Makindo Medical Notes

Also known as Willis-Ekbom Disease (WED)

About

• Willis-Ekbom Disease, also known as Restless Legs Syndrome (RLS), is a neurological disorder that manifests as an uncontrollable urge to move the legs. The condition is equally common in men and women.
• It is thought to be related to dopamine dysregulation in the basal ganglia, a brain area responsible for motor control. Iron depletion is also a possible contributing factor, often associated with low ferritin levels.
• Drugs that can exacerbate symptoms include:
  • Steroids
  • Calcium channel blockers
  • Dopamine blockers (e.g., antipsychotics)
  • Lithium
  • Tricyclic antidepressants (TCA's)
  • Selective serotonin reuptake inhibitors (SSRI's)

Clinical Features

• Most commonly seen in the elderly, but it can affect people of all ages.
• Characterized by an overwhelming urge to move the legs, typically accompanied by uncomfortable or abnormal sensations (e.g., tingling, itching, or "creepy" sensations in the legs).
• Symptoms tend to worsen at night, particularly when the patient is lying down, and are often relieved by movement such as walking or stretching.
• In more severe cases, the arms may also be involved.
• These symptoms can delay sleep onset, contributing to insomnia or poor sleep quality.
• Periodic limb movements, such as twitching or kicking during sleep, may also be present.

Causes

• Chronic kidney disease (renal failure) with uraemia increases the likelihood of RLS.
• Iron deficiency, particularly low ferritin levels, has been strongly associated with RLS, and symptoms may improve with iron supplementation.
• Genetic factors may play a role, especially in cases with a family history of the disorder.
• Pregnancy, particularly in the third trimester, can lead to temporary symptoms of RLS.

Investigations

• Check Full Blood Count (FBC) and Ferritin levels to rule out iron deficiency.
• Consider kidney function tests, especially in those with known renal disease.
• Sleep studies (polysomnography) may be helpful in differentiating RLS from other sleep-related movement disorders.

Differential Diagnosis

• Differentiate from nocturnal myoclonus (also known as periodic limb movement disorder), where jerking movements occur during sleep. These movements are often unrecognized by the patient and may only be noticed by a bed partner.
• Other differential diagnoses include neuropathies, Parkinson’s disease, and other sleep disorders (e.g., obstructive sleep apnea).

Management

• Treatment can be challenging, with a high risk of treatment failure or symptom recurrence.
• First-line treatment includes:
  • Iron supplementation if ferritin levels are below 50 ng/mL, as this may significantly improve symptoms.
• Dopamine agonists are commonly used:
  • Pramipexole
  • Ropinirole
  • Rotigotine (available as a patch for continuous delivery)
• Levodopa, particularly long-acting formulations, may be helpful for some patients, especially in intermittent or mild RLS.
• Benzodiazepines, such as Clonazepam or Temazepam, may be used to help with sleep, though long-term use should be approached cautiously due to the risk of dependence.
• Other medications that may be used include:
  • Gabapentin or Pregabalin (used particularly for patients with painful RLS or those who cannot tolerate dopamine agonists)
  • Baclofen (muscle relaxant)
  • Opioids, such as Codeine or Tramadol, are reserved for refractory cases due to the risk of addiction and side effects.
  • Carbamazepine, an anticonvulsant, may be considered in some cases.
• Non-pharmacological strategies, such as maintaining a regular sleep schedule, reducing caffeine and alcohol intake, and ensuring good sleep hygiene, may also improve symptoms.

Prognosis

• RLS is a chronic condition that can fluctuate in severity over time. Some patients experience remission, while others may have persistent symptoms.
• Early diagnosis and treatment can improve quality of life, but many patients require long-term management.