Friday, July 3, 2009

#217 Restless Leg Syndrome

FAST FACTS AND CONCEPTS #217

RESTLESS LEG SYNDROME

Jennifer Johnson MD, PhD and Robert Arnold MD

Background Restless Leg Syndrome (RLS) is a neurologic disorder characterized by unpleasant sensations in the legs causing an uncontrollable urge to move when at rest in an effort to relieve those feelings. Between 2-15% of the population have RLS, with a peak incidence between 40 and 60 years of age and a 1:2 male:female ratio. It occurs more frequently in end-stage renal disease patients on chronic hemodialysis (up to 60%, depending on the series), and in patients with Parkinson’s disease (up to 20%) than in the general public. RLS disrupts sleep, can lead to excessive daytime sleepiness, depression, and a decreased quality of life. This Fast Fact will review its evaluation and management.

Causes The etiology of primary RLS is unknown although it is thought to be a genetic disorder involving either central or peripheral dopaminergic pathways. Common secondary causes of RLS are polyneuropathies; diabetes mellitus; rheumatologic diseases such as rheumatoid arthritis, Sjogren’s syndrome, and fibromyalgia; renal failure; pregnancy; iron deficiency; and hypo- or hyperthyroidism. Drugs including nicotine, caffeine, alcohol, 2nd generation antidepressants such as SSRIs and SNRIs, neuroleptic agents, dopamine-blocking antiemetics such as metoclopramide, and sedating antihistamines are all known to worsen RLS symptoms.

Symptoms and Diagnosis The International RLS Study Group and the National Institutes of Health (NIH) criteria for diagnosis include: (1) an urge to move the legs, (2) temporary relief with movement, (3) onset or worsening of symptoms with rest or inactivity, and (4) worsening or onset of symptoms in the evening or night (3). Patients describe symptoms of trouble falling asleep, trouble getting back to sleep, "a funny feeling in the legs," or a "creepy or crawly feeling in the legs." Patients or their bed partners may also report ‘periodic limb movements of sleep’: stereotyped, repetitive flexion movements (‘jerking’) of the legs and occasionally arms, exacerbated when patients lie down for prolonged periods. RLS is a clinical diagnosis for which there is not a confirmatory diagnostic test. It should be differentiated from akathisia, a constant and generalized feeling of motor restlessness not associated with leg discomfort or rest. It can be differentiated from peripheral neuropathies, lumbosacral radiculopathy, and ordinary leg cramps by its circadian rhythm, relief with movement, and the prominence of pain symptoms in non-RLS syndromes.

Treatment Address any treatable secondary causes of RLS (e.g. with iron repletion or levothyroxine) and work with patients to avoid drugs and medications known to aggravate RLS. Distraction activities such as playing video games or crossword puzzles can decrease symptoms during wakeful periods. Drug treatment is recommended for patients who have not improved despite conservative interventions or who have persistent, distressing symptoms. Given the paucity of studies comparing different drugs, experts recommend the following (14):

· Dopamine agonists: The most frequently used drugs are the dopamine agonists, pramipexole and ropinirole. Both have been determined to be effective in industry-funded, double-blind, placebo-controlled studies (7, 8). Doses as low as 0.125 mg of pramipexole at bedtime or 0.25 mg of ropinirole are effective in improving sleep and decreasing discomfort in mild-to-moderate cases. Doses of greater than 0.75 mg/day of pramipexole or 4 mg/day of ropinirole are of unproven benefit. Side effects are usually mild, transient, and limited to nausea, lightheadedness, and fatigue. Both drugs cost roughly $100 (US) a month at the starting dose. While there are small series showing the effectiveness of levodopa/carbidopa, experts have recommended it only be used for intermittent RLS because of worries that levodopa may cause augmentation, rebound, or recurrence of symptoms. Finally, cabergoline, a dopamine agonist with a long half life may be useful for patients who experience rebound symptoms with shorter acting agents, although it is not FDA approved for this purpose.

· Other agents: There are small studies indicating that benzodiazepines (9), opioids (10), and select anticonvulsants such as gabapentin and carbamazepine (11, 12, 13) are effective in RLS. Expert opinion, however, generally recommends these drugs as second line agents due to the paucity of data supporting their use relative to dopamine agonists, side effects, and risk of abuse.

References

  1. Phillips B, Young T, Finn L, et al. Epidemiology of restless legs symptoms in adults. Arch Intern Med. 2000; 160:2137-2141.
  2. Zucconi M, Ferini-Strambi L. Epidemiology and clinical findings of restless legs syndrome. Sleep Med. 2004; 5:293-299.
  3. Allen RP, Picchietti D, Hening WA, et al. 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 Med. 2003; 4:101-119.
  4. Connor JR, Wang XS, Patton SM, et al. Decreased transferrin receptor expression by neuromelanin cells in restless legs syndrome. Neurology. 2004; 62:1563-1567.
  5. Pittock SJ, Parrett T, Adler CH, et al. Neuropathology of primary restless leg syndrome: absence of specific tau- and alpha-synuclein pathology. Mov Disord. 2004; 19:695-699.
  6. Silber MH, Richardson JW. Multiple blood donations associated with iron deficiency in patients with restless legs syndrome. Mayo Clin Proc. 2003; 78:52-54.
  7. Montplaisir J, Nicolas A, Denesle R, Gomez-Mancilla B. Restless legs syndrome improved by pramipexole: a double-blind randomized trial. Neurology.1999; 52:938-943.
  8. Adler CH, Hauser RA, Sethi K, et al. Ropinirole for restless legs syndrome: a placebo-controlled crossover trial. Neurology. 2004; 62:1405-1407.
  9. Peled R, Lavie P. Double-blind evaluation of clonazepam on periodic leg movements in sleep. J Neurol Neurosurg Psychiatry. 1987; 50:1679-1681.
  10. Ondo WG. Methadone for refractory restless legs syndrome. Mov Disord. 2005; 20:345-348.
  11. Telstad W, Sorensen O, Larsen S, et al. Treatment of the restless legs syndrome with carbamazepine: a double blind study. BMJ. 1984; 288:444-446.
  12. Garcia-Borreguero D, Larrosa O, de la Llave Y, et al. Treatment of restless legs syndrome with gabapentin: a double-blind, cross-over study. Neurology. 2002; 59:1573-1579.
  13. Eisensehr I, Ehrenberg BL, Rogge Solti S, Noachtar S. Treatment of idiopathic restless legs syndrome (RLS) with slow-release valproic acid compared with slow-release levodopa/benserazide. J Neurol. 2004; 251:579-583.
  14. Silber MH, Ehrenberg BL, Allen RP, et al. An algorithm for the management of restless legs syndrome. Mayo Clin Proc. 2004; 79(7):916-22.

