013). Seventeen tive deficits, speech problems, emotional difficulties, percent of all strokes occur in people who are older than problems with activities of daily living (ADLs), and 85 years (Go et al., 2013). The incidence of stroke is pain (National Institutes of Health, 2013). Recovery higher in men than in women between the ages of 45 and after stroke is complex and varies in terms of outcomes. 84 years. However, this changes after the age of 85 years The greater the initial damage, the longer and more dif- with women having a higher incidence of strokes than ficult the recovery and residual disability. ­Neurological men (Go et al., 2013). While stroke is most common in function begins to improve within a few days after older adults, it does occur in teenagers, children, infants, the onset of a stroke, with the greatest gain occurring and unborn babies. The overall incidence of stroke in within the first 4 to 5 weeks (Kreisel, Hennerici, & children 15 years of age and younger is 6.4/100,000. Bäzner, 2007). Neurological and functional gains can In contrast to adults, children have as many ischemic continue over 3 to 6 months; however, they will do so as hemorrhagic strokes (Go et al., 2013). Most people more slowly, “plateauing” (Langhorne, Bernhardt, & Matzo_27129_PTR_16_373-398_05-24-18.indd 374 5/25/18 2:55 PM  16. Neurological Disorders 375 Kwakkel, 2011). The National Heart, Lung, and Blood clumping by the beta-amyloid protein in the brain is Institute’s Framingham Heart Study reported that among thought to be responsible for blocking cell-to-cell signal- stroke survivors 65 years of age or older at 6 months ing at synapses and triggering inflammation leading to after discharge, 50% had some hemiparesis, 35% had neuronal dysfunction and death. Neurofibrillary tangles depressive symptoms, 30% were unable to walk without form an abnormal tau protein conglomerate in a helical assistance, 26% were dependent for ADLs, 26% were fashion inside of abnormal neurons. The neurofibrillary institutionalized in a nursing home, and 19% were apha- tangle development causes disruption inside the neuron sic (Go et al., 2013). The percentage of people dead at and later neuronal death (Querfurth & LaFerla, 2010). 1 year after stroke ranges from 14% to 19% for 45- to Neuritic plaques and neurofibrillary tangles are found in 64-year-olds and 23% to 28% for those older than 65 the hippocampus and other areas of the cerebral cortex. years of age. The percentage of people who die increases The hippocampus controls information processing, acqui- at 5 years, with a range from 26% to 41% for 45- to sition of new memories, and retrieval of old memories. 64-year-olds and 50% to 57% for those older than 65 In contrast, the cerebral cortex is involved in thinking years of age (AHA, 2017). and decision making (Book, 2009). As a result, people with Alzheimer’s disease experience progressive memory loss, loss of executive function, language difficulties, and Chronic Neurological Disorders psychiatric and behavioral disturbances, and eventually the disease affects the area of the brain that enables Alzheimer’s Disease. Alzheimer’s disease is the sixth the person to carry out basic bodily functions such as leading cause of death in the United States (Alzheimer’s walking or swallowing (Alzheimer’s Association, 2017; Association, 2017). Approximately 5.4 million people of Burns & Iliffe, 2009). all ages are living in the United States with Alzheimer’s Alzheimer’s disease progression and rate of decline disease and this number is expected to increase as the vary from person to person. People with Alzheimer’s population ages (Alzheimer’s Association, 2017). There disease die an average of 4 to 8 years after diagnosis; are more women than men with Alzheimer’s disease however, the disease can last for as many as 20 years ­because women tend to live longer. The greatest risk fac- (Alzheimer’s Association, 2017). The underlying patho- tor for Alzheimer’s disease is advancing age (Alzheimer’s physiological process of Alzheimer’s disease has been Association, 2017). It is estimated that dementia of purported to begin years, if not decades, before the clinical the Alzheimer’s type affects over 13% of adults older symptoms are apparent (Dubois, Padovani, Scheltens, than 65 years or one out of eight people (Alzheimer’s Rossi, & Dell’Agnello, 2016; Sperling et al., 2011). In Association, 2017). By 2050, the number of people age 2012, the National Institute on Aging and the Alzheimer’s 65 and older with Alzheimer’s disease is projected to Association issued new criteria and guidelines for the almost triple, from 5 million to a projected 16 million diagnosis of Alzheimer’s disease. One of the decisions (Alzheimer’s Association, 2017). was to consider Alzheimer’s disease a disease with two Alzheimer’s disease is a chronic, devastating, progressive, stages—mild cognitive impairment due to Alzheimer’s incurable neurodegenerative disease and predominant form and dementia due to Alzheimer’s. Persons with mild of dementia (Van Cauwenberghe, Van Broeckhoven, & cognitive impairment due to Alzheimer’s exhibit mild Sleegers, 2016). It is characterized by severe neuronal cognitive decline greater than that expected for their age and cognitive loss, accumulation of plaques, and forma- and education level (Alzheimer’s Association, 2017), but tion of neurofibrillary tangles (Van Cauwenberghe et al., these changes do not necessarily interfere with normal 2016). Known risk factors for Alzheimer’s disease are activities. The second stage, dementia due to Alzheimer’s, increasing age, family history, and genetics; however, is characterized by memory, thinking, and behavioral biological and lifestyle factors have also been associated symptoms that impair the person’s ability to function with Alzheimer’s disease (National Institute on Aging, in daily activities (Alzheimer’s Association, 2017). The 2016). The pathophysiological processes that trigger new guidelines also propose, for research purposes, the development and progression of Alzheimer’s disease a preclinical phase that occurs before symptoms like remain unclear and complex. While there is no single memory loss develop (Alzheimer’s Association, 2017). In event identified to date that explains the development this period prior to clinical symptoms, however, changes of Alzheimer’s disease, several hypotheses are being put in the brain, cerebrospinal fluid, and/or blood occur and forward to explain the disorder (Kumar, Singh, & Ekavali, can be measured (Alzheimer’s Association, 2017). 2015). The hallmark microscopic features of Alzheimer’s disease are the presence of beta-amyloid containing Parkinson’s Disease. Parkinson’s disease is the second neuritic plaques and neurofibrillary tangles in the brain most common, progressive, neurodegenerative disorder (Kawczynski Pasch, 2009; Takahashi, Nagao, & Gouras, after Alzheimer’s disease, affecting approximately 1 million 2017). Neuritic plaques occur as a result of an abnormal people in the United States, with as many as 60,000 accumulation and clumping of the beta-amyloid protein new cases appearing each year (Parkinson’s Disease between the nerve cells early in the disease process. The Foundation, 2017). This estimate may be low because Matzo_27129_PTR_16_373-398_05-24-18.indd 375 5/25/18 2:55 PM  376 III. PHYSICAL HEALTH: LIFE-THREATENING DISEASES diagnosis is uncertain and may take several years to person to person. In some people, the disease progresses conclude Parkinson’s disease. Life expectancy for people more quickly and in others it may take 20 years or more with Parkinson’s disease is not as good as once thought. (NINDS, 2014). Symptoms usually begin on one side of One study found the duration between disease onset to the body and eventually progress to both sides, causing death ranged from 7 to 14 years with differences being balance problems and severe disability. In the late stage explained by age at diagnosis and the year the study was of Parkinson’s disease, people are bedbound and are performed (MacLeod, Taylor, & Counsell, 2014). The commonly afflicted by complications such as choking, presence of a diagnosis of dementia was found to be pneumonia, and falls that can be the contributing factor an independent predictor of mortality (MacLeod et al., leading to death (NINDS, 2014). Currently, there are no 2014). Parkinson’s disease is the 14th leading cause of curative treatments, but early pharmacological treatment death in the United States (Kochanek, Murphy, Xu, & can limit the progression of the symptoms associated Tejada-Vera, 2016). Its symptoms mimic other condi- with Parkinson’s disease. Levodopa is considered the tions