CHAPTER 14. NEUROLOGICAL DISEASES
Debra E. Heidrich and Pamela Sue Spencer
CEREBROVASCULAR ACCIDENTS
Approximately 700,000 people in the United States experience a new or recurrent cerebrovascular accident (CVA) each year, with about 500,000 of these being first attacks. In 2002, about 275,000 people died from stroke, making it the third leading cause of death in the United States (American Heart Association, 2004; Kochanek, Murphy, Anderson et al., 2004). Stroke is also the leading cause of serious, long-term disability in the United States (Centers for Disease Control and Prevention, 2001). Individuals who do not die with the acute event of a CVA tend to stabilize during hospitalization and require intensive rehabilitation therapies. Those who show steady improvement with rehabilitation are likely not terminal but may have some permanent disabilities related to the stroke; those who show continuous decline have a poor prognosis and are candidates for palliative care (National Hospice and Palliative Care Organization [NHPCO], 1996).
Etiology and Pathophysiology of Cerebrovascular Accidents
CVAs may be caused by thrombotic or embolic occlusion of cerebral vessels (ischemic stroke) or a rupture in a blood vessel (hemorrhagic stroke). Approximately 88% are ischemic strokes and most of these are caused by thrombosis (American Heart Association, 2004; Bowman, 2005). Almost all elderly people have some degree of blockage of the arterial supply to the brain, mostly due to arteriosclerotic plaques in one or more feeder arteries. The plaque activates platelets that secrete growth factors, encouraging further proliferation of the plaque. Eventually, these plaques grow large enough to occlude the vessel or may rupture, releasing emboli (Messing, 2003). Hemorrhagic strokes most often result from high blood pressure and cause the most fatalities of all strokes (Bowman, 2005). Risk factors for CVAs include hypertension, cardiovascular disease, atrial fibrillation, diabetes mellitus, cigarette smoking, hyperlipidemia, heavy alcohol use, and obesity (Bowman, 2005).
Symptoms Associated with Cerebrovascular Accidents
The location and extent of the ischemia determine the type and severity of the resulting deficits. These deficits may include the following (Bowman, 2005):
▪ Motor changes: hemiparesis, hemiplegia, intention tremor, ataxia
▪ Sensory changes: hemisensory alterations, diffuse sensory loss
▪ Visual or ocular changes: hemianopia, deviation of eyes, papillary dysfunction, loss of depth perception, cortical blindness, double vision, nystagmus
▪ Speech changes: dyslexia, dysgraphia, aphasia, dysarthria
▪ Mental changes: memory deficits, confusion, disorientation, flat affect, shortened attention span, loss of mental acuity
▪ Miscellaneous changes: nausea and vomiting, apraxia, incontinence, visual hallucinations, tinnitus, hearing loss, vertigo, dysphagia, coma, Horner’s syndrome, hiccups, coughing
Depression is common among people living with the consequences of stroke and among their caregivers. In a study of 80 stroke survivors and their spouses, Ostwald (2004) reported that depression and stress in stroke survivors are predicted by functional status and perception of recovery, whereas depression and stress in spousal caregivers are associated with perceived caregiver burden and age.
Treatments for Cerebrovascular Accidents
Rehabilitation after stroke requires an interdisciplinary team, including physicians, nurses, social workers, counselors, physical therapists, occupational therapists, speech therapists, and dieticians. Patients who have had a stroke are at risk of developing another stroke, so it is important to institute measures to prevent a recurrence. Prevention strategies include control of hypertension, control of blood lipids, anticoagulant therapy, and treatment with antiplatelet agents, such as aspirin, clopidogrel, or dipyridamole (Ezekowitz, 2004).
ALZHEIMER’S DISEASE
Alzheimer’s disease (AD) is the most common form of dementia. In 2000 there were 4.5 million Americans with AD, and it is estimated that there will be 13.2 million people with AD in 2050 (Hebert, Scherr, Bienias et al., 2003). In 2000, 58,866 people died from AD (Kochanek et al., 2004). It affects about 10% of people over the age of 65, and the incidence increases with aging. It is estimated that about 3% of people 65 to 74 years of age, 20% of those 75 to 84 years old, and 50% of people over the age of 85 have AD (Evans, Funkenstein, Albert et al., 1989). People with AD live an average of 8 years and up to as many as 20 years from the onset of symptoms (U.S. Congress, Office of Technology Assessment, 1987). Factors associated with reduced survival include rapidity of cognitive decline, decreased functional status, history of falls, frontal release signs, and abnormal gait (Hui, Wilson, Bennett et al., 2003; Larson, Shadlen, Wang et al., 2004).
