Restless legs syndrome: a case study.
Restless legs syndrome
Restless legs syndrome (Research)
|Publication:||Name: Journal of Neuroscience Nursing Publisher: American Association of Neuroscience Nurses Audience: Professional Format: Magazine/Journal Subject: Health care industry Copyright: COPYRIGHT 2003 American Association of Neuroscience Nurses ISSN: 0888-0395|
|Issue:||Date: August, 2003 Source Volume: 35 Source Issue: 4|
|Topic:||Event Code: 310 Science & research|
|Geographic:||Geographic Scope: United States Geographic Code: 1USA United States|
Abstract: Restless legs syndrome (RLS) is a sensory motor
neurological movement disorder affecting 12 million Americans. Few
healthcare providers know of the existence of this syndrome. Often
underdiagnosed and unrecognized because of comorbid conditions, RLS is a
lifelong condition with worsening symptoms. For the healthcare provider,
RLS may present with confusing symptoms. With no cure, treatment is
often unsuccessful in alleviating the discomfort associated with the
syndrome. Although RLS is a common, potentially disabling condition,
neuroscience nurses may provide interventions to assist in the
successful management of the syndrome.
Restless legs syndrome (RLS) is an unpleasant sensory motor neurological movement disorder that affects up to 15% of the U.S. population (Allen & Earley, 2001; Earley, 2003; National Institute of Neurological Disorders and Stroke [NINDS], 2001; Phillips et al., 2000; Rothdach, Trenkwalder, Haberstock, Keil, & Berger, 2000). Also known as Ekbom's Syndrome, the prevalence for women is twice that for men (Allen & Earley; Glasauer, 2001). Onset of symptoms may occur in children who are often misdiagnosed with growing pains, anxiety disorders, or attention deficit hyperactive disorder (ADHD). Up to 20% of patients suffering from insomnia have RLS, marking it as the fourth-leading cause of insomnia (Glasauer, 2001). Because RLS is not associated with a specific mortality rate and is not considered a life-threatening illness, it is often ignored and not treated seriously. At this time, no nursing journals have reported any scientific research related to RLS. This article provides an overview of RLS, including history, onset and symptoms, pathophysiology, etiology, clinical presentation, diagnosis, and treatment as groundwork for a case study of a young woman with RLS.
History of RLS
Willis, who recognized that his patients experienced the greatest torture of leaping and contracture of tendons, first identified RLS in 1685 (Allen & Earley, 2001; Chokroverty, 2003; Minnick, 2003). In 1861, Wittmaack identified RLS as a psychiatric disorder known as "anxietas tibiarum," an accepted description as a mental health illness until the 1940s when Ekbom provided a current definition of the syndrome (Allen & Earley). Most recently, RLS has received recognition because of its effect on the gerontological population, which suffers from it the most, affecting quality-of-life issues. Because of the unknown cause and lack of a cure for RLS, NINDS (2001) has identified RLS as a condition that requires scientific, clinical investigations.
RLS researchers from the National Institutes of Health (NIH) formed the International Study Group on Restless legs syndrome (ISG-RLS) and have made progress in the research agenda for RLS, including a recent revision of the criteria for diagnosis (Allen et al., 2003). According to ISG-RLS, criteria required for a diagnosis of RLS include a distressing urge to move the legs accompanied by sensations in the legs that are brought on by rest, relieved with moving or walking, and worsening at night or evening (Earley, 2003).
Onset and Symptoms
The onset of RLS symptoms may begin to occur at any age. However, most patients are severely affected at middle age or older. Forty percent of patients with RLS report onset of symptoms before the age of 20 years (Walters, Picchietti, Ehrenberg, & Wagner, 1994; Walters et al., 1996). Almost 10% of people over the age of 65 years in the United States are diagnosed with RLS; older women have a prevalence of 13.9% compared to 6.1% for older men (Rothdach et al., 2000). Older patients experience symptoms more frequently and for extended periods of time while remission may occur intermittently.
Symptoms of RLS are described as mild, moderate, and severe in relation to severity and duration. Mild RLS, a rare occurrence, produces mild disruption of sleep with little distress. In moderate cases, symptoms occur up to two times a week, resulting in significant delay of sleep onset with disruption of daytime functioning. In severe cases, symptoms occur three to five times a week and result in interruption of sleep and impairment of daytime functioning (Allen et al., 2003; Earley, 2003). Of RLS patients, 94% experience sleep disturbances (We Move, 2002).
Few healthcare providers know of the existence of this syndrome and often misdiagnose RLS as leg cramps, peripheral neuropathy, varicose veins, intermittent claudication, positional discomfort, and neuroleptic-induced akathisia. Current treatment consists of pharmacological efforts that work for a short period of time, cause negative side effects, or do not work at all.
The proposed pathophysiology of RLS can be roughly divided into three areas: (a) localization of areas in the central nervous system, (b) neurotransmitter systems, and (c) iron metabolism (Allen & Earley, 2001).
Central Nervous System Localization
Because treatment of RLS involves the use of dopaminergic agents, this indicates that the RLS pathology involves the central nervous system, not the peripheral nervous system (Entezari-Taher et al., 1999; Tergau, Wischer, & Paulus, 1999). Increased activation in the thalamus and cerebellum has been associated with RLS sensations without movements and additional increases in activation for RLS sensations with movements; however, there is no increase in activation of cortical areas (Bucher, Seelos, Oertel, Reiser, & Trenkwalder, 1997; Parker & Rye, 2002). Activation of normal central nervous system pacemakers has been associated with the spinal cord (Parker & Rye). Additional evidence suggests that centrally active dopamine antagonists can cause symptoms of RLS while peripherally active dopaminergic antagonists do not.