and there is no biomarker for the disease. These first-line treatment for ­Parkinson’s. It is a precursor to figures are projected to grow as the age of the population dopamine. While l­evadopa is used because it is able to increases. The average age of onset of Parkinson’s disease cross the blood–brain barrier, it is still susceptible to is 60 years; however, 5% to 10% are diagnosed younger enzymatic breakdown. To minimize the metabolism of than the age of 50. Statistics have shown that men are levodopa in the body, it is often given in conjunction affected more often than women (National Institute of with carbidopa, which decreases the amount of levodopa Neurological Disorders and Stroke [NINDS], 2014). breakdown, allowing more levodopa to enter the CNS Like Alzheimer’s disease, the exact etiology of P ­ arkinson’s and be converted to dopamine (Gopalakrishna & disease is not known. The only known definitive risk Alexander, 2015). factor for the development of idiopathic Parkinson’s disease is aging. Scientists suspect that both genetics and Multiple Sclerosis. MS occurs when the body’s i­ mmune environmental factors play a role in the development system attacks myelin. Myelin is the fatty substance that of Parkinson’s disease; however, they are unsure of the surrounds and protects the nerve fibers of the central pathogenic role each plays and in what combination nervous system. MS is thought to be immune mediated and/or dose. Most cases of Parkinson’s disease occur (National Multiple Sclerosis Society, 2016). The damaged without an obvious genetic cause (Parkinson’s Disease myelin develops scar tissue (sclerosis), which disrupts Foundation, 2017). Possible environmental toxins that or slows the nerve impulses traveling to and from the have been connected to Parkinson’s disease are pesti- brain (National Multiple Sclerosis Society, 2016). Many cides/herbicide, exposure to metals, solvents and poly- researchers believe that MS is an autoimmune disease chlorinated biphenyls (PCBs), 1-methyl-4-phenyl-1,2,3, (NINDS, 2017b). Approximately 250,000 to 350,000 6-tetrahydropyridine (MPTP) found in some synthetic people in the United States are living with MS, with an forms of heroin, and viruses (NINDS, 2014). The hallmark estimate of 200 new cases a week (NINDS, 2017b). MS features of Parkinson’s disease are the loss of dopami- can occur between the ages of 10 and 80 years; however, nergic neurons in the substantia nigra and the presence a majority are diagnosed with their first attack between of Lewy bodies. Parkinson’s disease develops when the ages of 20 and 50 years. Women are two to three times brain cells in the substantia nigra begin to degenerate more commonly affected than men. The disease is more and die, resulting in progressive development of motor common in whites, especially those of Northern European and nonmotor symptoms (NINDS, 2014). This structure ancestry. There is a strong genetic link, especially for first- makes and stores the neurotransmitter dopamine; the degree relatives where there is approximately a 1 in 40 degenerative process results in the depletion of dopamine, chance of developing MS ­(National Multiple Sclerosis a compound involved in communication between brain Society, 2016). Most people with MS have a normal life cells. Approximately 60% to 80% of dopamine cells are expectancy. However, a rare form of the illness can be fatal lost before clinical symptoms start to appear. The four within weeks (National Multiple Sclerosis Society, 2016). primary motor symptoms are bradykinesia (i.e., slowness The exact cause of MS is unknown. It is thought to of movement), rigidity, postural instability or balance be caused by genetic vulnerabilities combined with envi- problems, and resting tremor (NINDS, 2014). Nonmotor ronmental factors, which subsequently trigger an autoim- symptoms associated with Parkinson’s disease are many; mune attack on the myelin, nerve fibers, and neurons in some are minor while others are debilitating, including the brain and spinal cord (NINDS, 2017b). Scar tissue depression, emotional changes, difficulty with swallow- forms hard sclerotic plaques in multiple regions of the ing and chewing, loss of sense of smell, speech changes, CNS. Early in the illness, the myelin sheath is affected; urinary problems or constipation, skin problems, sleep however, the nerve fiber does not allow nerve impulses problems, dementia, orthostatic hypotension, muscle to transmit signals through the nerve. As the damage cramps and dystonia, pain, sexual dysfunction, and fatigue to nerves progresses, the nerve axons are destroyed and and loss of energy (NINDS, 2014). The progression of nerve impulses are totally blocked, resulting in permanent Parkinson’s disease symptoms manifests differently from loss of function (NINDS, 2017b). Matzo_27129_PTR_16_373-398_05-24-18.indd 376 5/25/18 2:55 PM  16. Neurological Disorders 377 MS is highly unpredictable from person to person. of sight, touch, hearing, taste, and smell are not affected, There’s no set pattern to nerves affected, thus making it while ocular movements, bowel and bladder function, difficult to predict the disease course (NINDS, 2017b). and cognitive abilities are occasionally affected (NINDS, There are four main patterns to the presentation of MS. 2017c). ALS onset causes progressive loss of limb strength, Relapsing-remitting is the most common form of MS and dysphagia, dysarthria, and ultimately r­ espiratory failure is characterized by flare-ups that appear for several days (Chio, Mora, & Lauria, 2017). Initial symptoms com- to weeks followed by remissions, during which not all monly include twitching, cramping, or stiffness of muscles; symptoms resolve completely. Primary-progressive MS muscle weakness affecting an arm or a leg; slurred and is a less common form in which the disease manifesta- nasal speech; or difficulty chewing or swallowing. As the tions gradually worsen over time without periods of disease progresses, muscle weakness and paralysis spread remission. The secondary-progressive MS form starts to the muscles of the trunk of the body. Eventually, in out as a relapsing-remitting course but later progresses the more advanced stage of the disease, muscles that to primary-progressive MS. Progressive-relapsing MS control vital functions are impaired, causing difficulties is the least common form that is progressive from the in speech, swallowing, and breathing. Respiratory failure beginning, with episodes of acute increased worsening is the cause of most deaths (NINDS, 2017c). of existing symptoms or new ones (National Multiple Treatment of ALS remains focused on symptom Sclerosis Society, 2016). Symptoms of MS are not the management with the goal of maintaining QOL. An same for everyone; it depends on the location and extent interprofessional team is typically established, including of damage to the myelin in the CNS. Symptoms can come a neurologist, nurses, physical therapists, occupational and go, range from mild to severe, and differ greatly therapists, speech pathologists, psychologists, and many between one relapse to another (National Multiple others, to help navigate patient management (Vucic, Sclerosis Society, 2016). The most common symptoms Rothstein, & Kiernan, 2014). Drug therapy options are fatigue, numbness, walking balance and coordina- ­include riluzole, which was approved in 1995 by the FDA tion, bowel and bladder dysfunction, vision problems, to treat ALS and remains the only approved treatment dizziness, sexual dysfunction, pain, cognitive dysfunction, to date (Poppe, Rue, Robberecht, & Van Den Bosch, emotional changes, depression, and spasticity (National 2014). Potential therapeutic approaches focus on causal Multiple Sclerosis Society, 2016). treatment or modifying treatment. Causal treatment focuses on directly targeting disease-causing genes to Amyotrophic Lateral Sclerosis. ALS, or Lou Gehrig prevent their expression, while modifying treatments disease, is a rare but rapidly progressive neurodegenera- focus on targeting factors or mechanisms that influence tive disease that affects both upper and lower motor pathological processes related to ALS (Poppe et al., 2014). neurons leading to progressive muscle atrophy of the voluntary muscles in the arms, legs, and trunk (NINDS, Coma. Coma is the result of injury to the brain and is 2017c). The incidence of ALS is higher in men than in characterized as a deep state of unconsciousness in which women and typically strikes people between 40 and 60 a person cannot be awakened and does not purposefully years of age (NINDS, 2017c). Roughly, 20,000 to 30,000 respond to stimuli (NINDS, 2017a). Those in the deep- Americans are living with ALS at any given time with est coma are not conscious of self or their environment; approximately 5,000 new cases diagnosed each year. they show no sleep–wake cycles or auditory or visual ALS is a fatal disease; most die from respiratory failure. responses, and have reflex and postural responses only The average life expectancy after onset of symptoms is to external stimuli (Posner, Saper, & Schiff, 2007). 