Restless arms syndrome: prevalence, impact, and management strategies

Figures & data

Table 1 Reported cases restless arms syndrome and analyses for adherence to IRLSSG diagnostic and supportive criteria

		Five essential diagnostic criteria					Additional features supporting diagnosis
Case number	Publication	1. Urge to move	2. Worse at rest	3. Relief by movements	4. Worse in the evening or night	5. Symptoms not solely accounted for/by a medical/behavioral condition	1. PLMs	2. Dopaminergic treatment response	3. Family history	4. Lack of profound daytime sleepiness
1	Webb, 1976; JAMA^Citation20	Y	n.r.	Y	Y	Y	n.r	n.r	n.r	n.r
2	Freedom et al, 2003; Arch Neurol^Citation21	Y	Y	Y	Y	Y	Y	Y	N	n.r
3	Horvath et al, 2008; Lancet^Citation22	Y	Y	Y	Y	Y	Y	Y	n.r.	n.r
4	Konstantakopoulos et al, 2009; J Clin Psychopharmacol^Citation23	Y	Y	Y	Y	Y	n.r	n.r	Highly probable RLS grandfather	n.r
5	Munhoz et al, 2012; Clin Neurol Neurosurg. Case 1^Citation24	Y	Y	Y	Y	Y	Y	Y	n.r.	n.r.
6	Munhoz et al, 2012; Clin Neurol Neurosurg. Case 2^Citation24	Y	Y	Y	Y	Y	n.r.	Y	n.r.	n.r
7	Ruppert et al, 2012; Mov disord^Citation25	Y	Y	Y	Y	Y	Y	Y	N	Y
8	Gupta et al, 2013; J Neurosci Rural Pract^Citation26	Y	Y	Y	Y	Y	n.r	Y	N	n.r
9	Ruppert et al, 2015; Neurology^Citation27	Y	Y	Y	Y	Y	Y	Y	N	Y
10	Kim et al, 2017; Acta Neurol Belg^Citation28	Y	Y	Y	Y	Y	n.r	Y	n.r.	n.r.

Abbreviations: Y, yes; N, no; n.r., not reported.