Etiology and Pathophysiology of Alzheimer’s Disease
The cause of AD is not known, but it is associated with age and there is some evidence that genetic factors play a role; approximately 10% of cases are familial. Alterations in brain tissue seen in AD include the formation of beta-amyloid neuritic plaques (also called senile plaques) between neurons and neurofibrillary tangles within neurons (Delagarza, 2003; Messing, 2003). Structural changes include a thickening of the leptomeninges, shrunken gyri, widened sulci, enlarged ventricles, hippocampal shrinkage, and generalized atrophy (Black, 2005). Biochemically, there is a 50% to 90% reduction in the activity of choline acetyltransferase, the biosynthetic enzyme of acetylcholine. The severity of the cognitive losses with AD is roughly proportional to the loss of choline acetyltransferase (Mayeux & Chun, 1995). These changes lead to the memory failure, personality changes, and functional disabilities seen in AD, and these structural and chemical changes become more widespread as the disease progresses.
Symptoms Associated with Alzheimer’s Disease
Memory disturbances are usually the first sign of AD. Individuals with early AD may become irritable, suspicious, agitated, apathetic, or dysphoric. As the disease progresses, language disturbances and apraxia occur, swallowing may become difficult, and irritability and depression worsen (Black, 2005). These behaviors can place a great deal of both physical and emotional stress on the caregivers and may eventually lead to institutionalization of the patient.
Up to 25% of people with AD become clinically depressed (Mayeux & Chun, 1995). However, depression may be difficult to diagnose due to the cognitive and functional changes associated with the disease.
Malnutrition and weight loss often occur with AD and are associated with mortality risk and cognitive decline (Guerin, Soto, Brocker et al., 2005). Problems contributing to decreased oral intake include anorexia, agnosia, swallowing problems, functional impairment, and social support issues (Feldman & Woodward, 2005). The final stages of AD are characterized by an inability to ambulate independently, inability to dress without assistance, inability to bathe properly, urinary and fecal incontinence, and inability to communicate meaningfully (NHPCO, 1996).
Treatments for Alzheimer’s Disease
Medications that inhibit the degradation of acetylcholine are the mainstay of therapy for AD, but they can cause cholinergic side effects, such as nausea, anorexia, vomiting, and diarrhea (Delagarza, 2003). These acetylcholinesterase inhibitors include donepezil (Aricept), galantamine (Reminyl), memantine (Namenda), rivastigmine (Exelon), and tacrine (Cognex) (Delagarza, 2003; Hodgson & Kizior, 2006). Some studies show cognitive improvements with vitamin E and selegine (Eldepryl), but the evidence is not strong (Birks & Flicker, 2003; Tabet, Birks, & Grimley Evans, 2000). These medications should be discontinued when dementia is severe (Delagarza, 2003).
Symptomatic treatments of depression, anxiety, and agitation and of the side effects of medications are important supportive interventions throughout the course of the disease. Nutritional support may be helpful in early and mid-stages of the disease. However, enteral feedings in advanced dementia do not appear to improve outcome and may put the patient at risk for complications (Finucane, Christmas, & Travis, 1999).
AMYOTROPHIC LATERAL SCLEROSIS
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, is a disorder of progressive upper and lower motor neuron degeneration. The incidence of ALS is 0.4 to 1.8 per 100,000, and the prevalence is 4 to 6 per 100,000 people (Black, 2005). The course of ALS is relentless, without remissions, relapses, or even stable plateaus. Death usually occurs within 2 to 5 years of diagnosis, usually due to respiratory complications (Black, 2005; Messing, 2003). Older age at onset and early bulbar symptoms are associated with a shorter life expectancy.