3 to 5 years; however, approximately 10% will survive To date, precise estimates of the incidence and prevalence 10 or more years (NINDS, 2017c). of coma in the United States are not available due to lack The etiology and pathogenesis of ALS remains un- of surveillance, incomplete diagnostic codes for stages of clear and multifactorial, possibly involving both genetics recovery, and misdiagnosis in the assessment of disorders of and environmental exposures. However, over the years, consciousness (McNamee, Howe, Nakase-Richardson, & ­researchers have identified only a few possible pathogenic Peterson, 2012). However, prevalence estimates for processes that occur after symptom onset, such as mito- vegetative state (now known as unresponsive wakeful- chondrial dysfunction, protein aggregation, generation ness syndrome) and minimally conscious state (MCS) of free radicals, excitotoxicity, hypermetabolism, inflam- range from 25,000 to 420,000 and 112,000 to 280,000, mation, and apoptosis (Gordon, 2011; van Es et al., respectively (Hirschberg & Giacino, 2011). The state of 2017). In addition, only 5% to 10% of the cases are of arousal and wakefulness is dependent on functioning the inherited type, leaving 90% of the cases classified cerebral hemispheres and the brainstem’s regulatory as sporadic or meaning there is no clear cause (NINDS, system called the reticular-activating system (RAS; 2017c). Diagnosis is made primarily on the medical his- Posner et al., 2007). The RAS is located at the core of tory and the physical and neurological exam showing the brainstem. Damage to either the RAS area or both upper and lower motor neuron damage that cannot be cerebral hemispheres will result in an altered level of attributed to other diseases (NINDS, 2017c). The sense consciousness or coma (Posner et al., 2007). Matzo_27129_PTR_16_373-398_05-24-18.indd 377 5/25/18 2:55 PM  378 III. PHYSICAL HEALTH: LIFE-THREATENING DISEASES Coma etiology can be classified into one of three main Some of the causes are treatable and reversible, whereas categories: supratentorial lesions, infratentorial lesions, others are not. The prognosis depends on the cause, the and metabolic encephalopathy. Supratentorial-type coma severity of damage, and the site of neurological damage results from the formation of a mass (i.e., brain tumor, (NINDS, 2017a). Comas vary in their duration from days stroke, head trauma with brain swelling, brain abscess) that to weeks, typically lasting no longer than approximately 4 expands, producing brain herniation through the tentorial weeks (NINDS, 2017a). The most critical factors involved opening into the tentorial compartment, causing fatal in determining prognosis is etiology of the coma, the brainstem hemorrhages and ischemia (L. Davis, King, & clinical depth of coma, and the length of time the person Schultz, 2005). Infratentorial-type coma occurs when remains comatose. Other factors include the age of the a tumor or ischemic stroke involving the brainstem or person, neurological findings, and concurrent medical cerebellum damages or compresses reticular formation. complications (Posner et al., 2007). Poor prognostic signs Metabolic encephalopathy–type coma results from a are when the coma follows cardiac arrest or if the patient variety of sources that affect brain chemistry (i.e., drugs, has not regained pupillary function or purposeful move- hypoxia, blood glucose abnormalities, organ diseases, ment after 72 hours. vitamin B deficiencies, poor cerebral perfusion, toxins, Coma due to traumatic head injury has a worse prognosis etc.). Metabolic coma develops acutely and is often in older patients, with patients older than 60 years being reversible if the underlying disorder is treated (L. Davis three times more likely to die than patients younger than et al., 2005). Many comatose patients progress to being 20 years (Simon, 2000). Most comatose stroke patients brain dead. Diagnosing brain death remains challenging; do not survive. Medical comorbidities, advanced age, and to date, there is no one specific protocol to determine complications all negatively affect survival. The majority clinical criteria of brain death (Hills, 2010). Brain death of coma patients will die in the acute care setting, except is defined as the irreversible loss of function of the brain, for those whose coma is persistent. They are transferred including the brainstem (Posner et al., 2007). to a long-term care facility for supportive nursing care. The differential diagnosis for coma includes a struc- Similar to stroke, they may die from the initial damage tural lesion (stroke, head trauma, tumor), meningeal to the brain, which precipitated the coma, or from sub- irritation (infection or bleeding), metabolic encephalopa- sequent complications or comorbid conditions. thy (organ failure, drugs), and seizure (Simon, 2000). After the coma phase, some people gradually recover, Getting a history from witnesses or significant others is some progress into a vegetative state, and others become helpful in making a differential diagnosis. If the coma brain dead. People who do emerge from a coma may was preceded by a period of confusion or delirium, this have problems with complex thinking, emotional stabil- is more consistent with infection or metabolic etiolo- ity, and physical difficulties. The most common cause gies, whereas sudden loss of consciousness suggests an of death for someone in a persistent vegetative state is intracranial bleed or infarct. infection, such as pneumonia. Initial priority in the emergency management of coma- The common causes of pediatric coma are injury, tose patients is to evaluate and maintain respiratory and shock, metabolic disorders, ingestions, and CNS infec- circulatory function and then to establish the underlying tions. Altered mental status in children covers a range of disease process. Timely diagnosis will improve the likeli- behaviors, and irritability, lethargy, changes in feeding or hood of reversing the coma, when this is possible, and sleeping habits, and other subtle behavioral changes can reducing mortality. The Glasgow Coma Scale (GCS) is often be indicative of impairments in the child’s CNS. History used to assess and score the level of consciousness. Eye from the caregiver is critical. One recent study suggests opening, verbal response, and motor response to stimuli that studying reactive encephalographic patterns in are evaluated. The Consciousness Scale for P ­ alliative comatose children may be useful in prognostication of Care (CSPC) is designed to assess consciousness in PC morbidity and mortality outcomes (Ramachandrannair, patients. It is easy to use and has good psychometrics, with Sharma, Weiss, & Cortez, 2005). a Cronbach alpha of 0.99, intraclass correlation of 0.99, and correlation with the GCS of 0.82 (Gonçalves, Bento, Alvarenga, Costa, & Costa, 2008). COMORBIDITIES ASSOCIATED WITH A pediatric version of the GCS is available (Reilly, NEUROLOGICAL DISORDERS Simpson, Sprod, & Thomas, 1988). The CHOP Infant Coma Scale or Infant Face Scale (IFS) may be more Many of the predisposing factors for stroke, Alzheimer’s useful for children younger than 2 years of age. This disease, and vascular dementia overlap with cardiovascu- scale relies on objective behavioral observations, lar disease and include hypertension, diabetes, cigarette ­assesses cortical as well as brainstem function, and smoking, high cholesterol, and African American ethnic- is based on infant-appropriate behaviors. It can also ity. This can lead to complications with unstable blood be used with intubated patients and has better inter- pressure, angina, congestive heart failure, myocardial rater reliability than the pediatric version of the GCS infarction, and arrhythmias (Cechetto, Hachinski, & (Durham et al., 2000). Whitehead, 2008; Ostwald, Wasserman, & Davis, 2006). Matzo_27129_PTR_16_373-398_05-24-18.indd 378 5/25/18 2:55 PM  16. Neurological Disorders 379 As previously stated, the risk of stroke and most CNDs most frequent nonmotor symptoms the patient may face increases with age and the older patient is also more likely (Biundo, Weis, & Antonini, 2016). Cognitive impairments to have coexisting chronic illnesses. People with MS are affect a large proportion of people, with