Although there is no absolute theory about the cause of RLS, it is known that low doses of dopamine agonists can be used to provide, at least temporarily, almost complete relief of symptoms. This evidence provides support for the theory that RLS may involve abnormalities in dopaminergic function (Chokroverty, 2003; Minnick, 2003; Parker & Rye, 2002).
Other neurotransmitters also have gained attention in the treatment of RLS. Hypocretin, a neuropeptide involved in the physiology of sleep, increases arousal and interacts with activity levels and the dopamine system. Patients have increases in CSF hypocretin in the early onset of the syndrome, correlated with low levels of iron (Allen, Mignot, Ripley, Nishino, & Earley, 2002). Decreased serotonin also may be of interest in RLS patients because tryptophan, the precursor to serotonin, is also associated with dopamine production (Earley, Allen, Beard, & Connor, 2000).
Iron deficiency is identified in the three reversible forms of RLS--end-stage renal disease (ESRD), iron deficiency anemia, and pregnancy. This occurrence suggests that iron insufficiency may be a significant characteristic of the pathophysiology of RLS. Serum ferritin levels have been inversely associated with RLS severity (Aul, Davis, & Rodnitzky, 1998; O'Keeffe, Gavin, & Lavan, 1994; Sun, Chen, Ho, Earley, & Allen, 1998). Several studies have suggested that RLS pathophysiology involves iron metabolism, particularly iron in the brain, and that brain iron deficiency may cause RLS (Allen, Barker, Wehrl, Song, & Earley, 2001; Benz, Pressman, Hovick, & Peterson, 1999; Berger et al., 2002; Earley, Connor, et al., 2000). Iron has a direct influence on dopamine because iron is a co-factor for tyrosine, the enzyme necessary to metabolize dopamine. An iron deficiency has been associated with disruption of the dopaminergic system (Earley, Allen, et al., 2000).
Etiology of RLS
The role of genetics is involved in primary or idiopathic RLS and occurs with no comorbid conditions. In approximately 50% of RLS cases studied by Desautels, Turecki, Montplaisir, Sequiera, et al. (2001), a family history is seen, suggesting a genetic component to the disease. This cohort of patients will present with symptoms earlier in life and have a slower progression of the syndrome. Significant findings of family clustering are seen in primary RLS (Ondo, Vuong, & Wang, 2000; Trenkwalder, Seidel, Gasser, & Oertel, 1996). In females, an excess of the monoamine oxidase A (MAOA) gene contributes to the susceptibility of RLS (Desautels, Turecki, Montplaisir, Brisebois, et al., 2002). It may be that patients with late age of onset of symptoms are less likely to have affected family members than patients with primary RLS (Ondo & Jankovic, 1996). No genetic evidence of the involvement of the dopaminergic system has been found (Desautels, Turecki, Montplaisir, Ftouhi-Paquin, et al., 2001).
Secondary causes of RLS are associated with conditions such as iron deficiency anemia, pregnancy, and ESRD. This cohort of patients with RLS tends to develop severe symptoms rapidly (Glasauer, 2001). Complete recovery is seen in RLS after a successful kidney transplant, correction of iron deficiency anemia, and childbirth (Allen & Earley, 2001). Other secondary causes of RLS have been found in heart disease, neuropathy, rheumatoid arthritis, diabetes mellitus, fibromyalgia, folate and magnesium deficiencies, stress, and adverse reactions to medications such as tricyclic antidepressants, anti-nausea, selective serotonin reuptake inhibitors (SSRIs), and antihistamines (Ohayon & Roth, 2002). Treatment of the secondary conditions will usually cause RLS symptoms to diminish or disappear.
The neuroscience nurse may see the client present with the primary features of RLS. Features include dysesthesias, described as burning, creeping, tugging, pulling, drawing, insects crawling inside the legs, wormy, boring, tingling, pins and needles, prickly, electrical, and painful legs. These sensations occur deep inside the leg, between the knee and ankle, also in the feet, thighs, arms, and hands. The sensations can occur unilaterally or bilaterally and may include sudden muscle jerks (NINDS, 2001). Unfortunately, the symptoms worsen at rest and only go away with movement of the extremities that may include rocking, shaking, stretching, marching, bending, or bicycling. The dysesthesia is one of two neurological disorders, the other being doparesponsive dystonia, that has a circadian pattern, occurring during the evening or early part of the night (Earley, Allen et al., 2000).
More than 80% of people with RLS also experience periodic limb movement disorder (PLMD), also known as nocturnal myoclonus. PLMD involves involuntary leg twitch or jerking during sleep that occurs every 10-60 seconds, often throughout the night, which causes repeated awakenings and disturbed sleep. These movements are involuntary; and often patients have no control over them, resulting in exhaustion and daytime fatigue affecting employment, personal relations, and activities of daily living (ADLs). Also, concentration, memory impairment, and inability to complete tasks are often problems due to lack of sleep (Allen & Earley, 2001).
The clinical diagnosis of RLS is difficult to make because there is no single diagnostic test for RLS. The symptoms are self-reported, and an accurate description of symptoms is difficult to obtain. Because nocturnal symptoms are often not witnessed by the healthcare provider, documentation and diagnosis are difficult (Evidente & Adler, 1999).
RLS is diagnosed by a thorough patient history, including specific questions about symptoms (e.g., duration, frequency, and intensity), sleep patterns, and sleep history. Because RLS may affect so many aspects of the patient's life, the interference of ADLs and daily functioning should be evaluated. To rule out treatable secondary conditions that contribute to RLS, laboratory tests should be performed (Table 1). Serum ferritin levels of < 50 [micro]g/ml are suggestive of anemia and associated with increased severity of RLS symptoms (Earley, 2003). Serum chemistries should rule out renal disease (uremia) and diabetes.
Many medications can induce the symptoms of RLS. A thorough review of pharmacological medications being taken is an essential component of the health history. Ironically, some of the medications used to treat RLS may also exacerbate the symptoms. Drugs that may exacerbate the symptoms of RLS include diphenhydramine, phenytoin, lithium, prochlorperazine, metoclopramide, haloperidol, phenothiazine derivatives (i.e., chlorpromazine, amitriptyline), fluoxetine, and sertraline. These and any medications that worsen the symptoms of RLS should be discontinued (National Heart, Lung, and Blood Institute Working Group of Restless Legs Syndrome, 2000).
Neurological examinations and sleep disorder evaluations are considered for diagnostic work-up of RLS. Spinal cord and peripheral nerve function including peripheral nerve lesion testing, electromyography (EMG), and nerve conduction velocity tests should be integrated in the neurological examination. To rule out vascular disorders, a Doppler sonogram study should be completed (National Heart, Lung, and Blood Institute, 2000).
If all conditions are ruled out and negative results of these tests are found, then the healthcare provider may be led to the diagnosis of RLS (Allen & Earley, 2001). One diagnostic measure, the Suggested Immobilization Test (SIT), records leg activity in RLS patients for 60 minutes. SIT requires the patient to recline in bed at a 45-degree angle with legs stretched out. The patient is instructed not to move and to stay awake. The movement of legs is measured using electromyography while an EEG is used to ensure the patient remains awake (Allen & Earley, 2001). Although not routinely performed, polysomnography (PSG) may document sleep disturbances but is not indicated in the work-up of RLS because diagnosis is determined by history and clinical findings (Michaud, Paquet, Lavigne, Desautels, & Montplaisir, 2002).
Self-Reported Measures Used for RLS
Measures related to fatigue, sleep, depression, and quality of life (QOL) may be used to identify the outcomes of patients with RLS. Specific measurements related to patients with RLS that measure severity of symptoms and QOL have been developed. The following measures were used in the case study presented.
Severity of Symptoms
The International RLS rating scale was developed by the International RLS Study Group. Before answering the survey; patients are asked to verify that they meet the criteria for RLS, including dysthesia, motor restlessness, worsening symptoms at rest, and worsening symptoms at night. The patient is asked to rate 10 symptom factors on a scale of 0 (no symptoms) to 4 (most severe symptomatology). Five of these items pertain to symptom frequency and intensity, and the other five items address the effect of the symptom on ADLs and sleep. At the end of the 10 questions, the patient is asked to rate the symptom severity for the preceding week, overall: mild, moderate, severe, or very severe. Cronbach alpha has been reported at 0.93 and 0.95 with high levels of inter-examiner reliability, test-retest reliability, and convergent validity (The International Restless Legs Syndrome Study Group, 2003).
The RLS-QOL instrument was designed to assess QOL in patients with RLS. The questionnaire consists of 17 items that identify daily function, social function, sleep quality, and emotional well-being. On a scale of 1-9, patients are asked to rate the effect of RLS on their life during the last month. Scoring is done by each of the four functions with specific directions for each area. Scores range from 0 to 100. Cronbach alpha has been reported at 0.91 with a test-retest stability of 0.88 (Atkinson, et al., in press).
The Center for Epidemiologic Studies Depression Scale (CES-D) is a self-report measure of depressive symptomatology developed for the nonpsychiatric population aged 18 and older and has a reported reliability of 0.90 (Radloff, 1977). CES-D provides an index of cognitive, affective, and behavior depressive features and the frequency the symptoms have occurred. Major components include depressed mood, feelings of guilt and worthlessness, psychomotor retardation, loss of appetite, and sleep disturbances, which indicate current levels of functioning. CES-D is a 20-item questionnaire assessing frequency and duration of depressive symptoms in the last week. A score of 0-15 indicates no depression; 16-20, mild depression; 21-30, moderate depression; and 31 or higher, severe depression (Devins & Orme, 1985).
Epworth Sleepiness Scale
The Epworth Sleepiness Scale (ESS) is a self-administered, 8-item questionnaire that measures the general level of daytime sleepiness in adults (Johns, 1991, 1992). Participants are asked to rate the chances that they would doze off or fall asleep in eight situations encountered in daily life. Using a scale of 0 (no chance of dozing) to 3 (high chance of dozing), subjects can determine their daytime sleepiness using a mean score. Subjects with PLMD have an average score of 9.2. Test-retest reliability was highly correlated (r = 0.82). Cronbach alpha has been reported at 0.88 (Johns 1991, 1992).
The goal for treatment in RLS is to alleviate symptoms that are irritating and cause sleep disturbances and discomfort. Secondary conditions, specifically ESRD, pregnancy, iron deficiency, diabetes, and peripheral neuropathy, should be treated and controlled to successfully treat the symptoms of RLS. The selection of treatment modalities may depend on the (a) age of the patient; (b) severity, duration, and frequency of symptoms; and (c) comorbid illnesses including pregnancy and renal failure. Treatment modalities may include one or a combination of health promotion behaviors, pharmacological interventions, and complementary and alternative practices and products.
Health Promotion Behaviors
Major improvements can be seen in patients with RLS through health promotion behaviors. The benefits of lifestyle changes include increased QOL and more cost-effective health care. Health promotion behaviors include exercise, nutrition, social support, and stress management. For patients with mild symptoms of RLS, lifestyle changes often may alleviate many symptoms. A regular exercise regime consisting of mild to moderate exercise may be beneficial. For RLS patients, exercise may be done before bedtime to eliminate restlessness that may be felt at this time. However, if this keeps the patient awake, exercise is recommended 6 hours before bedtime. Smoking cessation is also encouraged. Health behaviors also include a regular sleep schedule, which includes going to bed and waking up at the same time every day. Nutrition should include a well-balanced diet that does not include substances that can interrupt sleep, such as caffeine, alcohol, or sugar. Because RLS patients often feel restless and anxious, a stress management program may be beneficial.
Whether the diagnosis is primary or secondary RLS, the medical treatment choices are the same, except when the underlying conditions can be treated (such as ESRD, iron deficiency, and pregnancy). Primary treatment using pharmacological agents may be started if health promotion behaviors have not improved symptoms, if appropriate treatment of secondary exacerbating conditions has not provided relief, if symptoms have interfered with QOL, and if the patient needs more progressive pharmacological treatments.
The major classes of drugs used to treat patients are dopamine antagonists (precursors), dopamine agonists (receptors), benzodiazepines, opioids, and other antiepileptics (Allen & Earley, 2001; Glasauer, 2001; We Move, 2002). These drugs are very effective given at lower doses rather than at prescribed doses for other medical conditions, such as Parkinson's disease.
Dopamine antagonists (precursors) enhance dopamine levels by increasing the availability at the synaptic junctions. These drugs may alleviate all major symptoms of the disorder including motor restlessness, paresthesias, and periodic limb movements in sleep (PLMS). Sleep impairments and fatigue are also minimized. If patients do not respond to these drugs, RLS is considered to be a misdiagnosis.
Levodopa (Sinemet) has long been the drug used in the initial treatment of RLS; however, rebound and augmentation may occur. Rebound, the reappearance of symptoms 2-6 hours after dosing, occurs in 25% of patients. Augmentation, the worsening of symptoms in the course of the therapy, occurs in up to 82% of patient being treated with levodopa, limiting the long-term usefulness of this drug (Comella, 2002). Augmentation is seen in dopamine precursors as well as dopamine agonists (National Heart, Lung, and Blood Institute, 2000). Combination treatment of regular-release and sustained-released levodopa has shown to be effective in controlling negative side effects of monotherapy with regular-release levodopa (Collado-Seidel et al., 1999).
Dopamine agonists produce dopamine-like effects by binding to dopamine receptors. These agonists are recommended for patients with mild to severe symptoms that threaten QOL. They are effective in alleviating subjective and objective features of RLS, are well tolerated, and have few side effects such as rebound and augmentation, which are seen more frequently with dopamine-precursor treatment. These medications include bromocriptine mesylate, pergolide mesylate, pramipexole dihydrochloride, and ropinirole hydrochloride and are more often being used as a first choice in treatment.
Depressed CNS activity in patients with RLS can result in the improvement of sleep and reduced number of arousals in the night. Benzodiazepines act upon the hypothalamic, thalamic, and limbic regions, depressing CNS activity. The benzodiazepines include clonazepam, temazepam, diazepam, and triazolam.
Opioids have been effective in lessening the symptoms of paresthesias and dysesthesias, motor restlessness, and sleep disturbances associated with RLS. The stimulation of opiate receptors serves to decrease the release of neurotransmitters, producing analgesic effects in the CNS. These opioids include codeine, oxycodone hydrochloride, and methadone hydrochloride. Caution regarding addiction to opioids should be considered.
Anti-epileptic drugs are used in RLS to inhibit neuronal activity for abnormally increased CNS activity. These drugs include carbamazepine and gabapentin.
Other medications may be effective in relieving symptoms associated with RLS, including clonidine, baclofen, tramadol, and zolpidem. However, no controlled studies have been conducted to evaluate the effectiveness of these drugs in RLS. The complete mechanism of action of these drugs in RLS is not understood.
Complementary and Alternative Practices and Products
Although conventional treatments have not been successful in controlling the symptoms of RLS, many patients have reported the use of complementary and alternative practices and products (CAPPs). These include nutritional supplements to correct deficiencies including multivitamins, electrolytes, folate, vitamin E, and magnesium. Oral administration of iron supplements generally corrects iron deficiency and reduces RLS symptoms (Benz et al., 1999; O'Keeffe et al., 1994; Sun et al., 1998).
Other CAPPs that may be helpful in alleviating symptoms include acupuncture, massage therapy, cold or heat compresses, vibration, transcutaneous electrical nerve stimulation (TENS), and mild exercise. Behavioral modification includes learning coping strategies such as relaxation, hypnosis, biofeedback, and guided imagery. Although the research on the effectiveness of CAPPS is inconclusive, they remain helpful to RLS patients. The RLS Foundation (2002) provides a list of support groups across the country to help in the management of the RLS symptoms.
"Meg" is a 27-year-old married, Caucasian female with a history of RLS. She reports that she has tried a variety of medications (e.g., clonazepam, levodopa, citalopram, trazodone, pergolide, cyclobenzaprine) to alleviate her symptoms of RLS. She complains of difficulty sleeping due to her restless legs. She has severe pain in her legs when the weather turns cold. She has been to a variety of physicians who have been unable to help her. Due to her own persistence, Meg did her own online research and discovered she had the symptoms of RLS. She has finally found a physician at a sleep disorder clinic who has helped her in the treatment of her disease. Comorbidities include asthma, PLMD, upper airway resistance syndrome, and auto-immune thyroiditis.
Meg is a college graduate and works full time as a customer service representative. Her income is between $40,000 and $50,000 per year. The syndrome has not affected her marital status or employment status at this time. Both of her parents are still alive, but divorced, and her mother is remarried. Meg does not have any natural siblings. A family history reveals that her father's sister and a cousin both have RLS. She suspects that her mother may have it but has never been diagnosed. Meg has never been pregnant so cannot report if symptoms worsened during pregnancy.
Meg has the urge to move more often when the temperature drops into the 30s or when she first goes to bed, with the sensations occurring in her legs, feet, or back. She must continuously move her legs, as if to generate heat in some way. When a passenger in a car for over one hour, she begins to get very restless, feeling as if she must flap her arms, do jumping jacks, or anything to move. If she sits still, lies down to read, or sits at the computer, she begins to feel the "creepies."
She is not sure whether the symptoms really worsen at night as much as they worsen with rest. She does have PLMD. Her husband says it is much better now but states she kicked all the time, despite the fact that Meg insists she did not kick. Meg reports that she used to wake up with bruises on her legs, not sure where she got them. She has awakened during the night with her arms flailing. She reports she has pain in her joints and legs but does not think it is related to her RLS. She has been diagnosed with auto-immune thyroiditis (Hashimoto's thyroiditis), a chronic inflammatory glandular autoimmune disease of the thyroid with symptoms of fatigue, weight gain, cold intolerance, aching, or pain in muscles and joints with increased muscle cramping at night, high cholesterol levels, shortness of breath, menstrual irregularities, and depression.
Her symptoms of RLS started at age 6 when she was told she had "growing pains." She remembers always kicking and thrashing around in her sleep as far back as early childhood but is not sure of the exact onset of her RLS. She says her mother never wanted her to sleep with her as a child because Meg would kick so much. She used to get a "tingling" sensation in her heel that became quite uncomfortable, which she never mentioned to anyone.
Meg was not formally diagnosed until 1998. At this time, she went to her doctor and basically told him that she had RLS. For the previous 4 years before this, she had moved three times and had several doctors who were knowledgeable, yet unable to diagnose her accurately.
Meg's symptoms usually do not last more than an hour. She describes a trip to Manhattan with a friend.
With her medications, severe symptoms like this do not occur that often. They do occur after strenuous activity or long periods of standing/walking. On a scale of 1-10, her symptoms usually rank 2-3 except after strenuous activity when she ranks the pain between 8 and 10.
Meg cannot remember the last time she slept through the night. Each night, her RLS usually keeps her from being able to fall asleep, until she can get warm, about 15-20 minutes, in most cases. She says once she is asleep, she awakens at least two times during the night, unable to get back to sleep within an hour. She cannot sleep before midnight and wakes up around 6:45 am on weekdays. She reports the quality of her sleep is very poor. On weekends, she tries to sleep late to catch up on her sleep. Her bedtime ritual takes 45 minutes and includes taking out the dog, doing evening care (e.g., bathroom, change, brushing teeth, taking medication), and then going to bed. Her husband reports she is doing better while asleep. He is happy they are now sleeping in the same room and in the same bed. Previously, they had slept in separate bedrooms because her kicking was so bad. She says she is very tired during the day because of her late sleep time and poor quality of sleep. She feels like she is not getting enough sleep.
Health promotion behaviors. Meg limits her caffeine intake, drinking 1-2 cups of tea or cola a month. She does eat chocolate and has that at least a few times a month. She does not smoke. She drinks alcohol maybe three times a year, drinking up to two drinks at a time. She is 50 pounds overweight and does not exercise on a regular basis. She follows a regular sleep routine. She does not follow a specific diet but does take a multivitamin daily.
Pharmacological treatment. Meg has been on a variety of medications that were discontinued either because they were ineffective or caused adverse side effects. The first drug was clonazepam, which she took for about a month but did not relieve the symptoms. The next medication was levodopa. During a 4-month period, the dosage was doubled to twice a day. Citalopram was prescribed but did not help the symptoms either. At this time, the levodopa began to worsen her RLS symptoms during the day as a result of augmentation. She stopped taking the citalopram and levodopa abruptly with side effects of weakness, dizziness, nausea, and vomiting. She was told that antidepressants were the only way to stop her PLMD, so was started on trazodone, 25 mg at bedtime. She stayed on trazodone for more than a 1 year. At this time she moved to a new location and found a new doctor who changed her medication to pergolide, which resulted in nausea and vomiting. Her doctor encouraged her to continue taking it, but she could not. So he switched her to pramipexole, which she has used for more than 2 years on varying dosages. She moved again and went to a new doctor who specialized in sleep disorders. He decreased her pramipexole from 1 mg to 0.125 mg/night, which caused her to get very sick with nausea and vomiting. She went back up to 0.375 mg and added cyclobenzaprine 10 mg/night. Cyclobenzaprine caused insomnia for a week, so she stopped taking it. She is currently taking norgestimate daily, pramipexole 0.375 mg every night, temazepam 15 mg every night, and albuterol prn.
Of all the medications she has been on, she believes the pramipexole has produced the best control of her symptoms with the least side effects. She has also been able to be on the drug for almost 2 years.
CAPPs. Meg has tried taking folate supplements, but they didn't seem to help much. She takes a multivitamin daily but is not sure whether it is helping the RLS symptoms. She has used self-massage to her legs but has not ever had a professional massage. She is interested in trying acupuncture and yoga.
Meg presents with severe pain as a result of winter temperatures in the 20s. She reports her legs are hurting, The pain feels like the bone where my legs connect with my body. My knees are starting to hurt also." She says she has had this type of pain since at least high school. The physicians call it "phantom pain" because no one has ever been able to tell her what the pain was from.
On the RLS rating scale, Meg scored 16, indicating moderate severity of her symptoms. Using the RLS-QOL Instrument, Meg scored 87.5 on social functioning, 70.83 on daily functioning, 44.44 on sleep quality, and 54.44 on well-being. The instrument's scores range from 0 to 100 with 100 being the least disturbance of QOL. RLS is affecting her sleep quality and well-being more than her social and daily functioning. She scored a 21 on the CES-D scale, indicating moderate depression. She scored a 10 on ESS, indicating a high sleepiness level throughout the day.
Meg was tested for anemia, which included serum ferritin, complete blood cell count, serum iron, total iron-binding capacity, and iron saturation; all were within normal limits. Fasting glucose, sodium, potassium, chloride, carbon dioxide, calcium, magnesium, blood urea nitrogen (BUN), and creatinine also were within normal limits.
She has been tested for Lyme disease and rheumatoid arthritis several times with negative results. She recently was evaluated for an enlarged goiter and was diagnosed with autoimmune thyroiditis. She has a history of thyroid disease in her family, so these levels are checked yearly.
Previous EMG and nerve conduction velocity tests were negative. A vascular examination ruled out any vascular disorders through Doppler sonogram studies. Previous sleep studies revealed sleep-wake states that had a profound effect on her leg movement characteristics.
The patient with RLS who presents to the healthcare clinic may challenge the neuroscience nurse. It is a priority to thoroughly assess a person for RLS, when he or she complains of fatigue or inability to sleep due to restlessness. A major role of the nurse is to listen with empathy and compassion, because patients with RLS often are frustrated with ineffective treatments or feelings of being ignored. A thorough sleep history will provide the neuroscience nurse with clues related to cause of sleep disorder All healthcare providers should note that many of the medications used to prepare patients for procedures or surgeries may cause tremendous discomfort to the RLS patient, specifically diphenhydramine and promethazine, to name a few. Referrals to a neurologist or sleep disorder clinic may be recommended. Three major nursing diagnoses, including sleep, depression, and fatigue (see Fig 1), can be used to help manage symptoms of the RLS patient.
Nursing interventions for Meg included a thorough assessment of her sleep patterns. She was encouraged to maintain a regular sleep pattern, keeping a sleep journal to record time of sleep, time of awakening, number of times awakened during night, and quality of sleep. Also, things that promote relaxation and sleep were reviewed, such as hot baths, massage, music, or reading. She was started on carbamazepine to take at bedtime to help with sleep. Since she is not on any antidepressants and her CES-D score indicated moderate depression, she was started on citalopram, an antidepressant. She was encouraged to begin a moderate exercise program to assist in weight loss and management of symptoms. A nutritional consult was scheduled to promote a healthy diet. She was informed of organizations that provide information on RLS (see Resource List). Currently, she attends a support group through the RLS Foundation.
The prognosis of RLS is a lifelong condition for which there is no cure. Remissions do occur, but symptoms will usually reappear. Because RLS is a self-reported syndrome and symptoms may not occur daily, it is difficult to perform research in this population. The NINDS (2001) has the responsibility to conduct research in RLS. The goals for the institute are to find improved methods of diagnosing and treating the syndrome and to discover ways to prevent it. Specific research in the outcomes of interventions for older people is of primary concern.
Meg reported to the healthcare provider after a month. She had lost 5 pounds and was walking half a mile 3 times a week. She had kept her sleep diary and discovered activities that did not promote sleep for her at bedtime such as watching television. The addition of carbamazepine and citalopram made a big improvement on her sleep quality and depression. She still reported some daytime sleepiness and fatigue, but it was better than before. Repeat scores were an 11 on the RLS rating scale on severity of her symptoms. Using the RLS-QOL instrument, Meg scored 90.5 on social functioning and 80 on daily functioning with the biggest improvements seen in sleep quality (62) and well-being (68). She scored a 16 on the CES-D, indicating minimal depression and a 7 on the ESS. All her repeat scores showed improvement.
RLS is a common, potentially disabling condition that affects a significant portion of the general population. Lack of recognition from under- or misdiagnosis has led to little research being done in the area to validate effective treatment modalities. Neuroscience nurses should be aware of this syndrome and be prepared to assist in a thorough assessment including sleep evaluation, fatigue, depression, and pain.
This manuscript was developed with support from grant awards T32-AT-00052 CAM Research Training Program funded by the National Center for Complementary and Alternative Medicine, National Institutes of Health.
Allen, R., Barker, P., Wehrl, F., Song, H., &Earley, C. (2001). MRI measurement of brain iron in patients with restless legs syndrome. Neurology, 56, 263-265.
Allen, R., &Earley, C. (2001). Restless legs syndrome: A review of clinical and pathophysiologic features. Journal of Clinical Neurophysiology, 18(2), 128--147.
Allen, R., Picchiett, D., Hening, W.A., Trenkwalder, C., Walters, A.S., & Montplaisi, J. (2003). Restless legs syndrome: Diagnostic criteria, special considerations, and epidemiology. A report from the restless legs syndrome diagnosis and epidemiology workshop at the National Institutes of Health. Sleep Medicine, 4, 101-119.
Allen, R., Mignot, E., Ripley, B., Nishino, S., &Earley, C. (2002). Increased CSF hypocretin-1 (Orexin-A) in restless legs syndrome. Neurology; 59, 639-641.
Atkinson, M., Allen, R., DuChane, J., Murray, C., Kushida, C., Roth, T., & the RLS Quality of Life Consortium. (in press). Validation of the Restless Legs Syndrome-Quality of Life Instrument (RLS-QLI). Quality of Life Research.
Aul, E., Davis, B., & Rodnitzky, R. (1998). The importance of formal serum iron studies in the assessment of restless legs syndrome. Neurology, 51, 912.
Benz, R., Pressman, M., Hovick, E., & Peterson, D. (1999). A preliminary study of the effects of correction of anemia with recombinant human erythropoietin therapy on sleep, sleep disorders, and day-time sleepiness in hemodialysis patients (The SLEEPO study). American Journal of Kidney Disease, 34, 1089-1095.
Berger, K., von Eckardstein, A., Trenkwalder, C., Rothdach, A., Junker, R., & Weiland, S. (2002). Iron metabolism and the risk of restless legs syndrome in an elderly general population--The MEMO study. Journal of Neurology, 249, 1195-1199.
Bucher, S., Seelos, K., Oertel, W., Reiser, M., & Trenkwalder, C. (1997). Cerebral generators involved in the pathogenesis of the restless legs syndrome. Annals of Neurology, 41, 639-4545.
Chokroverty, S. (2003). Editor's corner: Restless legs syndrome, a common disease uncommonly diagnosed. Sleep Medicine, 4, 91-93.
Collado-Seidel, V., Kazenwadel, J., Wetter, T., Kohnen, R., Winkelmann, J., Selzer, R., et al. (1999). A controlled study of additional sr-L-dopa in L-dopa-responsive restless legs syndrome with late night symptoms. Neurology, 52, 285-290.
Comella, C. (2002). Restless legs syndrome: Treatment with dopaminergic agents. Neurology, 58, S87-S92.
Desautels, A., Turecki, G., Montplaisir, J., Brisebois, K., Sequeira, A., Adam, B., et al. (2002). Evidence for a genetic association between monoamine oxidase A and restless legs syndrome. Neurology 59, 215-219.
Desautels, A., Turecki, G., Montplaisir, J., Ftouhi-Paquin, N., Michaud, M., Chouinard, V., et al. (2002). Dopaminergic neurotransmission and restless legs syndrome: A genetic association analysis. Neurology, 57, 1304-1306.
Desautels, A., Turecki, G., Montplaisir, J., Sequeira, A., Verner, A., & Rouleau, G, (2002). Identification of a major susceptibility locus for restless legs syndrome on chromosome 12q. American Journal of Human Genetics, 69, 1266--1270.
Devins, G., & Orme, C. (1985). Test Critiques II. Kansas City, MO:Test Corporation of America.
Earley, C. (2003). Restless legs syndrome. The New England Journal of Medicine, 348(21), 2103-2109.
Earley, C., Allen, R., Beard, J., & Connor, J. (2000). Insight into the pathophysiology of restless legs syndrome. Journal of Neuroscience Research, 62, 623--628.
Earley, C., Connor, J., Beard, J., Malecki, E., Epstein, D., & Allen, R. (2000). Abnormalities in CSF concentrations of ferritin and transferrin in restless legs syndrome. Neurology, 54, 1698-1700.
Entezari-Taher, M., Singleton, J., Jones, C., Meekins, G., Petajan, J., & Smith, A. (1999). Changes in excitability of motor cortical circuitry in primary restless legs syndrome. Neurology, 53, 1201-1205.
Evidente, V., & Adler, C. (1999). How to help patients with restless legs syndrome. Postgraduate Medicine, 105(3). Retrieved September 10, 2002, from http://www.postgradmed.com/issues/1999/03_99/evidence.htm
Glasauer, F. (2001). Restless leg syndrome. Spinal Cord, 39, 125-133.
International Restless Legs Syndrome Study Group. (2003). Validation of the International Restless Legs Syndrome Study Group rating scale for restless legs syndrome. Sleep Medicine, 4, 121-132.
Johns, M. (1991). A new method for measuring daytime sleepiness: The Epworth Sleepiness Scale. Sleep, 14, 540-545.
Johns, M. (1992). Reliability and factor analysis of the Epworth Sleepiness Scale. Sleep, 15, 376--381.
Michaud, M., Paquet, J., Lavigne, G., Desautels, A., & Montplaisir, J. (2002). Sleep laboratory diagnosis of restless legs syndrome. European Neurology, 48, 108-113.
Minnick, M.E. (2003). Restless legs syndrome: Recognition and management in primary care. Advance for Nurse Practitioners, 11(3), 71-76.
National Heart, Lung, and Blood Institute Working Group on Restless Legs Syndrome. (2000). Restless legs syndrome: Detection and management in primary care. American Family Physician. Retrieved September 10, 2002, from http://www.aafp.org/afp/20000701/108.html
National Institute of Neurological Disorders and Stroke (NINDS). (2001). NINDS Restless Legs Information Page. Retrieved September 10, 2002, from http://www.ninds.nih.gov/health and_medical/disorders/restlessdoc.htm
O'Keeffe, S., Gavin, K., & Lavan, J. (1994). Iron status and restless legs syndrome in the elderly. Aging, 23, 200-203.
Ohayon, M., & Roth, T. (2002). Prevalence of restless legs syndrome and periodic limb movement disorder in the general population. Journal of Psychosomatic Research, 53, 547-554.
Ondo, W., & Jankovic, J. (1996). Restless legs syndrome: Clinicoetiologic correlates. Neurology, 47, 1435-1441.
Ondo, W., Vuong, K., & Wang, Q. (2000). Restless legs syndrome in monozygotic twins: Clinical correlates. Neurology, 55, 1404-1406.
Parker, K., & Rye, D. (2002). Restless legs syndrome and periodic limb movement disorder. The Nursing Clinics of North America, 37, 655-673.
Phillips, B., Young, T., Finn, L., Asher, K., Henning, A., & Purvis, C. (2000). Epidemiology of restless legs syndrome in adults. Archives of Internal Medicine, 160, 2137-2141.
Radloff, L. (1977). The CES-D scale: A new self-report depression scale for research in the general population. Applied Psychological Measurement, 1, 385-401.
Rothdach, A., Trenkwalder, C., Haberstock, J., Keil, U., & Berger, K. (2000). Prevalence and risk factors of RLS in the elderly population: The MEMO Study. Neurology, 54, 1064-1068.
Sun, E., Chen, C., Ho, G., Earley, C., & Allen, R. (1998). Iron and the restless legs syndrome. Sleep, 21, 371-377.
Tergau, F., Wischer, S., & Paulus, W. (1999) Motor system excitability in patients with restless legs syndrome. Neurology, 52, 1060-1063.
Trenkwalder, C., Seidel, V., Gasser, T., & Oertel, W. (1996). Clinical symptoms and possible anticipation in a large kindred of familial restless legs syndrome. Moving Disorders, 11, 389-394.
Walters, A., Hickey, K., Maltzman, J., Verrico, T., Joseph, D., Hening, W., et al. (1996). A questionnaire study of 138 patients with restless legs syndrome: The 'Night-Walkers' survey. Neurology, 46, 92-95.
Walters, A., Picchietti, D., Ehrenberg, B., & Wagner, M. (1994). Restless legs syndrome in childhood and adolescence. Pediatric Neurology. 11, 241-245.
We Move. (2002). Restless Legs Syndrome: Diagnosis and evaluation. Retrieved September 11, 2002, from http://www.wemove.org/rls_mde.html
Restless Legs Syndrome Foundation www.rls.org
National Institute of Neurological Disorders and Stroke www.ninds.nih.gov
WE MOVE: Worldwide Education & Awareness for Movement Disorders www.wemove.org/rls.html
Questions or comments about this article may be directed to: Norma Cuellar DSN RN CCRN, by phone at 434/924-0446 or by e-mail at Ngc2n@virginia.edu. She is a postdoctoral fellow at the University of Virginia, School of Nursing Center for the Study of Complementary and Alternative Therapies, Charlottesville, VA.
We walked a large portion of the city and my feet/legs were killing me from wearing the wrong shoes. On the train ride back to her house (which lasted an hour) I thought I was going to tear myself apart because it was so unbearable. It lasted about 2 hours, when I could finally take my medicine, which calmed it. I wanted to run the length of the train, screaming and ripping my hair out!
"I hate the cold, because that's when the pain is the worst. Rofecoxib works wonders for it, but the doctors won't prescribe it for me. Besides, I'm on enough meds as it is, I don't want to have to be on more."
Fig 1. RLS Interventions for Healthcare Providers Sleep Follow a regular sleep routine, keep a sleep diary Concentrate on sleeping later in the circadian cycle Receive a massage before going to sleep Perform tasks that engage mind during sedentary periods Take hot baths Control pain or discomfort Use relaxation techniques, hypnosis, biofeedback, and guided imagery Provide music therapy Depression Engage in regular moderate exercise program Use interpersonal therapy or group therapy for emotional support Identify support groups for emotional support such as RLS Foundation Practice cognitive-behavioral therapy Consider antidepressants that do not exacerbate symptoms Fatigue Eat a healthy diet, nutritional supplements as needed Eliminate foods that increase symptoms of RLS such as caffeine, chocolate, and sugar Promote rest and comfort Rule out anemia Stress management Table 1. Laboratory Tests to Rule Out Conditions That May Cause RLS Condition Test Anemia Reticulocyte count, serum ferritin, CBC (RBC), serum iron, total iron binding capacity, iron saturation Diabetes Fasting plasma glucose, glucose tolerance test Renal disease CBC, BUN, Creatinine, protein, albumin, electrolyte panel, calcium, magnesium, 24-hour urine, urine creatinine clearance
|Gale Copyright:||Copyright 2003 Gale, Cengage Learning. All rights reserved.|