Table 2 Reported cases and characteristics of restless arms syndrome

Case #	Publication	Sex	Age at RAS onset	Diagnosis delay	Symptoms’ localization	Clinical characteristics	Triggering factor/comorbidities	Impact on sleep/daytime functioning	Treatment efficiency	Symptoms’ evolution
1	Webb, 1976; JAMA^Citation20	M	23	n.r.	Bilateral	“urge to move the arms in an aimless manner”; “movements were more athetoid than jerky or irregular”	Onset 6 months following spinal cord injury with complete paralysis below the fourth thoracic vertebra and persistent paresthesia in both legs	n.r.	n.r.	Symptoms gradually disappeared without recurrence for 23 years
2	Freedom et al, 2003; Arch Neurol^Citation21	M	77	1 year	Bilateral; symmetric	“twitching and wiggling”	Possible beginning peripheral neuropathy; history of coronary artery disease with coronary bypass surgery; hiatal hernia; pulmonary tuberculosis in the past	Y	Ropinirole tramadol; clonazepam, gabapentin	Two years after RAS onset the restlessness had progressed to involve the legs
3	Horvath et al, 2008; Lancet^Citation22	M	39	25 years	Bilateral	“burning and itching”	Hay fewer; asthma	Y	Pramipexole	Extension to daytime RAS
4	Konstantakopoulos et al, 2009; J Clin Psychopharmacol^Citation23	M	24	<1 week ?	Bilateral	“tingling and prickling”	Drug -induced (24 h following olanzapine introduction, but not with risperidone nor amilsupride); chronic paranoid schizophrenia	Y	n.r	No recurrence after 3 months follow-up
5	Munhoz et al, 2012; Clin Neurol Neurosurg. Case 1^Citation24	M	47	7-months	Bilateral arms and lateral part of shoulders	“inner discomfort”	n.r	n.r	Pramipexole	Significantly improved using pramipexole after 30 months follow-up
6	Munhoz et al, 2012; Clin Neurol Neurosurg. Case 2^Citation24	M	44	4-months	Bilateral; symmetric	“unpleasant” “cramp”	n.r	Y	Pramipexole	Complete symptoms remission using pramipexole after 18 months follow-up
7	Ruppert et al, 2012; Mov disord^Citation25	F	35	5 years	Bilateral	"pins and needles"	Focal epilepsy controlled with oxcarbazepine; mild right hemiparesis with periventricular lesions resulting from perinatal complications	Y	Pramipexole and iron; tramadol	RAS extended 3.5 years later with minor discomfort in the legs; augmentation syndrome
8	Gupta et al, 2013; J Neurosci Rural Pract^Citation26	F	28	7-months	Right	"tickling sensation deep inside the muscles”	Bilateral cervical ribs; depression	Y	Pramipexole and iron; clonazepam and duloxetine	Depression appeared after RAS onset; almost complete regression of RAS at 3 weeks follow-up
9	Ruppert et al, 2015; Neurology^Citation27	M	73	<2 years	Bilateral	"electric shock sensations and myoclonic-like jerks”	Hypertension; obstructive sleep apnea syndrome efficiently treated using CPAP; non-evolutionary monoclonal IGA Kappa pic; vitiligo; chronic, moderate, bilateral compressions of the ulnar nerves at the level of the elbows, as well as the median nerves at the carpal tunnel, treated surgically in the past with normal neurological examination and the patient had noticed no relationship between restlessness severity and the evolution of these comorbidities; spinal stenosis C3-7 without signs of compression, minor left antero -lateral pontine hypersignal more likely of vascular origin and an état criblé in both insula and lentiform nuclei revealed by cervical and brain MRI	Y	Piribedil; ropinirole; pramipexole; rotigotine; gabapentin; pregabalin; clonazepam; tramadol; oxycodone; fentanyl	RAS was preceded about 8 years earlier by transient RLS with no need for specific medication; augmentation syndrome
10	Kim et al, 2017; Acta Neurol Belg^Citation28	M	65	10 days after discharge post stroke hospitalization	Right, predominant on the proximal part of the arm	“prickling and pain with irregular and coarse hyperkinetic movements”	Stroke-related after left thalamic infarction involving the posterolateral nucleus with mild hemiparesis and dysesthesia on the right side, predominantly on the upper extremity, no neurological deficits were observed on discharge	n.r	Levodopa improved symptoms previously worsened using haloperidol	n.r.

Abbreviations: F, female; M, male; Y, yes; N, no; n.r., not reported.

Figure 1 Dopaminergic treatment response in patient number 7. Hypnograms of patient number 7 having vPSGs with a first night under the habitual dosage of 0.27 mg pramipexole (A), followed by vPSG on the second night after an abrupt drug withdrawal causing very severe sleep onset and maintenance insomnia with sleep loss due to a major rebound of restlessness (B) and a third night of vPSG after reintroduction of pramipexole 0.27 mg relieving symptoms and permitting the patient to recover sleep (C).

Abbreviation: vPSG, videopolysomnography

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Konstantakopoulos G, Oulis P, Michalopoulou PG, Papadimitriou GN. Olanzapine-induced “restless arms syndrome.”. J Clin Psychopharmacol. 2009;29(1):89–90. doi:10.1097/JCP.0b013e31819302a019142116

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Ruppert E, Cretin B, Meyer C, Kilic-Huck U, Bourgin P. Characterization of periodic upper limb movement disorder in a patient with restless arms syndrome. Mov Disord. 2012;27(11):1459–1461. doi:10.1002/mds.2403522927037

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Gupta R, Lahan V, Goel D. Restlessness in right upper limb as sole presentation of restless legs syndrome. J Neurosci Rural Pract. 2013;4(1):78–80. doi:10.4103/0976-3147.10562523546363

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Ruppert E, Tranchant C, Kilic-Huck U, Carpentier N, Bataillard M, Bourgin P. Bedtime-related jerks in the upper limbs associated with restless arms syndrome. Neurology. 2015;84(9):959. doi:10.1212/WNL.000000000000130625732362

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Kim H-Y, Park M-H, Kwon D-Y. Post-stroke restless arm syndrome mimicking hemichorea-hemiballism. Acta Neurol Belg. 2017;117(3):791–792. doi:10.1007/s13760-017-0758-y28181185

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