MS occurring likely to be younger at age of onset and thus have fewer in 40% to 70% of cases and mainly affecting attention, coexisting chronic problems. Any limitations associated information-processing speed, and episodic memory with comorbid conditions can impede functional status and (Houtchens et al., 2007; Minden, 2000; Rocca et al., complicate management. Comorbid illnesses have consistently 2015; Bede et al., 2011). been shown to affect function, mortality, and utilization of There is evidence that memantine and cholinester- services (Studenski, Lai, Duncan, & Rigler, 2004). ase inhibitors are modestly effective in decreasing the People with stroke and CNDs are susceptible to severity of cognitive deficits in Alzheimer’s disease and secondary complications, including pressure sores, mal- Parkinson’s disease. Cholinesterase inhibitors are used in nutrition, venous thrombosis, contractures, pneumonia, mild-to-moderate dementia, and memantine alone or in conjunctivitis, depression, and problems with bowel combination with cholinesterase inhibitors is used in the and bladder function. These complications are frequent late stages of the illness (Buckley & Salpeter, 2015; Lökk & enough that they should be anticipated, and nursing care Delbari, 2012). Reality orientation is not recommended should be aimed at prevention, early recognition, and for people with Alzheimer’s disease, but the use of envi- treatment of problems. ronmental cues and decreasing visual clutter are recom- mended. Pleasant music has been found to improve visual attention in ­patients with unilateral neglect after stroke COMMON SYMPTOMS AND TREATMENTS (M. Chen, Tsai, Huang, & Lin, 2013). Effective treatment often requires an interdisciplinary approach, including Symptoms vary greatly among people who have had a nurses, physical therapists, and occupational therapists. stroke or neurodegenerative illness, depending on the Treatment methods include reminding the patient to location(s) and severity of damage or pathology. Specific look toward his or her affected side, continual use of touch chapters in this book that cover management of symptoms on the affected side, and intentionally approaching the such as dyspnea, pain, immobility, and gastrointestinal patient from the affected side. symptoms should be consulted for an overview of treat- ment options. This section focuses on some of the PC Communication issues and their management for individuals with stroke and chronic neurodegenerative diseases such as cognition Impairments in communication are common with all and communication, affect and behavior, recognizing and disorders discussed in this chapter. Strokes occurring in treating pain, eating and swallowing, dyspnea and air the left hemisphere, in particular, can impair the abil- hunger, sleep, and infection. ity to communicate. Voice disorders can be grouped into dysarthria and dysphonia. Dysarthria is caused by neurological damage to the motor components of Cognition speech. Impairments in neuromuscular function cause dysarthria in people with Parkinson’s disease, MS, and There is great heterogeneity in cognitive deficits fol- ALS. ­Dysphonia is disordered sound production at the lowing a stroke. The person can exhibit a reduced level level of the larynx and may have a neurological, struc- of consciousness, attention deficits, and an array of tural, or functional etiology (S. M. Cohen, Elackattu, perceptual deficits that commonly impact functional Noordzij, Walsh, & Langmore, 2009). ability. For example, some patients develop neglect or a Cognitive deficits are associated with receptive and loss of awareness of their affected limbs. It is associated expressive aphasia in CNDs. As Alzheimer’s disease pro- with right hemispheric strokes and, in the extreme, can gresses to late stage, the number of words in the person’s result in patients being completely unaware of the left vocabulary is usually limited to 20 or less. Those indi- side of their body or of stimuli coming from the left side viduals with expressive aphasia may comprehend spoken of their environment. Patient safety is compromised as language but are unable to express themselves verbally to neglect can increase the risk of injury and falls. varying degrees. Receptive aphasia is the result of deficits Neurodegenerative illnesses are associated with the in receiving the message/auditory perception or retaining development of cognitive deficits; symptoms worsen as the it. Stroke patients can experience several different forms illness progresses. Early in Alzheimer’s disease, short-term of aphasia: expressive, receptive, or global aphasia. Due memory, judgment, and visuospatial problems are c­ ommon. to difficulty expressing their needs or interpreting what During later stages, the person has severe impairment is going on around them, aphasic patients may become of all cognitive functions and may no longer recognize noncompliant, angry, fearful, or withdrawn. family members. Attention, orientation, and short- and Difficulty communicating severely reduces QOL. Voice long-term memories are impaired. In P ­ arkinson’s disease, amplifiers may be helpful in the final stages of certain mild cognitive impairment and dementia are among the illnesses in which nerve damage or reduced respiratory Matzo_27129_PTR_16_373-398_05-24-18.indd 379 5/25/18 2:55 PM  380 III. PHYSICAL HEALTH: LIFE-THREATENING DISEASES support for speech contributes to a soft voice. Surgically hello, pointing, beckoning with outstretched hands, placing a tracheoesophageal prosthesis and augmenting and hugging used judiciously by the caregiver may be an atrophic vocal fold with collagen can palliate the effective communication tools. The presence of a family dysphonic patient (S. M. Cohen et al., 2009). Another member or caregiver conveys to the individual that he treatment option that has had a promising effect on or she is not alone and that the individual is respected. phonation in patients suffering from dysphagia with When cognitive impairment is present, the strategies dysphonia is laryngopharyngeal neuromuscular electri- outlined in Table 16.2 may be useful to facilitate com- cal stimulation (Ko, Park, Hyun, Seo, & Kim, 2016). munication. A calm, gentle voice communicates safety The nurse should develop a plan with the interprofes- and security. Listening to the person, even if the message sional team for providing meaningful communication and is unclear, communicates respect. Compared to those socialization that considers the wishes of the individual with mild cognitive impairment, the individual who is and the accommodations needed because of communica- severely impaired may require more focused stimula- tion deficits. A consistent caregiver or family member tion to elicit a response. Making a compassionate and is often able to understand speech that others consider meaningful connection with a person who has severe unintelligible. Supporting the remaining demonstrations dementia will often soothe a troubled anxious state. of attempts to communicate enables the patient to feel connected and accepted; he or she may begin using many Affect and Behavior more nonverbal cues to communicate needs. Behaviors listed in Table 16.1 may be used by patients The development of depression, apathy, mood disorders, to communicate to the caregiver that there is an unmet and anxiety associated with stroke and neurodegenerative need such as pain or hunger, or the need to eliminate or illnesses is common. Frontal lobe pathology and disturbed change positions (Kovach et al., 2012). neurotransmitter metabolism increase a person’s susceptibil- Anticipating physical needs decreases frustration for ity to depression. Also, adapting to the loss of physical or the person who is unable to clearly or consistently ver- cognitive abilities can be overwhelming. The individual’s balize needs. Nonverbal communication through touch, premorbid personality, coping skills, and resources are massage, and eye contact should be used. Gestures are a all factors in making this adaptation. Medications can three-dimensional language of communication; waving contribute to depressive and psychotic symptoms. People experiencing CNDs often experience periods of uncon- trolled laughing or crying, also known as pseudobulbar TABLE 16.1 Behavioral Symptoms of Unmet Needs in affect (PBA). The pathophysiology of PBA appears to People With Impaired Communication and/or Cognition be a lack of voluntary control, or disinhibition, but the pathways are yet to be clearly understood (Ahmed & Any change in behavior Simmons, 2013). Patients with Parkinson’s disease are Restless movement increasingly seen with impulse control disorders like