This is a case study of Mr. Richard, an 80-year-old man living with his daughter and son-in-law who has Parkinson's disease in its early stages. He also suffers from chronic osteoporosis and is deaf. This paper investigates the biophysical and psychosocial aspects impacting Mr. Richard in relation to his Parkinson disease diagnosis (PD). Parkinson's disease (PD) is a progressive neurodegenerative disorder marked by four key clinical features: rest tremor, stiffness, bradykinesia, and postural abnormalities. Parkinson's disease is the second most common adult-onset neurodegenerative disease after Alzheimer's (Bertram and Tanzi,2005). PD affects up to 1% of people over the age of 60. Prevalence is estimated between 18- 328 cases per 100,000. The etiology still remains unclear, however, a combination of environmental (exposure to industrial toxins and pesticides) and genetic factors is hypothesized probable. Depletion of dopamine from the basal ganglia with subsequent connection disruption to the motor cortex and thalamus results in the manifestation of the parkinsonian clinical features. Neuropathological findings in PD include (1) Loss of pigmented dopaminergic neurons of the substantia nigra pars compacta, and (2) Presence of Lewy bodies and Lewy neurites.
Investigations and Tests
The diagnosis of Parkinson Disease is largely clinical. The accuracy of diagnosis depends on the clinician’s ability to recognize the characteristic signs and symptoms. Accurate diagnosis basing on clinical features solely is reported to be about 75% increasing to about 90% with long term follow up (Rajput et al., 1991) and (Hughes et al., 2002). It is essential to establish a firm diagnosis of PD relative to other causes of the parkinsonian syndrome and ancillary tests albeit not always helpful in evaluating a suspected case of PD, do aid in ruling out some important differentials.
Imaging studies
The role of imaging investigations in the diagnosis of PD is limited and usually non diagnostic.
Magnetic Resonance Imaging(MRI)
Conventional MRI and some more advanced MRI techniques such as Striatal dopamine transporter imaging using 1231-FP-CIT single photon emission tomography (DaTscan) play a key function in excluding structural abnormalities in the brain (such as tumors and infarcts) and distinguishing PD and other parkinsonian syndromes from essential tremor. These imaging modalities still lack the ability to differentiate PD from other parkinsonian syndromes (Kägi et al. 2010), (Stoessl et al. 2011)
Positron Emission Tomography (PET)
Studies employing use of PET scans with 18F-flurodopa tracer and 18F-flurodeoxyglucose (FDG) PET have illustrated greater diagnostic accuracies in discerning PD from other parkinsonian syndromes, with a specificity of 84 to 90% (Holtbernd and Eidelberg 2014). By evaluating uptake of the tracer in the basal ganglia and metabolism in particular areas of the brain, studies have revealed PET scans yield better at differentiating PD from other parkinsonian syndromes. (Hellwig et al. 2012). Limitation of this imaging tool is its availability.
Sonography
Transcranial ultrasound is evolving into an important preclinical diagnostic imaging tool. A study by Gaenslen et al. (2008) has shown hyperechogenicity of the substantia nigra is predictive of a clinical diagnosis of PD. Hyperechogenicity of the substantia nigra, present in patients with early parkinsonism 9 times more often than in other differential ailments such as progressive supranuclear palsy, (Walter et al. 2003), strongly suggests this imaging modality be considered a key utility tool in enhancing the accuracy of clinical diagnosis of PD.
Other tests
Olfactory testing
Olfactory dysfunction, unlike in most other neurodegenerative diseases, is a common feature in PD. This attribute has heralded olfactory testing as a promising method for diagnosis PD in the early stages. (Deeb et al., 2010). Interestingly, patients with PD seldom notice the olfactory deficits.
Autonomic testing
A myriad of other tests has been proposed in the diagnosis of PD and differentiating if from other neurodegenerative disorders (Suchowersky et al., 2006). These include urodynamic tests, anal/urethral sphincter EMG, autonomic function, and quantitative Sudimotor axon reflex testing. A lack of their availability widely and absence of sufficient research data on their routine use limits them as routine diagnostic tests.
