Hereditary Acromelalgia

Neurologic movement disorder characterized by an unpleasant sensation in the lower extremities, eliciting intolerable restlessness with twitching and jumping of the legs when the patient is at rest, especially at night.

Restless Legs Syndrome; Acromelalgia-Painful Legs-Moving Toes Syndrome; Anxietas Tibialis Syndrome; Asthenia Crurum Dolorosa; Asthenia Crurum Paresthetica Syndrome; Ekbom Syndrome; Leg Jitters Syndrome; Moving Toe Syndrome; Painful Leg Syndrome, Wittmaack-Ekbom Sequence.

First described by the English anatomist Thomas Willis in 1672, then by Theodor Wittmaack, a German clinician, in 1861. Restless legs syndrome may occur as a primary condition (idiopathic) or may be a result of other conditions (secondary or symptomatic restless legs syndrome).

Prevalence is evaluated at 5.5% of the general population, with a female predominance. Prevalence increases with age.

The disorder is usually familial and inherited as an autosomal dominant trait. Candidate gene map locus is 12-q21.

Unknown. Idiopathic myoclonic jerks occur just prior to sleep and severely affect it. However, it disappears with the onset of stage 1 REM (rapid eye movement) sleep. It is associated with a variety of conditions, including iron deficiency, folic acid anemia, B vitamin deficiency, drug intake (phenothiazine), barbiturate withdrawal, diabetes, renal insufficiency, neuropathy, stroke, and chronic respiratory illness. It is common during pregnancy and is precipitated by fatigue, anxiety, and stress.

History of repeated, asymmetric flexion jerks of the legs occurring at night just before and interfering with sleep. Acromelalgia, myoclonus, and paresthesia are characteristic.

Symptoms worsen with age. Hereditary acromelalgia is a lifelong condition with no cure. Opioids are effective for relieving pain, and correction of iron deficiency may improve symptoms for some patients. Sleep deprivation may become severe with increasing age.

Check usual medication (affected patients often take either neuroleptic and/or sedative medications). Ensure that regular medications are continued until the morning of surgery. Check red blood cell count and evaluate for iron deficiency.

No specific considerations with this condition.

None reported. Most affected patients take medications such as temazepam, levodopa/carbidopa, bromocriptine, pergolide mesylate, oxycodone, propoxyphene, and codeine, which are effective in relieving symptoms but may interfere with anesthetic techniques and requirements for postoperative pain relief.

Erythromelalgia: Characterized by repeated episodes of paroxysmal vasodilation with burning pain in the hands and feet. Diminished sensitivity to temperature changes, especially with cold acting as pain depressor and heat as stimulator. Affects middle-aged patients of both sexes. Unknown etiology but may occasionally be inherited as an autosomal dominant trait.