compulsive gambling or buying and sexual and eating Moaning behaviors (Weintraub & C ­ laassen, 2017). These impulse Tense muscles control disorders have been most closely related to the use of dopamine agonists (Weintraub & Claassen, 2017). Facial grimace People with CNDs are both underdiagnosed and Agitation inadequately treated for mood and anxiety disorders (Hallford, McCabe, Mellor, Davison, & Goldhammer, Combative/angry 2012). Depression was exhibited in 31% of stroke survi- Pulling away vors at any time from the moment of the stroke to 5 years (Hackett & Pickles, 2014). In people with MS, mental Changes in mobility health comorbidities contribute to secondary disability Rubbing/holding or bracing of a body part and decrease their QOL (Turner et al., 2016). People with neuromuscular problems may appear less animated at Crying/tears in eyes baseline, so flat affect and decreased i­ nvolvement in activ- Change in sleep ity are not useful cues for depression or mood disorders. A systematic literature review using Cochrane Confusion methodology found a lack of evidence of an associa- Changes in appetite tion between the severity of Alzheimer’s disease and the prevalence of comorbid depressive symptoms or Verbal perseveration depression (Verkaik, Nuyen, Schellevis, & Francke, Withdrawal/quietness 2007). This review suggests that prevention and inter- vention strategies for depression should be aimed at Increased pulse or respiration all people with Alzheimer’s disease regardless of their Matzo_27129_PTR_16_373-398_05-24-18.indd 380 5/25/18 2:55 PM  16. Neurological Disorders 381 TABLE 16.2 Communication Strategies With the Cognitively Impaired Make sure the person knows you are present before communicating to avoid startling or frightening the person. Touchingthe person gently may be used to begin the communication. A conventional handshake may be well tolerated. Assess the person’s reaction and gradually increase the use of appropriate touch, if tolerated. Keep voice, facial expression, and body movements calm, slow, clear, and positive. Use short, simple, adult sentences. Use the name of the person most familiar to them. Avoid the use of pronouns. Use visual cues to augment verbal message. Limit choices to two options to avoid overwhelming the person’s cognitive ability. Avoid “why” questions which may be perceived as threatening. Avoid negative feedback statements such as “Don’t.... ” Avoid working to teach or orient the person. Since short-term memory is severely impaired, this is ineffective. Listen to the person’s verbal message attentively and allow enough time for the person to communicate with you. Validate the feelings behind the words. For example, “I hear that you are upset and I am here to help” or “I’m glad you’re okay.” Tapes of family members may be used to provide simulated presence therapy. End all interactions with positive feedback such as “I appreciate this time with you,” or “It was nice to visit with you today.” disease severity. Geropsychiatry consultations are often People with dementia have heightened sensitivity to needed to competently assess and treat those with more environmental stressors and cues (Chaudhury, Cooke, complicated symptomatology. Cowie, & Razaghi, 2017). High decibel sound is associated There are few systematic studies of psychotherapy with higher levels of agitation in people with dementia and pharmacotherapy in individuals with CNDs. Evi- (Joosse, 2012). Health professionals should conduct a dence supports the use of selective serotonin reuptake noise assessment by listening at various times of the day inhibitors (SSRIs) as the drugs of choice for depressive to sources of noxious or extraneous noise. Eliminate disorders because of their safer drug profile, and anxiety echo, background conversations, and television used for disorders may be treated effectively with combined drug background sound. Provide brief periods of music listening and nonpharmacological therapy (Chan, Cordato, & with selections that are pleasing to the patient (Padilla, O’Rourke, 2008; Lökk & Delbari, 2012). 2011). The visually accessible environment may be quite Behaviors listed in Table 16.1 are common in people circumscribed, so it is important that it be pleasant and with dementia and may indicate an underlying need such as stress free as possible. Avoid fluorescent lighting that as pain or hunger. People with dementia are very sensi- often creates a glare. Keep some items that are familiar to tive and reactive to stressful stimuli in the environment, the person in the immediate area; for example, pictures, though this behavioral response is somewhat less evident afghans, and pillows may convey home and familiarity. in later stages. The person may have active hands and Spaces that are too big or too small, as well as cluttered repetitive movements or vocalizations (i.e., persever- areas, should be avoided. One or two plants or flower ance). Delusions and hallucinations may be present and arrangements are preferred to an overwhelming clutter of the person may display agitation, wandering, aggressive flora. Avoid tactile stressors by keeping the room tempera- outbursts, and spontaneous screaming. ture comfortable. Avoid itchy skin by keeping the skin well The person who has decreased competence, particu- lubricated and treating with medicated emollients; flannel larly cognitive competence, is more affected by stressors sheets and silk pillowcases may provide some comfort. from the environment and has a decreased threshold In addition to decreasing environmental stress, there is for tolerating stressors from the environment (Kovach, a need to balance sensory stimulating and sensory calming 2000). Consideration of this environmental vulnerability activity (Kovach et al., 2004). As the illness progresses, creates the need for two foundational interventions: there may be a need for more sensory calming time, and the person will probably tolerate less than 1 hour Provide a positive environment with few environ- of activity before needing a decrease in environmental mental stressors. stimulation. Often, only brief visits of 10 minutes or Balance sensory stimulating and sensory calming less will be tolerated. The person may need to engage activity throughout the day. (Kovach et al., 2004) in frequent inner retreat by withdrawing from others. Matzo_27129_PTR_16_373-398_05-24-18.indd 381 5/25/18 2:55 PM  382 III. PHYSICAL HEALTH: LIFE-THREATENING DISEASES This need should be explained to the family so they do Eating and Swallowing not feel shunned; if the patient shuns socialization, allow him or her some solitary time and approach again later. Dysphagia and aspiration are problematic in stroke and Agitated behaviors are associated with cognitive impair- neurodegenerative illnesses. Swallowing is a complex ment and increase in frequency as dementia progresses process that involves precise coordination of the oral (Y. Chen, Ryden, Feldt, & Savik, 2000; Kales, Gitlin, & cavity and the pharynx (Sasegbon & Hamdy, 2017). Lyketsos, 2015). Physical aggression is a behavioral Damage to any portion of the complex process of swal- manifestation for many dementia patients, and it is lowing can lead to dysphagia (Sasegbon & Hamdy, usually related to identifiable triggers or communication 2017). Dysphagia resulting from stroke is temporary challenges (Wharton & Ford, 2014). Social contact and in 90% of cases but is a part of the general progress of focused, therapeutic stimulation have been associated the illness for CNDs discussed in this chapter (Broadley with decreases in agitated behavior (Draper et al., 2000; et al., 2005). Dysphasia can occur in the oropharyngeal McGonigal-Kenny & Schutte, 2006). Stimulation of or esophageal phases of swallowing. The oropharyngeal multiple senses may enhance engagement in the activity. phase is voluntary and depends on motor and sensory Friendly visit, hand massage, music listening, and pet pathways triggering a series of movements that move food therapy are just a few examples of therapeutic activities. posteriorly to the oropharynx. Oropharyngeal dysphagia Multiple activity therapy books provide suggestions for has a neurological cause in 75% of cases (Ertekin & therapeutic activities that accommodate any level of Aydogdu, 2003). Esophageal dysphagia is more likely caused cognitive or functional deficit and enhance QOL. by obstruction or stricture (Navaneethan & ­Eubanks, Perseverant behavior, defined as repetitive movement 2015; White, O’Rourke, Ong, Cordato, & Chan, 2008). or verbalization, may also occur in patients with CNDs. Patients with swallowing disorders are at risk of aspira- Perseverance may indicate boredom, discomfort, or