Etiology and Pathophysiology of Amyotrophic Lateral Sclerosis
The cause of ALS is unknown, but about 5% to 10% of cases appear to have a genetic link; others are believed to be sporadic. Many different causes of sporadic ALS have been proposed, including exposure to heavy metals, viral infection, and autoimmune disorders, but there is no strong evidence for any of these theories (Rowland & Shneider, 2001). The pathology of ALS includes the replacement of large motor neuron cell bodies of the anterior horn of the spinal cord by fibrous astrocytes, resulting in gliosis. Abnormal protein aggregation, disorganization of intermediate filaments, and glutamate-mediated excitotoxicity are proposed to be involved in mediating this process (Messing, 2003; Rowland & Shneider, 2001).
Symptoms Associated with Amyotrophic Lateral Sclerosis
Upper motor neuron degeneration causes hyperactive tendon reflexes, Babinski’s reflex, and clonus. Lower motor neuron disease leads to weakness, muscle wasting, muscle cramps, and muscle fasciculations (Rowland, 1995). Interestingly, the following functions do not appear to be affected by ALS: cognition, sensation, bladder and bowel control, autonomic function, and extraocular movements. Involvement of the corticobulbar tracts causes dysphagia and dysarthria (Black, 2005; Rowland, 1995).
The functional decline and uncomfortable symptoms of ALS cause distress to both the patient and the family. A study by Adelman and colleagues (2004) showed that caregivers accurately report information about a patient’s physical function but that both patients and caregivers overestimated the psychosocial impact of the disease on the other. Caregivers rated patients as having less energy, greater suffering, and greater weariness than the patients indicated for themselves, and patients rated caregivers as more burdened than the caregivers reported for themselves.
Treatment of Amyotrophic Lateral Sclerosis
The only medication approved in the United States for the treatment of ALS is the glutamate antagonist riluzole (Rilutek). In a review of studies on riluzole, Miller and associates (2002) concluded that it probably prolongs survival by about 2 months and may improve bulbar and limb function, but it does not improve muscle strength. A longer survival benefit may be possible if riluzole is started early in the course of the disease (Wicklund, 2005). Many other treatments have undergone trial, including antioxidants, amino acids, neurotrophins, human insulin-like growth factor, creatine, minocycline, selegiline, and amantadine, but either these medications were ineffective or results were inconclusive (Mitchell, Wokke, & Borasio, 2005; Orrell, Lane, & Ross, 2005; Parton, Mitsumoto, & Leigh, 2005; Wicklund, 2005).
Individuals with ALS experience many discomforts that must be addressed to optimize quality of living.
▪ Muscle cramps can be excruciatingly painful. Use a combination of stretching, massage, and skeletal muscle relaxants to treat this problem (Simmons, 2005). One study showed improvement in spasticity by implementing an exercise program (Drory, Goltzman, Reznik et al., 2001).
▪ People with bulbar dysfunction are at risk for malnutrition and dehydration as well as aspiration due to dysphagia. Thin liquids are particularly difficult to swallow. Consult with a dietician about use of thickening agents for liquids as well as other food suggestions. Feeding via a gastrostomy tube may improve nutritional status, but the decision to accept artificial feedings is highly individual.
▪ Excessive drooling due to the inability to swallow secretions is sometimes a problem. Interventions include having suction available or use of an anticholinergic agent (e.g., hyoscyamine, atropine) to dry secretions. If anticholinergics are used, monitor for uncomfortable side effects.
▪ The dysarthria of bulbar involvement is extremely distressing to individuals. Explore other forms of communication, such as “magic slates,” Magna-Doodle slates, or communication boards. Consult with a speech therapist for additional suggestions.
▪ Dyspnea and respiratory compromise result as the intercostal muscles and diaphragm weaken. Noninvasive positive pressure ventilation (NIPPV) may prolong survival, delay the need for tracheostomy, improve cognitive function, and improve quality of life (Bourke, Bullock, Williams et al., 2003; Simmons, 2005). When NIPPV no longer provides sufficient ventilatory support, some patients may choose tracheostomy and mechanical ventilation. Again, this is a highly individual decision.