Clinical manifestations
Most of the clinical symptoms and signs observed in PD are due to the four cardinal clinical features of the disease. Clinical manifestations are both motor, which tend to start unilaterally, and non-motor.
Motor symptoms
Bradykinesia
Defined as generalized slowness of movement, bradykinesia is a major cause of disability in patients with PD. It tends to start distally and asymmetrically in the limbs. Difficulty in completing simple actions or those requiring dexterity such as buttoning clothes, picking coins, typing and getting out of sitting positions are usual common complaints in the early stages of the illness. Progression of the disorder results into gait disturbances such as shuffling, gait freezing, and festination. Other clinical features attributable to bradykinesia include:
dysphagia with subsequent ptyalism
dysarthria
Hypophonia
decreased eye movements
decreased eyelid blinking
Tremor
Tremors in PD typically occur whilst the tremulous body part is at rest/not engaged in a purposeful activity. Tremors tend to start in an intermittent manner, and similarly in an asymmetrical fashion, becoming more overt and severe with disease progression. Tremors tend to be exacerbated by emotional agitation.
Rigidity
Occurring in about 90% of patients with PD (Hughes et al., 1993), rigidity contributes to stiffness and pain reported by patients with PD. Rigidity tends to start ipsilateral to the body part affected with tremors and/or bradykinesia. A “cogwheel” pattern of the rigidity is seen during clinical examination of patients with PD. Some clinical features associated with rigidity include
Spine abnormalities (kyphosis and scoliosis)
stooped posture
difficulties turning in bed
Postural instability
This usually is a late manifestation in the course of the illness. It reflects impairment in the centrally mediated postural reflexes causing a feeling of imbalance.
It is important to note some of the motor features are due to a combination two or more of the cardinal manifestations. An example includes the ratchet joint movements which are thought to be a combination of tremor superimposed on dystonia.
Non motor symptoms
PD is increasingly being recognized as a complex disorder involving motor and neuropsychiatric manifestations. Studies show over 90% of patients with PD have non motor symptoms with some psychiatric associated elements (Barone et al., 2009). Commonly described non-motor features include
Olfactory dysfunction
Psychosis and hallucinations
Dementia
Dementia in PD is associated with memory difficulty, altered personality and psychomotor retardation, difficulty in multi-tasking and decision making. The incidence of dementia increases with age.
Cognitive dysfunction
Fatigue
Sleep disturbances
Autonomic dysfunction such as hypotension
Mood disorders such as anxiety, apathy, and depression
Depression is essentially one of the largest neuropsychiatric disorders associated with PD that affects the quality of life in PD. However, reports indicate as many as 70% of health care providers do not recognize it in clinically proven cases of PD (Simuni and Sethi, 2008), (Miyasaki et al., 2006)
Pain and sensory disturbances.
constipation
Prognosis and disease progression
Progression of PD is variable thus predicting the course of disease progression in not clinically accurate. Discrepancies have also been found in some of the severity grading systems proposed, such as the Hoehn and Yahr scale (Ray Chaudhuri et al. 2013) The American Academy of Neurology postulates the following clinical features may aid predict progress of PD (Suchowersky et al. 2006)
Faster rate of motor progression may be predicted if the patient is male, has associated comorbidities and has postural instability or gait difficulties
Older age of onset, dementia, and decrease responsive to dopaminergic therapy may predict earlier nursing home placement and decreased survival.
older age of onset and initial rigidity/hyperkinesia can be used to predict (1) a more rapid rate of motor progression in those with newly diagnosed PD and (2) earlier development of cognitive decline and dementia. However, initially presenting with tremor may predict a more benign disease course and longer therapeutic benefit from levodopa.
Duration from disease impairment to disability or death, as shown by some studies, spans between three and seven years. (Shulman et al., 2008). Within 15 years, up to 89% of patients with PD are severely disabled. Mortality in persons with PD was 3 fold more than the that of the general population of matched demographics before the introduction of the pharmaceutical agent- levodopa. This mortality rate has since reduced approximately 50%. (Grimes and Lang 1999), (Thobois et al. 2005).