an tion pneumonia. Sialorrhoea, or an excessive secretion unmet need; it may also be a simple tension reduction of saliva, is common for those with Parkinson’s disease, mechanism. Calm repetitive movements or verbaliza- ALS, and MS (Andersen et al., 2012; McGeachan & tions may be a coping mechanism and may not require McDermott, 2017). Low food consumption, food treatment. It is important to determine if environmental pocketing, difficulty manipulating food on the plate and stress needs to be decreased or, alternatively, if stimulating transporting it to the mouth, weight loss, and nutritional activity should be provided. Health professionals should impairments are common in Parkinson’s disease (Simons, assess for basic comfort needs: offer a drink, be certain 2017; Westergren, Ohlsson, & Hallberg, 2002). elimination needs have been met, provide a warm blanket The swallowing mechanism is quite complex, involving or sweater, check for pressure points, and ensure good 26 sets of muscles and six nerves, and is dependent on positioning. If pain is suspected, administer an analgesic. critical timing of several phases and highly coordinated Aggression and resisting care may also be present. movement (Shapiro & Downe, 2003). Individuals in Resisting care may indicate that pain control is inad- the late stages of neurodegenerative diseases will have equate. This behavior is often temporary, so the caregiver dysphagia and be at risk of aspiration and malnutri- should repeat the attempt to provide care following a tion (Altman, Richards, Goldberg, Frucht, & McCabe, short break. Paratonia is a primitive reflex that may be 2013; Langmore, Grillone, Elackattu, & Walsh, 2009). present and is often mistaken for resisting care. Paratonia Management strategies are important in preventing is involuntary resistance of an extremity in response to morbidity. All stroke patients should be evaluated by sudden passive movement. A caregiver who moves a speech therapy to determine if feeding by mouth is safe patient’s arm may evoke this movement that appears to and if aspiration precautions are needed. Techniques be resistance to care. Slow and gentle touch decreases commonly used to assist individuals with dysphagia the likelihood of inducing paratonia. to swallow safely will be reviewed. Other options for Delusions and hallucinations, when present, are a real managing eating problems will be discussed. part of the person’s mental life and can be very discom- Prior to the meal, several interventions may be helpful. forting. These alterations in perception often respond For example, oral hygiene is important in maintaining a well to psychotropic drugs. Caregivers should not agree normal viscosity to the saliva, and aggressive oral care or disagree with the false perception, but there is a need may reduce the risk of pneumonia (Marik & Kaplan, to provide comforting intervention. For example, saying, 2003; Sura, Madhavan, Carnaby-Mann, & Crary, 2012). “I hear that you are afraid and I will keep you safe,” If the person is taking medications that dry the mouth, validates feelings and provides reassurance. Distraction or artificial saliva products should be used. Safe swallowing provision of a comforting intervention such as a friendly methods include upright posture, chin-tucking, careful visit will often soothe the person’s troubled state. Also, slow swallowing, and specific maneuvers designed to check to be sure the person’s glasses and hearing aid improve swallowing (D. L. Cohen et al., 2016; Marik & are in place and functioning properly. Many suspected Kaplan, 2003). For the person with dementia, provid- delusions are actually mixed messages resulting from ing some cueing that mealtime is coming is helpful. For impaired hearing. example, in a long-term setting on units for individuals Matzo_27129_PTR_16_373-398_05-24-18.indd 382 5/25/18 2:55 PM  16. Neurological Disorders 383 with late-stage CND, in the late afternoon a tablecloth relative to the desire to initiate assisted feeding is helpful. and a vase of flowers are placed on each table to signal There are no randomized clinical trials examining the that it is the start of evening mealtime. It is important to outcomes of tube feeding. In the case of ALS, percuta- reduce distractions during mealtime, so the focus is on neous endoscopic gastrostomy (PEG) tube placement is eating and swallowing. If the person is in a long-term common and evidence suggests that nutrition and QOL care facility, the dining room should optimally seat 16 or are improved and survival is prolonged (Andersen et al., fewer residents to decrease the potential for overstimula- 2012; Dorst et al., 2015). There is no evidence that tube tion. Turning up lights, increasing visual contrasts, and feedings reduce the incidence of aspiration pneumonia, improving acoustics have been suggested to enhance prolong life, or improve QOL for people with advanced mealtimes in group dining rooms (Brush & Calkins, dementia (Gessert, Mosier, Brown, & Frey, 2000). Other 2008). Be certain the person is comfortable and that possible problems associated with tube feeding include the environment is comfortable and free from odors. diarrhea and possible skin breakdown, as well as the Provide verbal cueing to assist the person to eat; for need for physical restraints if tubes are pulled and wound example, say “the food is coming” and “swallow now.” infection occurs. Do not rush the person to eat and swallow too quickly, but be aware that excessive time spent at the task may Dyspnea lead to fatigue and decreased eating. Provide positive encouragement during the meal. Plastic utensils should In people with CND involving motor systems, respira- not be used because a biting reflex may occur, especially if tory insufficiency is common late in the illness. Chronic the gums or teeth are touched with the utensil. Applying nocturnal hypoventilation is also common. Weakness of gentle pressure on the jaw and cheek muscles may break the respiratory muscles produces a restrictive ventilatory the biting reflex. The following feeder behaviors may defect with resulting atelectasis and a feeling of dyspnea help to sustain eating behaviors of the patient: talking or increased work of breathing. Expiratory weakness and reorienting the person to the meal, offering drinks is generally more prominent than inspiratory weakness between bites, holding the spoon ready for a bite, and and may contribute to impaired coughing, aspiration, warmly touching the person. and the development of pneumonia. Thick respiratory There is a need for research on drugs used to stimulate secretions may be difficult to manage and uncomfort- appetite and promote weight gain in anorexic individu- able. Respiratory insufficiency is particularly severe in als with neurological illnesses. Dronabinol is a cannabis ALS and MS. derivative, which in one study of people with Alzheimer’s In people with CND involving motor systems, disease was associated with a 0.5 to 1 kg greater weight ­respiratory insufficiency may cause chronic nocturnal gain than with placebo (Chapman, 2007). hypoventilation and sleeplessness. Respiratory manage- The person with dysphagia will require alterations ment of these patients has evolved from no treatment to in diet. Dietitians can suggest foods that are easier to the use of strategies to avert respiratory failure and to swallow. Calorie-dense pureed diets and thickened permit the extubation of unweanable patients without liquids may be used. One large randomized controlled a tracheostomy. An international panel, experienced in trial found that honey-thick liquids were more effective continuous noninvasive intermittent positive pressure than nectar-thick liquids, and chin-tucking was least ventilatory support (CNVS), reviewed the strength of effective. All three interventions were more effective in evidence for the efficacy of interventions and made people with Parkinson’s disease than with Alzheimer’s recommendations for achieving prolonged survival by disease (Logemann et al., 2008). Smaller, more frequent CNVS. Controlled studies demonstrated prolongation of meals are used, and the person often takes in more food survival for several months using nocturnal bilevel positive at meals earlier in the day. For some people, the stimula- airway pressure (PAP). Daily air stacking involves the tion of a soft bolus of food, such as mashed potatoes, glottis and holding consecutively delivered air volumes may provide more stimuli for swallowing than liquids. from a volume cycled ventilator or a manual resuscita- The person should drink sufficient liquid to produce tor until no more can be held (maximal insufflation straw-colored urine. Consuming 20 to 35 g of fiber each capacity), and is recommended once the vital capacity day can help to manage constipation (Shagam, 2008). is lower than 80% of normal. Following air stacking, For patients who have had a stroke, enteral feed- a cough-timed abdominal thrust was demonstrated to ings may be seen as a temporary intervention to allow significantly increase cough peak flow. Because bilevel time for rehabilitation and recovery or as a permanent PAP cannot be used for air stacking, expiratory PAP is intervention to prolong life. For those with CND, oral counterproductive for assisting weak respiratory muscles feeding may eventually become impossible and a person and, because portable ventilators eventually become should never be force fed. The decision to tube feed is necessary for CNVS, the panel recommended that all complex and controversial; in addition, courts have patients with the ability to air stack use nocturnal non- recognized tube feeding as a medical treatment that invasive intermittent positive pressure ventilation rather can be refused. Prior decision making by the patient than bilevel PAP. The panel unanimously recommended Matzo_27129_PTR_16_373-398_05-24-18.indd 383 5/25/18 2:55 PM  384 III. PHYSICAL HEALTH: LIFE-THREATENING DISEASES tracheotomy only for patients who cannot maintain a medications should be considered possible ­interventions SpO2 of greater than or equal to 95% despite CNVS for people with CND. Relaxation techniques such as and mechanically assisted coughing (Bach et al., 2012). breathing exercises, massage, imagery, or music may Clinicians are challenged to support this strong desire also promote sleep and reduce fatigue. to live while providing appropriate expectations for life Light and fragmented sleep impairs QOL. Nocturnal after tracheostomy. hypoventilation is discussed under dyspnea. It is important Supplemental oxygen therapy for patients with respi- to keep a consistent schedule so that the diurnal rhythm ratory muscle weakness is not recommended because it is encouraged. Most people will need an afternoon nap, has been found to decrease ventilator drive and worsen but keeping the person engaged in some activities dur- high CO2 levels, decrease effectiveness of nocturnal ing the daytime might help to improve nighttime sleep. ­bilevel PAP and NIV, and render oximetry ineffective as Sundowning is a term used to describe an increase a gauge of hypoventilation, airway mucus congestion, in a number of behaviors that occur in some patients atelectasis, and pneumonia (Bach et al., 2012). with dementia in the late afternoon or evening including People who choose to not receive ventilator support agitation, aggression, wandering, yelling, and hallucina- need to be given information regarding the mechanism tions (Canevelli et al., 2016; Yevchak, Steis, & Evans, of terminal hypercapnic coma and the resulting peaceful 2012). This often indicates there is a need to improve death, so that fear is decreased. Medications discussed in the balance between sensory stimulating and sensory the chapter on dyspnea need to be administered skillfully calming activity earlier in the day (Kovach et al., 2004). to successfully prevent the feeling of “choking to death.” Specifically, there may be a need for more physical ­activity early in the day, followed by an afternoon nap. Fatigue, Activity, and Sleep Then, during the usual sundowning period, the person should be engaged in a quiet one-on-one activity. Also, Fatigue is a subjective feeling of early exhaustion that because of the severe sleep variations experienced by impacts an individual’s ability to interact mentally or people with CND, try to keep diurnal rhythms intact physically with his or her environment. It is a com- by keeping lighting low during the night and up during monly reported symptom and can be overwhelmingly the day. This may require increasing the use of artificial debilitating, especially for people with MS (Caminero & light beginning in the late afternoon. Bartolomé, 2011; Mateen, et al., 2017). An understanding of the relationship among fatigue and depression, sleep Movement disturbances, and specific deficits is needed in order to better recognize and treat fatigue. Stroke and neurodegenerative disease affect movement. Sleep disruptions and insomnia are common in people These symptoms can severely impact functional ability with stroke, dementia, and other neurodegenerative illnesses and QOL. People who suffer from a stroke may display (Amara, Chahine, & Videnovic, 2017; Brass, Duquette, hemiparesis, aphasia, dysphagia, and a variety of other Proulx-Therrien, & Auerbach, 2010; Subramanian & symptoms, depending on the location and severity of Surani, 2007). Impaired sleep and alertness affect the the damage. People with neurodegenerative disease have majority of patients with Parkinson’s disease, negatively slowed movement and gait disturbances, which progress impacting safety and QOL (Amara et al., 2017). People so that the person becomes chair- or bedbound. People with dementia show great alterations in the sleep–wake with late-stage Alzheimer’s disease may display primi- cycle with increased nighttime awakenings, decreased time tive reflexes such as hand grasping, sucking reflexes, and spent awake versus time spent in bed, increased daytime paratonia. Paratonia is the involuntary resistance of an napping, and changes in the amount of rapid eye move- arm or leg to movement of the limb by another person. ment and nonrapid eye movement sleep (Ancoli-Israel & This may be misinterpreted by a caregiver as aggressive Cooke, 2005; Subramanian & Surani, 2007). Sleep- behavior, but is actually a reflexive process.Resting tremor disordered breathing is a common symptom experienced occurs when the muscle is relaxed and may be present in neurological disorders. Identifying and treating patients when the muscle is rigid (Kurlan, Richard, Papka, & experiencing sleep disorders can have a positive effect Marshall, 2000; NINDS, 2017d; Wilson et al., 2000). on their QOL (Deak & Kirsch, 2014). Sleep is greatly Late in Parkinson’s disease, motor problems result impaired by involuntary movements and muscle spasms from drug therapy and include dyskinesias (involuntary for those with Parkinson’s disease and MS, and dyspnea movements), shorter duration of benefit or lack of benefit during the night for those with ALS and MS. from medication, and end-of-dose deterioration. Severe Patients should be carefully evaluated for factors that muscle rigidity and hypokinesia contribute to the person could be contributing to their fatigue, such as anemia, becoming bed- or chairbound. When movement does pain, occult infections, hypothyroidism, medications, mal- occur, the person often needs physical help initiating the nutrition, and sleep apnea (Oken et al., 2006). ­Adapting movement. Akinesia or freezing of movement becomes the environment to promote sleep, pacing activities, debilitating. Giving short-acting levodopa/carbidopa treating pain, and eliminating or reducing any offending every 3 to 4 hours with catechol-O-methyltransferase Matzo_27129_PTR_16_373-398_05-24-18.indd 384 5/25/18 2:55 PM  16. Neurological Disorders 385 (COMT) inhibitors is recommended to minimize episodes muscles that have been weakened by ALS. For people of hypokinesia. This regimen causes the least variation with MS, exercise may decrease symptoms but it must of levodopa in blood levels, with less off-time, more be done judiciously as overheating or overstressing the on-time, and better QOL (Lökk & Delbari, 2012). body can actually exacerbate symptoms. Occupational For people with MS, problems with walking, or gait, therapy may help patients to maintain independence for can arise from muscle weakness or stiffness, numbness, longer periods of time as the CNDs progress. poor balance, spasticity, lack of ability to coordinate muscle movements, extreme fatigue, or visual distur- Pain bances. Depending on the type of MS, symptoms can become progressively worse or recur over time. People Spasticity due to increased muscle tone is common, with ALS experience weakness, particularly of the upper especially post stroke, with MS and Parkinson’s disease limbs, and progressive muscle wasting. The person loses and presents as resistance to passive range of motion muscle strength, muscle mass, and mobility, until becom- (PROM). Brain lesions can interfere with the descend- ing completely dependent. Riluzole has antiglutamate ing CNS pathways that regulate muscle tone (Barnes & properties and has been shown to slow the course of ALS. Campbell, 2010). For people with ALS, spasticity can It is not considered useful in the later stages (Andersen actually be helpful in maintaining function as the rigid- et al., 2012; Poppe et al., 2014). ity helps replace normal muscle strength, but it causes Physical exercise is a cornerstone of rehabilitation jerky, hard-to-control movements. following stroke. However, evidence is inconclusive Shoulder pain after stroke is common, affecting from regarding the benefits of cardiovascular exercises on 16% to 72% of patients (Adey-Wakeling et al., 2016; disability, ADLs, QOL, and death (Meek, Pollock, Walsh, 2001). Both flaccidity and spasticity in the paretic Potter, & Langhorne, 2003). The goals of treatment arm can cause subluxation (partial separation), insta- of Parkinson’s disease are to slow the progression of bility of the shoulder joint, pain, and increased risk of the illness and to reduce disability without inducing subacromial bursitis. Shoulder hand syndrome or reflex long-term complications. Motor symptoms associated sympathetic dystrophy is due to autonomic dysfunction with Parkinson’s disease are disabling. While levodopa/ in the affected upper extremity. Paresis in the shoulder carbidopa is the cornerstone of treatment of motor and arm can lead to joint instability and trauma that symptoms of Parkinson’s disease, optimal response only may trigger overstimulation of the sympathetic nervous lasts 5 to 7 years. The classes of drugs approved for system. It develops in stages; typically, there is vasocon- the treatment of Parkinson’s disease include dopamine striction in the affected arm with complaints of burning agonists, COMT inhibitors, monoamine-oxidase type B pain. If it progresses beyond 3 months, the limb may (MAO-B) inhibitors, and anticholinergics. These drugs develop trophic changes with decreased hair, thin shiny do not prevent neuronal degeneration but decrease skin, increased or decreased sweating, edema, and bone ­motor symptoms (Connolly & Lang, 2014; Lo, Leung, & demineralization. Movement and touch usually cause Shek, 2007). Research is currently focused on develop- pain, and patients tend to guard the limb, leading to ing neuroprotective drugs that will slow or halt the further dysfunction. After 9 months, atrophy and contrac- progression of Parkinson’s disease. Deep brain stimu- tures may also occur. Central poststroke pain (CPSP) is lation is an option for some patients with ­Parkinson’s ­described as a neuropathic pain in all or part of the body disease who are not effectively managed through drug affected by the stroke that may develop i­ mmediately or therapy. up to 6 months after the initial stroke (Klit, Finnerup, & Research into exercise for people with Alzheimer’s Jensen, 2015). It is associated with sensory deficits and disease and related disorders has shown maintenance tactile allodynia (pain elicited by a normally nonpainful of motor skills, decreased falls, reduced rate of cogni- stimulus). This often prompts patients to lie perfectly tive decline, and improved mood (Paillard, Rolland, & still in order to avoid discomfort. de Souto Barreto, 2015; Teri et al., 2003). A systematic Pain in MS occurs as a consequence of both the review and meta-analysis showed that strengthening and disease process and the resulting disability. The preva- balance exercises in Parkinson’s disease provide benefits lence of pain in patients with MS is nearly 50%, and to physical function, strength, balance, gait speed, and ­approximately 75% of patients report having had pain health-related QOL (Goodwin, Richards, Taylor, Taylor, & within 1 month of assessment. The presence of pain in Campbell, 2008). Rhythmic auditory stimulation, in patients with MS is associated with increased age, duration which rhythms of sounds function as a cue to stabilize of illness, depression, degree of functional impairment, and enhance the organization of movement, may be ben- and fatigue. Pain in MS may originate from trigeminal eficial for improving gait parameters in stroke patients, neuralgia, headache, facial pain, tonic seizures, and limb including gait velocity, cadence, stride length, and gait pain. The pain may indicate an underlying inflamma- symmetry (Bradt, Magee, Dileo, Wheeler, & McGilloway, tory process or demyelinating lesion affecting a pain 2010). For people with ALS, exercise can help to main- pathway (O’Connor, Schwid, Herrmann, Markman, & tain the flexibility of muscles but it will not strengthen Dworkin, 2008). Matzo_27129_PTR_16_373-398_05-24-18.indd 385 5/25/18 2:55 PM  386 III. PHYSICAL HEALTH: LIFE-THREATENING DISEASES Dysesthetic extremity pain in people with MS is due to spasticity has also responded to intramuscular a result of demyelinating lesions and is described as botulinum toxin injections. Nonpharmacological treat- persistent and burning. The legs and feet are most com- ments such as ice, heat, transcutaneous electrical nerve monly affected, but the upper extremities and the trunk stimulation (TENS), and ultrasound may also relieve can also be affected. Pain is often worse at night and pain in some patients. ROM exercises, proper position- after exercise, and may be precipitated by changes in ing, and techniques to manage edema should be initiated temperature, particularly the use of warm water. Joint immediately post stroke in the affected limb. pain and back pain are common, resulting from the Treatment options used for neuropathic pain can disease process, steroid-induced osteoporosis, postural include anticonvulsants (e.g., gabapentin) and tricyclic changes, immobility, and weakness with improper use antidepressants (e.g., amitriptyline and nortriptyline). of compensatory muscles (Maloni, 2000). Pain in ALS The risk versus benefit of a trial of a tricyclic antide- is often undertreated. It can occur at all stages of the pressant in an older adult must be carefully considered disease, can affect QOL, and increase the prevalence of given the anticholinergic side effect profile. Opiates depression (Chio et al., 2017). play a relatively small role in the management of Pain experienced by individuals with stroke and neu- neuropathic pain. Vestergaard, Andersen, Gottrup, rodegenerative illnesses may also arise from the medical Kristensen, and Jensen (2001) found that the anticon- conditions commonly prevalent in the older age group, vulsant, lamotrigine, for patients with CPSP reduced or as a result of comorbid conditions such as pressure pain scores by 30% and, at 200 mg/day, the drug was ulcers, urinary retention, constipation, and contractures. well tolerated. Another study found gabapentin effec- Older people more commonly suffer from arthritis, back tive for paroxysmal pain experienced by people with problems, and other chronic conditions. MS (Yetimalar, Gürgör, & Basoglu, 2004). Adapting The consequences of untreated pain are far reaching, relaxation techniques to assist patients in coping with and assessing for pain and treating it early may help to neuropathic pain may be helpful. improve psychosocial and functional outcomes. S­ pasticity It may be difficult to recognize pain in people who is a common problem that can become disabling, ­affecting have dementia and/or communication impairment. While ADLs and, ultimately, QOL. While there is a growing self-report remains the gold standard for assessing pain, interest in the use of cannabinoids for palliation of for those who do not communicate pain verbally, it is refractory spasticity and neuropathic pain in CNDs, important to be vigilant in assessing for potential causes controversies remain over side effects versus benefits (da of pain, to observe for changes in patient behavior that Rovare et al., 2017; Peat, 2010). Early and continued may be indicators of pain, to seek surrogate reports from ROM exercises done at least twice daily to the affected caregivers, and to attempt an analgesic trial if pain is areas and individualized splinting may reduce the risk suspected (Kovach et al., 2012). Refer to Chapter 20 for of contractures. Given the risk of sedation and mental further discussion of pain management. status changes, particularly in the older adult, the decision to treat pharmacologically must be weighed carefully. Drugs such as baclofen, tizanidine, benzodiazepines, ADDITIONAL ILLNESS-SPECIFIC SYMPTOMS or dantrolene each work slightly differently, but all AND INFECTION patients using them must be closely monitored for side effects. Baclofen can also be administered intrathecally Autonomic nervous system symptoms are most common for spasticity. Nerve blocks with phenol or botulinum in MS and Parkinson’s disease, and can include postural toxin to focal areas of spasticity can be effective for a hypotension, constipation, bowel dysfunction, urine number of weeks to months but need to be repeated for