HUNTINGTON’S DISEASE
Huntington’s disease (HD) is a relentless, noncurable neurological disorder that causes gradual devastating effects to individuals in both mind and body. HD causes profound decline in cognition, behavior, and motor function in an unpredictable manner. The prevalence of HD is estimated at 5 to 8 per 100,000 individuals (SuttonBrown & Suchowersky, 2003), affecting both children and adults of all races and ethnicity around the globe. With no cure available, there is an unwavering need to embrace palliative care as a critical priority for individuals living with HD.
Etiology and Pathology of Huntington’s Disease
The main manifestations of HD include choreiform and athetoid movements, accompanied by cognitive and behavioral changes, eventually involving dementia. HD, the most common hereditary cause of chorea, is transmitted in an autosomal dominant pattern, with complete penetrance (Ravina & Hurtig, 2002). Additional features of the inheritance of HD include anticipation, a trend toward earlier onset in successive generations, and paternal descent, which refers to the tendency for anticipation to be most pronounced in individuals who inherit the disease from their father (Yamada, Tsuji, & Takahashi, 2002). Clinical manifestations usually begin between the ages of 30 to 40 years (Black, 2005). About 6% of the cases start before the age of 21 years (juvenile-onset HD) with an akinetic-rigid syndrome (the Westphal variant). Approximately 28% of cases start after the age of 50 years (late-onset HD). “Senile chorea” is simply chorea in an older person and is considered a diagnosis of exclusion; however, the majority of cases likely have HD (Quinn, 2003).
HD is caused by an anomaly of chromosome 4 and is fully penetrant, so the children of an affected parent have a 50% risk of developing the disease (Harper, 2002). The gene abnormality appears as an excessively long repeat of trinucleotides CAG, the length of which determines not only the presence of the disease but also the age of onset (Adams & Victor, 2001). The pathological process in HD is characterized by severe neuronal loss and gliosis occurring selectively in the caudate nucleus and putamen (basal ganglia), with vulnerability in other regions, such as the deep layers of the cortex (Paulsen, Zhao, Stout et al., 2001). Further studies in HD patients have shown alterations in the metabolic activity in muscle tissue and the basal ganglia. (Gu, Gash, Mann et al., 1996; Lodi, Shapira, Manners et al., 2000). Changes in the concentration of neuropeptides in the basal ganglia have also been found, including decreased substance P, methionine enkephalin, dynorphin, and cholecystokinin and increased somatostatin and neuropeptide Y. Positron emission tomography (PET) has shown reduced glucose utilization in an anatomically normal caudate nucleus (Yamada et al., 2002).
Symptoms Associated with Huntington’s Disease
Early signs and symptoms of HD include subtle abnormal eye movements, uncoordinated fine motor movements of the hands and face, clumsiness, impulsiveness, decreased concentration, and increased fluctuations of impulsiveness, anxiety, and restlessness. Poor self-control may also be reflected in outbursts of temper, fits of despondency, alcoholism, or sexual promiscuity. Disturbances in mood, particularly depression, are common (almost half of the patients in some series) and may constitute the most prominent symptoms early in the disease (Adams & Victor, 2001).
As HD progresses, physical, emotional, and cognitive symptoms become more compromised. Speech, walking, swallowing, and motor control also worsen throughout the disease trajectory. In many individuals, the involuntary, forceful, random movements worsen in an unpredictable manner and the patient is seldom still for more than a few seconds (Adams & Victor, 2001). Anxiety, stress, and lack of appropriate amounts of sleep can aggravate chorea involvement, adding to the patient’s increased fatigability. Thus, with the progression of HD, signs, symptoms, and functional decline become more debilitating. Surprisingly, during periods of sleep, the chorea movements subside and the individual is able to fully rest. Other clinical features in advanced stages of HD include urinary incontinence, motor instability, and loss of proper swallowing, which causes aspiration and cachexia. The hyperkinetic state combined with abnormalities of muscle and adipose tissue metabolism is the postulated explanation for cachexia (Lodi et al., 2000; Sanberg, Fibiger, & Mark, 1981). Inevitably, complications of functional decline and onset of infections (such as pneumonia and aspiration pneumonia due to impairment of swallowing, choking, and coughing) contribute to the cause of death in these individuals.