Author Affiliations: University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.

#216 Asking About Cultural Beliefs in Palliative Care

FAST FACTS AND CONCEPTS #216

Asking about cultural beliefs in palliative care

Hillary Lum MD, PhD and Robert Arnold MD

Background Patients’ cultural backgrounds profoundly influence their preferences and needs regarding discussing bad news, decision-making, and the dying experience. This Fast Fact offers a framework for taking a ‘cultural history’ to better understand a patient’s and family’s needs. See also these related Fast Facts: #17 (illness experience), #19 (spiritual history), #26 (explanatory model), #183/184 (conflict resolution).

C – Communication. Identify the patient’s preferences regarding how and to whom medical information is shared. Some people want to know everything about their medical condition, and others do not. How much would you like to know? For those who request that the physician discuss their condition with family members: Would you like me to speak with them alone, or would you like to be present? Identify main contacts to give information to about the patient’s condition. Carefully explore with families requests to hide information from a patient (see references 4 and 5) – a future Fast Fact will address this topic in more detail.

U – Unique cultural values. Use respectful, curious, and open-ended questions about a patient’s cultural heritage to identify their values. Is there anything that would be helpful for me to know about how you and your family view serious illness? Are there cultural beliefs, practices, or preferences that affect you during times of significant illness? If the patient is open to discussing death: What concerns do you have about dying? Are there things that are important to you or your family that I should know about?

L – Locus of decision-making. For some patients medical decision-making is communally driven rather than individualistic. Multiple family members or a community elder or leader may need to be involved, often without prior official documentation because it is assumed or understood from the patient’s perspective. Do you prefer to make medical decisions about tests and treatments yourself, or would you prefer that others in your family or community make them for you?

T – Translators. Language barriers are extremely challenging, especially during times of severe illness. Utilize medical interpreters frequently and effectively. Refer to Fast Fact #154 for a detailed discussion on using interpreters in palliative care.

U – Understanding the patient and learning as a provider. Reassess what is being heard, understood, and agreed upon frequently, from both the patient’s and clinician’s standpoint. Specifically confirm the patient’s understanding or agreement (beyond nodding or “yes” responses). This is particularly important if a medical translator is involved as miscommunication is common even when using trained medical interpreters – see reference (6). Can you tell me – in your own words – what you have heard from me and what’s most important to you about what I’ve said?

R – Ritualized practices and restrictions. Determine if there are specific customs the patient desires to be followed. These must be communicated to other health care providers, especially in the hospital setting. It may be necessary to advocate for the patient and negotiate with healthcare facility administrators to find an agreeable way to honor a patient’s wishes. Are there specific practices that you would like to have in the hospital or at home? Are there aspects of medical care that you wish to forgo or have withheld because of your cultural beliefs? Is anything discouraged or forbidden? If the patient is approaching death, and willing to discuss it: Are there specific practices that are important to you at the time of death or afterwards that we should know about?

E – Environment at home. Given that a majority of hospice care happens in the patient’s home environment, respectfully explore whether there are any needs that can be met by the health care system, and how open the patient, family or community is to receiving care at home. Recognize that patients may be hesitant to voice needs, or resistant to accepting help from outside the community. Even if a trusting, collaborative relationship has developed between a patient/family and clinicians in the hospital, this may not immediately translate into the home setting. With the patient’s permission, expectations about cultural-specific aspects of a patient’s care should be explicitly communicated to care providers outside the hospital.

References

  1. Searight HR, Gafford J. Cultural Diversity at the End of Life: Issues and Guidelines for Family Physicians. Am Fam Phys. 2005; 71 (3)
  2. Crawley LM, et al. Strategies for Culturally Effective End-of-Life Care. Ann Internal Med. 2002; 136:673-679.
  3. Maugans TA. The SPIRITual History. Arch Fam Med. 1997; 5:11-16.4. Arnold R. Palliative Care Case of the Month: The Family Says Not to Tell. University of Pittsburgh Institute to Enhance Palliative Care. May 2006. Available at: http://www.dgim.pitt.edu/SPC/cases/May 06.doc.
  1. Hallenbeck J, Arnold R. A request for non-disclosure: don’t tell mother. J Clin Oncol. 2007; 25(31):5030-4.
  2. Pham K, et al. Alterations During Medical Interpretation of ICU Family Conferences That Interfere With or Enhance Communication. Chest. 2008; 134(1):109-116.
Author Affiliations: University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.