Treatment and management
The goal of treatment in PD is symptomatic control for as long as possible. Delay in establishing a treatment plan promptly has been shown to have deleterious outcomes on patient’s quality of life (Grosset et al. 2007). Treatment modalities are both pharmacology and non-pharmacological.
Pharmacological treatment options
Pharmacological treatment offers both symptomatic and mild neuroprotective roles. Symptomatic treatment is further altered depending on whether the disease is in the early stages or advanced.
Treatment in early disease
Pharmaceutical agents employed in management of early disease include levodopa, dopamine agonists, and monoamine oxidase (MAO-B) inhibitors.
Levodopa
This provides greatest short term benefit. It is usually combined with carbidopa, a dopa decarboxylase inhibitor, which prevents enzymatic activity in peripheral circulation to favor accumulation in the central nervous system. Adverse effects of this medication include nausea, headaches, hallucinations, and dyskinesia (at high doses).
MAO-B inhibitors (examples; Selegiline and rasagiline)
These provide mild symptomatic benefit and have good side effect profiles. This makes them ideal as initiating medications in the management of DP. Levodopa and dopamine agonists can be added as adjunct therapy if their effect is insufficient.
Selegiline and Rasagiline have been shown, albeit inconclusively, to offer some neuroprotective effects through reducing the need to add levodopa as a treatment adjunct. (Pålhagen et al., 2006), (Hauser et al., 2009). Dizziness, and nausea are most common adverse effects of these pharmaceutical agents.
Dopamine agonist (examples: pramipexole, ropinirole and amantadine)
These agents have been shown to provide a symptomatic benefit similar to levodopa in early disease and simultaneously shielding patients from dyskinesia and fluctuation, adverse effects more common with levodopa use (Constantinescu et al., 2007), (Rascol et al., 2000). Efficacy in advanced disease, however, is poor when used by themselves. They are usually reserved for the younger patient in good health and cognitively intact.
Adverse effects of dopamine agents include nausea, agitation impulse control disorders, confusion somnolence, orthostatic hypotension, hallucinations and depression.
Other medications
Anticholinergic agents such as trihexyphenidyl which provide relief in a good number of tremulous patients. Neuropsychiatric side effects limit their use.
Antidepressants in the form of selective serotonin reuptake inhibitors (SSRIs) and/or tricyclic antidepressants such as paroxetine and nortriptyline respectively are useful in managing PD associated depression.
Laxatives such as polyethylene glycol are expedient in the management of chronic constipation that’s associated with PD.
Antipsychotics such as quetiapine and clozapine are recommended drugs of choice in managing psychotic episodes in PD. However, it is advisable to ensure identification and reducing the dose of an offending antiparkinsonian medication is done before considering the addition of a neuroleptic.
Treatment of advanced disease
Motor fluctuations and dyskinesia are the two most problematic clinical features in advanced disease. Management requires a combination and proper titration of levodopa dose and other agents MAO-B inhibitors, dopamine agonists, Catechol-O-Methyl transferase(COMT)inhibitor).
Deep Brain Stimulation (DBS) is a surgical alternative that has shown positive outcomes in persons with PD whose symptoms are poorly responsive to pharmacological treatment. (Benabid, 2003)
Non pharmacological management
Non pharmacological management options in PD are equally as fundamental as pharmacological treatment. It encompasses
Patient and family education
PD is a chronic and progressive disorder, a prospect that is frightening. It is imperative to provide as much information about the illness to the patient and their support circles. The caretakers need to appreciate the demands associated with the illness, demands which shall increase with disease progress.
Support
The cognitive and physical burden of PD necessitates support for the patient. Caregivers, support groups composed of patients with the disease, professional help (psychologists, social workers, and counsellors) constitute this supporting framework.