Juvenile-onset HD often presents between the ages of 15 to 18 years (Davis, Cheema, Oliver et al., 2005). Early signs and symptoms of juvenile-onset HD include poor school performance, difficulty in comprehending new information, slowness and stiffness, awkwardness in walking, clumsiness and frequent falls, slurred speech, choking and drooling, behavioral changes, personality changes, and slowness in responding. Seizures occur in 25% to 30% of children and usually develop after the first obvious motor abnormalities appear (Huntington Society of Canada, 2000). As the disease progresses, muscles stiffen, causing worsening rigidity. Variable signs and symptoms include weight loss, swallowing difficulties, aggressiveness, mania, hallucinations, and paranoia (Higgins, 2001).
Treatment of Huntington’s Disease
There is no cure for HD and currently no treatments that will reverse or halt the progression of this illness. Treatment is aimed at reducing symptoms, preventing complications, and maximizing the individual’s ability to function as long as possible throughout the disease trajectory (Davis et al., 2005).
Abnormal movements may be suppressed with medications that block dopamine receptors. Many dopamine antagonists, such as risperidone and olanzapine, have neuroleptic properties, which have the added benefit of controlling psychiatric symptomatology (SuttonBrown & Suchowersky, 2003). Amantadine, tetrabenazine, and other antiparkinsonian medications may also be helpful.
Depression affects 22% of patients the first year after diagnosis and remains high throughout the course of the illness (Harper, 2002; Kirkwood, Su, Conneally et al., 2001). Selective serotonin reuptake inhibitors and tricyclic antidepressants are used for the management of depression and anxiety. Treatment recommendations used specifically for juvenile-onset HD include anticonvulsant medications to help prevent and control seizures (Davis et al., 2005).
MULTIPLE SCLEROSIS
Multiple sclerosis (MS) is an idiopathic inflammatory disease characterized by demyelination of neurons in the central nervous system. MS typically occurs between the ages of 20 to 45 and affects twice as many women as men (Black, 2005; Calabresi, 2004). About 400,000 individuals are living with MS in the United States (National Multiple Sclerosis Society [NMSS], 2005). Although there is no cure for MS, the course tends to include relapses and remissions. MS usually takes one of four clinical courses:
1. Relapsing-remitting course (most common form of MS)—characterized by partial or complete recovery after attacks.
2. Secondary-progressive course—a relapsing-remitting course that later becomes progressive with only partial recovery after attacks.
3. Primary-progressive course—symptoms generally do not remit; even though there are no acute attacks, there is progressive disability.
4. Progressive-relapsing course—progressive from the outset, with obvious, acute attacks along with way.
Approximately 95% of people with MS can expect a normal life expectancy. Patients with early symptoms of tremor, difficulty with coordination, difficulty walking, frequent attacks with incomplete recoveries, or more lesions on MRI tend to have a more progressive course (NMSS, 2005).
Etiology and Pathophysiology of Multiple Sclerosis
It is believed that MS involves an autoimmune process. The trigger that initiates this process is not known, but viruses, especially the Epstein-Barr virus and herpes virus, are suspected (NMSS, 2005; Sundstrom, Juton, Wadell et al., 2004; Villoslada et al., 2003). Individuals with a first-degree relative with MS have a much greater risk of developing MS than does the general population, and some common genetic factors have been found in families where more than one person has MS. It may be that genetic susceptibility alters the body’s response to a viral infection (Black, 2005; NMSS, 2005).
When this autoimmune process is triggered, plaques form along the myelin sheath of nerves in the central nervous system, leading to demyelination. Inflammation, edema, and death of the myelin-producing cells occur over time. Remissions are probably due to reformation of some myelin when inflammation subsides. Progressive decline occurs due to scarring and destruction of the nerve axons. Although plaques may form anywhere in the white matter of the central nervous system, the optic nerves, cerebrum, and cervical spinal cord are most commonly involved (Black, 2005).
Symptoms Associated with Multiple Sclerosis
Nerve demyelination and axon scarring cause motor changes (e.g., weakness, spasticity, hyperreflexia) and sensory impairment (e.g., impaired vibration and position sense); impaired perception of pain, temperature, or touch; and moderate to severe pain. When nerves of the cerebellum are involved, the patient experiences ataxia, tremors, nystagmus, and dysarthria. Visual disturbances, bulbar signs, and vertigo occur when the cranial nerves or brainstem is affected. Changes in autonomic functioning may cause bowel incontinence, neurogenic bladder, or sexual dysfunction. Patients with MS are also at risk for depression and cognitive abnormalities (Sadiq & Miller, 1995).
Treatments for Multiple Sclerosis
Two biological response modifiers, interferon β-1a and interferon β-1b, and one immunosuppressive agent, glatiramer, are approved for the initial management of MS in the United States. These medications reduce the frequency of relapses. Another medication, mitoxantrone, is an antineoplastic agent and is used in patients with progressive forms of MS. Mitoxantrone can be used for only 2 to 3 years due to the risk of cumulative cardiotoxicity. Also, intravenous IgG, azathioprine, methotrexate, and cyclophosphamide, either alone or in combination with standard therapy, are not Food and Drug Administration approved but have been used to treat MS (Calabresi, 2004).
PARKINSON’S DISEASE
Parkinson’s disease (PD) is an idiopathic progressive neurological disorder with six cardinal features: tremor at rest, muscle rigidity, bradykinesia, flexed posture, loss of postural reflexes, and freezing movement (Black, 2005). Approximately 1% of the U.S. population over age 65 is diagnosed with PD (Centers for Disease Control and Prevention, 2005). There is no cure for this very slowly progressive disease. In 2002, 16,959 people died from PD (Kochanek et al., 2004).
Etiology and Pathophysiology of Parkinson’s Disease
The etiology of PD is unknown. PD is not thought to be an inherited disease, but the etiology may involve an interaction of environmental and genetic factors (Guttman, Kish, & Furukawa, 2003). In PD, there is selective degeneration of the dopaminergic neurons of the substantia nigra and eosinophilic inclusion bodies (Lewy bodies) in the basal ganglia, brainstem, spinal cord, and sympathetic ganglia (Messing, 2003). Dopamine is the neurotransmitter that enables people to move normally and smoothly; a severe shortage of dopamine in relation to acetylcholine in the basal ganglia leads to the clinical manifestations of PD (Black, 2005).
Symptoms Associated with Parkinson’s Disease
As mentioned above, there are six cardinal features of PD. The tremors are most often coarse movements at rest, which disappear with intentional movement. However, some people may have intention tremors as well. The bradykinesia is exhibited in a slow, shuffling gait, a loss of spontaneous facial expression (i.e., a masklike face), drooling from failure to swallow spontaneously, and stooped posture. Muscles become rigid with increased muscle tone. Although PD does not affect intellectual ability, dementia develops in about 15% to 20% of people with PD (Black, 2005).
Treatments for Parkinson’s Disease
Classes of medications used for early stage PD include the following (Guttman et al., 2003; Horn & Stern, 2004):
▪ Monoamine oxidase inhibitors, such as selegiline and rasagiline
▪ Anticholinergic medications, such as trihexyphenidyl and benztropine
▪ Antiviral dopaminergic agonist, that is, amantadine
▪ Dopamine agonists, such as pergolide, ropinirole, and pramipexole
▪ Levodopa, such as carbidopa/levodopa
There is much debate regarding which drug to choose first in the treatment of PD. Decision-making criteria include the level of disability and age of the patient. Minor symptoms may be treated with amantadine or an anticholinergic drug. These medications should be used with caution in the elderly due to the risk of cognitive and psychiatric side effects. Levodopa is used for symptoms that interfere with daily living or when other medications are no longer working. These medications may be used in combination as the disease progresses. Catechol- O-methyltransferase (COMT) inhibitors are adjunctive medications that enhance the delivery of levodopa. Examples of COMT inhibitors are tolcapone and entacapone. COMT inhibitors are added when breakthrough symptoms occur at the end of the dosing interval.
HISTORY AND PHYSICAL EXAMINATION
History
When obtaining the history, it is important to consider the ability of the patient to provide accurate and complete information. Some neurological diseases impair mentation, resulting in less-than-reliable responses from patients. A family member can assist in providing important details. Be careful to not suggest symptoms. It is important to assess the following:
▪ History of the neurological disease to date: presenting symptoms, diagnosis, and changes since diagnosis
▪ Mentation status
Language skills—ability to read, speak, and understand spoken words; for people who have lost the ability to speak, any form of meaningful communication
Memory losses: short-term or long-term
Difficulty concentrating or making decisions
▪ Functional ability
Weakness, tremors, or abnormal movements
Balance problems
Difficulty chewing or swallowing
Difficulties with self-care activities
▪ Sensory perception
Changes in ability to see, smell, hear, or taste
Abnormal sensations or pain, such as burning, numbness, tingling, or aching
▪ Bowel and bladder function
Urinary or bowel incontinence
Urinary retention or hesitancy
Constipation
▪ Breathing difficulties
▪ Psychosocial impact of disease
Signs of anxiety, fear, or depression
Patient and family assessment of coping
Strength of social support network
Physical Assessment
The physical assessment identifies the patient’s status on the initial visit and is used as a baseline to compare changes over time. In end-of-life care, it is anticipated that the disease will progress, and documentation of these changes is important for monitoring the patient’s status. Because documentation of declining patient status is required to maintain hospice support for some patients, even subtle changes are significant. However, not all of the following data are important to monitor in all patients:
▪ Mental status
Level of consciousness
Orientation
Mood and affect
▪ Motor function
Muscle strength of major muscle groups. Use a strength scale:
• 5 = Full strength
• 4 = Movement against gravity and some resistance
• 3 = Movement against gravity but not against resistance
• 2 = Movement but not against gravity
• 1 = Trace movement
• 0 = No movement
• Upper extremity: finger abductors, wrist extensors, wrist flexors, biceps, triceps, and deltoids
• Lower extremity: hip flexors, quadriceps, hamstrings, foot dorsiflexors, and foot plantar flexors
Muscle tone: any rigidity, spasticity, or flaccidity; any signs of muscle wasting
▪ Cranial nerve functioning
Babinski’s reflex
Any akinesia, dystonia, myoclonus, or tremors
▪ Ability to speak
▪ Signs of respiratory compromise
▪ Sensory function
Ability to sense light touch, pain (pin-prick), and vibration
Ability to identify numbers traced in the palm of patient’s hand (graphesthesia) and ability to identify objects by feel (stereognosis)
Sensory extinction or inattention
▪ Deep tendon reflexes
DIAGNOSTICS
Most of the neurological diseases are diagnosed based on symptoms and patient history. Magnetic resonance imaging or computed tomography scanning can confirm a diagnosis of cerebral bleeds (CVA) or show cerebral atrophy in AD or HD. PET studies in people with HD can show changes in metabolism of the caudate that correlate with clinical decline (Feigin, Leenders, & Moeller, 2001; Reynolds, Hellman, Tikofsky et al, 2002). Electromyographic findings assist in the diagnosis of ALS. Genetic testing is an important part of screening and detection of HD.
PSYCHOSOCIAL CONSIDERATONS
These progressive neurological diseases eventually cause individuals to become fully dependent on health care providers and family members for their daily functioning needs. Recommendations include integrating supportive therapy, such as physical therapy, speech therapy, occupational therapy, and nutritional counseling, into the plan of care to optimize the patient’s functional capabilities for as long as possible. Additionally, advance directives need to be discussed early in the disease course, as cognitive decline occurs with most of these diseases. Advance directives are a sensitive issue and should be discussed with patients and family members in a supportive environment. A diagnosis of any of these neurological diseases can be overwhelming. It remains central to not only engage in dialogue but to maintain open communication channels. Participating in advance care planning discussions and completing advance directives allow individuals to direct their own plan of care throughout the disease process, including their end-of-life care requests. Although not all future decisions that must be made are predictable, recognizing individuals’ concerns, hopes, and goals throughout the illness trajectory will promote a sense of control in decision making. Acknowledging and legitimating patients’ concerns communicate respect and lead to a sense of enhanced dignity (Back, Arnold, & Quill, 2003). Finally, emotional and spiritual needs are high priorities with both patients and families experiencing these progressive, incurable illnesses.
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