Now preferably known as Willis-Ekbom disease, this sensorimotor neurological disorder is characterized by an urge to move the legs. It’s an oft-missed opportunity to improve sleep quality.
Mom, what’s wrong with you? How come you keep stomping your feet? You’re so weird!
Sound familiar? Sufferers of restless legs syndrome (RLS), now preferably called Willis-Ekbom disease (WED), likely have had similar conversations as the one I had with my children while pregnant with my last child. At least I knew what the problem was!
For many people who have WED, an accurate diagnosis may not be so forthcoming. Sufferers may think no one will take their complaints seriously. In fact, the Restless Legs Syndrome Foundation recently changed its name to the Willis-Ekbom Disease Foundation to “recognize the seriousness of the disease using a simple, easily recognized term that represents the full scope of the disease rather than just the leg symptoms.”1
What is WED?
WED is a sensorimotor neurological disorder. It is thought to have a genetic component. The receptors for neurotransmitter dopamine in the basal ganglia may be faulty or ferritin levels may be low (<45-50 µg/L), particularly in the brain, even though levels in the blood may be normal.2
As reported by Jonesa and Cavanna, WED is a common problem. The estimated prevalence is 12% to 15% with 2% to 3% of patients reporting significant symptoms requiring pharmaceutical therapy.3 It seems to occur more frequently in the female population. The most typical symptom is the irresistible urge to move the legs, usually when at rest or quietly sitting, more frequently during the evening or at night, which may lead to symptoms of insomnia. The sensations have been described as creepy crawly, “jimmy legs,” or cramping and are sometimes painful.
Although a prevalent condition, patients often do not discuss it with their physicians, and thus it is undertreated. Symptoms can be exacerbated by other conditions, such as end-stage renal failure, pregnancy, iron deficiency, and diabetes, often referred to as “secondary WED.” According to the International Restless Legs Syndrome Study Group (IRLSSG), RLS/WED also can result in significant sleep loss and anxiety.4 The National Institute of Neurological Disorders and Stroke states that almost 1 million children are impacted, and that WED negatively influences activities requiring inactivity such as travel, work life, and relaxing.5 Thus, WED can play a significant role in negatively impacting quality of life and restorative sleep.
In 2012, the IRLSSG revised the diagnostic criteria.6 These criteria include:
- urge to move the legs usually but not always accompanied by or felt to be caused by uncomfortable and unpleasant sensations in the legs;
- urge to move the legs and any accompanying unpleasant sensations begin or worsen during periods of rest or inactivity;
- urge to move the legs and any accompanying unpleasant sensations are partially or totally relieved by movement such as walking or stretching at least as long as the activity lasts;
- urge to move the legs and any accompanying unpleasant sensations during rest or inactivity only occur or are worse in the evening or night than during the day;
- occurrence of the above features is not solely accounted for as symptoms primary to another medical or behavioral condition.
While these criteria exist, many primary care physicians have little to no experience with and/or exposure to the diagnosis and treatment of WED. This poses a significant problem. Many of the difficult to treat patients seek consultations with up to 10 specialists. Thus, patients are suffering needlessly and may have limited access to appropriate care with increased cost to the healthcare system.7
In 2013, the IRLSSG produced a consensus statement regarding the long-term management of WED. In addition, Michael H. Silber, MB, ChB, et al published an updated algorithm to accompany these recommendations.8 This algorithm provides guidance for the clinician and implementation of the plan of care.
The IRLSSG outlined the most widely given agents and recommendations for prescribing. Non-ergot-derived dopamine receptor agonists, which include pramipexole (Mirapex), ropinirole (Requip), and rotigotine (Neupro), are deemed effective for 6 months and probably effective up to 12 months. Levodopa is probably effective for up to 2 years in the 24% to 40% of patients who tolerate the therapy. Alpha-2-ligands, gabapentin and pregabapentin, are stated to be probably effective for up to 1 year. High potency opioids can be possibly effective for treatment for WED refractory to other medications.
Nonpharmacologic approaches include checking iron status, introducing mental alerting activities, reducing caffeine intake, and consideration of medication that might be causing the problem.8
When considering therapy with the dopaminergic agents, augmentation may occur. Augmentation is an overall enhanced feeling of WED that may occur at differing times during the day and spread to different parts of the body. It is difficult to manage as there are few options.7
Loss of efficacy over time also should be considered during the management of WED.4
There is also a concern about the development of impulse control disorders (ICDs), a side effect of the dopaminergic agents that should be routinely assessed. ICDs may manifest in numerous ways such as excessive gambling, hypersexuality, compulsive shopping or eating, and punding.9
A new nonpharmacologic product was recently cleared by the FDA. The pad, called “Relaxis,” may be dispensed via prescription. It is not indicated as a treatment for WED, but rather a method to improve the sleep of those patients with WED.10
Although pregnancy can be a time of great joy, for those experiencing WED, it is a time of anxiety and sleeplessness. WED is present in a significant portion of this population, but little data are published.
WED presents itself often during the first pregnancy and typically worsens over subsequent pregnancies, with the third trimester being the worst. Additionally, those with multiple gestation have a higher incidence.11 In a study, Neau et al found that one-third of the population studied had WED and that following delivery, 60% had no further symptoms.12 Reduced iron, even with prenatal vitamins, can remain low. Treatment can be difficult during pregnancy, with iron supplementation being the most commonly provided therapeutic agent. An improvement of symptoms may occur with hot or cold compresses, light massage, and stretching.11
WED is estimated to have a prevalence of 2% to 4% of the pediatric population, and by consensus the diagnostic criteria are the same as for adults.13 Although the age-related description may not include the word “urge” due to cognitive development, the descriptions obtained from children are adequate to make an assessment. Such descriptive words include “have to move” or “my legs have to run” and are clearly associated with “urge.”
The literature has explored the relationship between WED and attention deficit hyperactivity disorder (ADHD). Several hypotheses have been suggested, including that an increase of sleep disruption may manifest as hyperactivity or that the need to move the legs during the day (possibly a different circadian influence) may lead a child to have to move about in class.14
There are also complaints that may mimic WED, which include muscle soreness, positional discomfort and numbness, and growing pains.15 Therapeutic options are limited, and the data to support the use of dopaminergic agents are lacking. Iron supplementation may be tried.
WED is a common, underdiagnosed, and often mistreated condition. WED leads to decreased quality of life and increased costs to the patient and healthcare system. Improvement in the education of, and thus recognition and appropriate treatment regimens by, primary care physicians play an important role, as WED specialists may not be readily accessible. Careful attention regarding the side effects of the dopaminergic agents is of utmost importance, particularly as to impulse control issues. A review of the most current guidelines for the management of WED would be advantageous in the care and management of these patients.
Robyn Woidtke, MSN, RN, RPSGT, CCP, CCSH is principal at RVW Clinical Consulting/Sleep Apnea Risk Associates. Her WED symptoms increased during pregnancy, and she found morning exercise provided some relief. CONTACT: [email protected]
1. Personal correspondence from Karla M. Dzienkowski, executive director, WED Foundation, July 2014.
2. Causes of Restless Legs Syndrome. Johns Hopkins Center for Restless Legs Syndrome. www.hopkinsmedicine.org/neurology_neurosurgery/centers_clinics/restless-legs-syndrome/what-is-rls/causes.html. Accessed July 23, 2014.
3. Jonesa R, Cavanna AE. The neurobiology and treatment of restless legs syndrome. Behav Neurol. 2013;26:283–292.
4. Garcia-Borreguero D, Kohnen R, Silber MH, et al. The long-term treatment of restless legs syndrome/Willis–Ekbom disease: evidence-based guidelines and clinical consensus best practice guidance: a report from the International Restless Legs Syndrome Study Group. Sleep Med. 2013;14(7):675-684.
5. NINDS Restless Legs Syndrome Information Page. National Institute of Neurological Disorders and Stroke. www.ninds.nih.gov/disorders/restless_legs/restless_legs.htm. Accessed July 23, 2014.
6. Diagnostic Criteria. International Restless Legs Syndrome Study Group. www.irlssg.org/diagnostic-criteria. Accessed July 22, 2014.
7. Buchfuhrer MJ. Contemporary challenges and strategies for improving outcomes for patients with restless legs syndrome. Am J Manag Care. 2012;18(12 Suppl):S283-90.
8. Silber MH, Becker PM, Earley C, Garcia-Borreguero D, Ondo WG. Willis-Ekbom Disease Foundation revised consensus statement on the management of restless legs syndrome. Mayo Clin Proc. 2013;88(9):977-986.
9. Cornelius JR, Tippmann-Peikert M, Slocumb NL, Frerichs CF, Silber MH. Impulse control disorders with the use of dopaminergic agents in restless legs syndrome: a case-control study. Sleep. 2010;33(1):81–87.
10. Food and Drug Administration Searchable 510(k) database. www.accessdata.fda.gov/scripts/cdrh/cfdocs/cfpmn/pmn.cfm?ID=K102873. Accessed July 22, 2014.
11. Lee K. Sleep promotion in the childbearing family. In: Redeker N, McEnany GP, eds. Sleep Disorders and Sleep Promotion in Nursing Practice. New York: Springer Publishing Co; 2011:265-266.
12. Neau JP, Marion P, Mathis S, et al. Restless legs syndrome and pregnancy: follow-up of pregnant women before and after delivery. Eur Neurol. 2010; 64:361-366.
13. Picchietti DL, Bruni O, de Weerd A, et al; International Restless Legs Syndrome Study Group (IRLSSG). Pediatric restless legs syndrome diagnostic criteria: an update by the International Restless Legs Syndrome Study Group. Sleep Med. 2013;14:1253–1259.
14. Cortese S, Konofal E, Lecendreux M. The relationship between attention-deficit–hyperactivity disorder and restless legs syndrome. Eur Neurol Rev. 2008; 3(1):111-114
15. Salas RE, Kwan AB. The real burden of restless legs syndrome: clinical and economic outcomes. Am J Manag Care. 2012;18(9 Suppl):S207-12.