Exercise and physical therapy
Mounting evidence from studies has shown exercises tailored at improving balance, flexibility and strength improve functional outcomes in persons with PD. Exercises further improve the confidence of patients and instill a sense of control over some aspects of the disease (Ridgel et al., 2009), (Ahlskog, 2011). Tai-chi training, for example, was found to be better than resistance training in improving postural stability (Li et al. 2012). Other exercises that appear helpful include swimming and brisk walks The American Academy Neurology proposes these physical therapy modalities for patients with PD (Suchowersky et al., 2006);
Treadmill training with body weight support
Balance training and high intensity resistance training
Cued exercises with visual(mirror), auditory(metronome) and tactile feedback
Active music therapy
Multidisciplinary rehabilitation with standard physical and occupational therapy components
Nutrition
Poor nutrition is a common conundrum in individuals with chronic illnesses such as PD, especially elderly patients. Depression, dysphagia, constipation and hyposmia can occur in early stages of PD, remain undetected and severely affect eating habits in patients. Studies investigating micronutrient deficiency and the role of Vitamins D, C, and E in slowing PD progression have revealed conflicting results, thus no firm conclusive recommendation currently exists (Suchowersky et al., 2006). The cause of Vitamin D deficiency, an important micronutrient in bone homeostasis, is still debatable whether it is nutritionally affiliated or associated with the disease process of PD. Weight loss and bone loss are not uncommon in this group. Investigations done by Seidl et al. (2014) show a well-balanced diet rich including servings of vegetable and fruit, omega-3 fatty acids and caffeine may offer neuroprotective effects.
Speech therapy
Hypophonia, face masking, and dysarthria make speech a major challenge in persons with PD. Slurring and facial masking further tend to make communication difficult and frustrating for both the patient and person they are talking to. Speech therapy aims to improve on phonation and loudness.
Mr. Richard was diagnosed with early stage Parkinson’s Disease, thence, Levodopa/carbidopa combination would be the ideal drug for treatment. Adjunct medication shall depend on contemporaneous complaints. However, his history of dehydration, hyponatremia and chronic osteoporosis demands attention be made to his dietary (ensuring adequate fluid, micronutrient intake) and physical attributes as well.
Conclusion
Parkinson’s Disease is another neurodegenerative disorder that invites further studies in identifying instigating factors and management protocols. The importance of clinically identifying the illness as early as possible and instituting a management plan cannot be overstressed. PD presents a multifaceted challenge to the patient, their support group, and health care provider. Often missed comorbidities such as depression on top of the more salient cardinal symptoms are a major encumbrance to the patient’s support circle, and, hasten the patient’s progression to disability or death. Developing a timely treatment plan at diagnosis helps manage the illness as best as feasible and make modifications required to acclimatize to the changes associated with the disease process.
Regarding Mr. Richard, with proper medication and, importantly, educating the caregivers, comorbidities such as dehydration, hyponatremia, and osteoporosis can be combated. Engaging specialists such as a physician, physiotherapist, nutritionist and otolaryngologist in the management plan is also required.
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Appendix
Name:
Nursing diagnosis/ Problem/Issue identified:
Goal/Aim
Interventions/Actions
Rationale/Evidence
Evaluation
Dehydration
Patient will have adequate fluid intake
Patients will show no signs of dehydration
Assess patient’s ability to take fluids
To provide information regarding factors associated with reduced fluid intake
The patient is sufficiently hydrated and fewer or no episodes of dehydration ensue.
Imbalanced nutrients intake
Patient shall maintain adequate nutritional status with use of nutritional support
Patient will show no signs of nutrient deficiency
Asses patient’s eating habits
Monitor patient’s serum levels of sodium and potassium
To acquire information on factors associated with decreased nutrient intake
To assess whether nutrient replenishment is adequate
Patients serum sodium levels return to normal level and no symptoms or signs of hyponatremia are present
Risk of fractures and injuries
Caregivers will ensure enforcement of safety precautions
Patient will gain ideal bone density for his age
Evaluate patient’s ambulation
Assess patient’s diet
Assists in developing interventions
To establish whether patient’s intake of nutrients essential for bone homeostasis is adequate.
No injuries nor fractures associated with osteoporosis.
Developed by:
