by Matthias Schiess & Torsten Liem
Table of contents
The following overview provides an insight into the etiology, epidemiology, diagnosis, and treatment of Restless Legs Syndrome (RLS) in children. Etiologically, multicausal factors are suspected: iron deficiency, hormonal (dopaminergic mechanism), vegetative dysfunctions, and genetic predispositions. For a long time, diagnosis in children was difficult to establish. It is primarily made clinically. Complete recovery through conventional medical (as well as osteopathic) treatment is currently not proven.
Introduction
Approximately 7%-11% of the Western population suffers from Restless Legs Syndrome (RLS) (57). A prevalence of at least 1.9%-2% exists in children and adolescents (56). In osteopathic literature, RLS currently does not appear, with one exception (55). RLS symptoms and their consequences, such as sleep disturbances, often represent an unrecognized problem in children and adolescents. ADHD or growing pains can be associated with RLS. Diagnosis is made clinically.Historical Background The first written references to RLS were found in the 17th century (1). Later in history, psychological etiologies were also suspected, characterized by the term “anxietas tibiarum.” In the mid-20th century, a comprehensive description of the clinical symptoms and the still valid term “Restless Legs Syndrome” (RLS) was created by K.A. Ekbom (2). Involuntary leg movements (Periodic Limb Movements in Sleep, PLMS) were first associated with RLS in 1965 (3). A familial accumulation of RLS indicated the heritability of the disease (3). From the same period came the first effective therapeutic attempts with iron and clonazepam, as well as numerous ineffective medications such as heparin, aspirin, and nitroglycerin. Current dopaminergic-based therapeutics, antiepileptics, and opiates have only been part of the standard pharmacological treatment for RLS since the late 20th century.
Definition
Recommended criteria were formulated by the International Restless Legs Study Group (IRLSSG) (4), see Tab. 1:
Epidemiology
The late definition of RLS diagnostic criteria explains why only a few epidemiological studies are currently available. Significant differences in disease distribution between Germany and India (6,7,9) suggest possible (partially proven) genetic predisposition and environmental influences such as nutritional habits, lifestyle, work, psychological factors, illnesses, and traumas, etc., which negatively affect the course of RLS (=epigenetic factors). Significant epidemiological data for children with RLS were only collected in a large study in 2008. This study reported a prevalence of 1.9% to 2% in children and adolescents (56). Epidemiological, retrospective RLS surveys in adults even suggest higher prevalences: 20% of respondents stated that they had suffered from RLS symptoms in childhood, mostly before the age of 10 (5). Furthermore, the high prevalence of the disease, often with onset in young adulthood, suggests that RLS symptoms and their consequences, such as sleep disturbances, represent an often unrecognized problem in children and adolescents. Distinctions from other clinical pictures are sometimes difficult. It has been proven that ADHD or growing pains can be associated with RLS and vice versa (see below). If the disease was diagnosed in childhood, a positive family history is often found. Conversely, the family history is negative for diseases in adulthood. In studies in pediatric practices and sleep laboratories, researchers found a prevalence for RLS between 0.2-3% (12,13,14).
Etiology – Pathogenesis
Etiology and pathophysiology are still largely unknown. Multicausal factors are suspected: iron deficiency (56), hormonal (dopaminergic mechanism (21,22,23,24,25)), autonomic dysfunctions, and genetic predispositions (17). There appears to be a primary dysfunction in the neurotransmitter system located in the basal ganglia of the human brain, particularly in the striatum (putamen and caudate nucleus) (26, 27, 28). Currently, the “iron theory” is particularly favored, according to which RLS is associated with a disorder of iron metabolism (29, 30,31). Iron is essential for the synthesis of dopamine. Iron, copper, tyrosine, and the enzyme tyrosine hydroxylase are converted to L-Dopa and then to melanin and the catecholamines dopamine, norepinephrine, and adrenaline (32). Reduced iron content has been detected in RLS patients in MRI and ultrasound examinations of the substantia nigra and putamen (33,34). Hormonal influences (7,35): Progesterone and estrogen appear to have a strong influence on dopamine metabolism (7,8,36). The ovaries and adrenal glands are primarily responsible for the formation of these hormones. Consequently, these aspects could explain the twofold higher incidence of RLS in women. The organs of the H-P axis (pituitary/hypothalamus) function as control centers. These centers regulate the sensitive control loops of hormones and the autonomic nervous system. The trigger for RLS is still largely unclear, but there is evidence for epigenetic factors (15,16). Women appear to be affected twice as often as men. Data on the sex ratio in childhood are not yet available. Heredity is suspected as the most common and primary cause of RLS, the idiopathic form of RLS (60% positive family history) (3,17, 57). In the idiopathic/primary form, the first symptoms appear earlier (age 35) than in the non-familial/secondary form (age 47). In the secondary RLS form, triggering factors such as iron or vitamin B deficiency, pregnancy, menopause, kidney failure, diabetes mellitus, polyneuropathies, medications, and rheumatoid arthritis have been proven (18). Genetic components and iron deficiency appear to be of particular importance in childhood RLS (56). Further suspected associations for the onset of RLS: with fibromyalgia, vitamin deficiency, Parkinson’s disease, sleep apnea, chronic obstructive pulmonary disease (COPD), thyroid diseases, etc. Increased consumption of caffeine, chocolate, and alcohol leads to an increased frequency of symptoms. Approximately 60% of RLS patients show depression, and 30% experience tension headaches (19,20). An increased incidence of gastrointestinal complaints is also possible.
Diagnostics
In the general population, RLS is widely underdiagnosed (39,40). Diagnosis can only be made through clinical (not physical) findings (4,5). For children, experts agreed on the following 2 criteria (see Tab. 2). The diagnostic criteria differ in terms of comprehensibility for children and adolescents, as they often have difficulty adequately expressing their complaints. A striking feature in children with RLS is a close connection to “Periodic Limb Movement Disorder” (PLMD) (56). Symptoms of both can vary from mild to severe (56).
How do you proceed with this?
When, through our hand contact on the head and pelvis with gentle longitudinal compression, we copy the tensions in the body, the child usually assumes birth positions in which they may have experienced traumatic situations, for example, a stalled birth process, a too-rapid birth, or a cesarean section. For instance, the child calms down when experiencing pressure at certain points in the body. Or conversely: they react in certain positions with hyperarousal or a freezing response. I also examine the midline structures or functions, meaning the neural tube, the notochord, and the anterior midline. After trauma, I often have the impression that dysfunctions occur here. This feels palpatorily to me as if compressions, interruptions, stagnations, slowed dynamics, or thinning, alterations in flow directions occur. Subsequently, I palpate the arms and legs to determine whether tensions are more apparent in the splanchnopleura or somatopleura. Strong tension zones would be contacted as part of trauma treatment. 1. Suggested criteria for PLMD in childhood: 1. The polysomnogram shows a PLM index >5/h is found in the polysomnogram, and 2. sleep disturbances exist in the form of difficulty falling asleep and staying asleep or significant daytime sleepiness, and 3. the leg movements did not occur as a result of disturbed nocturnal breathing (i.e., they are independent of respiratory abnormalities) or as a result of medication (e.g., with antidepressants). Only in cases of unclear clinical symptoms, but with existing accompanying phenomena or insufficient information, are optional additional criteria used. Onset: For example, disturbed sleep behavior can be recorded using polysomnography based on periodic limb movements during sleep (37,38). PLMS in childhood frequently occur in various clinical pictures. Their significance for the diagnosis of RLS is lower than in adulthood and can therefore only be used to a limited extent. The following diseases must be excluded in RLS in children and adolescents: growing pains (42), malignant diseases / rheumatic diseases (43), fibromyalgia (44), somatization disorders (45), as well as childhood sleep onset and maintenance disorders (46). Comorbidity exists with Tourette syndrome, obstructive sleep apnea syndrome*, and attention deficit/hyperactivity disorder (ADHD)** (47, 48, 50). I: 1. The child meets all 4 obligatory criteria for adults. 2. Their description corresponds to sensations of discomfort (“leg discomfort”) in the legs (the child may use expressions such as ouchy spot, tickling, tingling, 1000 needles, pricking, “legs want to run,” or “lots of energy in the legs.” Age-appropriate descriptions should be encouraged). II: 1. The child meets the 4 obligatory diagnostic criteria for adults and 2. 2 of the following 3 supportive criteria: a) Sleep disturbances b) First-degree relatives suffer from confirmed RLS c) A PLMS index of >5/h sleep time was documented in the polysomnogram. III: 1. The child’s behavior indicates discomfort in the legs when sitting or lying down, accompanied by movement of the affected leg. The discomfort has similar characteristics to those described in adults in obligatory criteria 2, 3, and 4 (worsening during rest and inactivity, improvement with movement, and worsening in the evening and at night) and 2. The child has first-degree relatives who suffer from confirmed RLS. 3 Sleep problems are common in children. Recent epidemiological studies estimate a prevalence of 12-37%. When parents and children were surveyed, long sleep onset times, unwillingness to go to bed, frequent nocturnal awakenings, and breathing problems such as snoring were cited as the most common sleep problems. Further clarification is required for the following anamnestic data: snoring and other sleep-related breathing problems, restless sleep, bedwetting (enuresis), enlarged tonsils and nasal polyps; overweight and nutrient deficiencies should also be considered. OSAS can be confused with ADHD. The good response to Ritalin suggests a neurotransmitter disorder in dopamine metabolism (51), also with indications of iron/ferritin deficiencies in the storage organs, in blood serum, and in the CNS in children with ADHD or RLS (52).
Therapy
To date, there is no treatment that brings about complete recovery from RLS symptoms (unless RLS occurs only as an accompanying symptom of other disorders). L-Dopa and dopamine agonists are the primary drugs of choice in adults (18, 57), possibly in combination with hypnotics or benzodiazepines. Antiepileptics are considered particularly suitable for painful forms of RLS or in cases of neuropathic pain in polyneuropathy. Opiates are only used when other medications fail. Currently, there is a lack of well-founded clinical studies on the effect of drug therapies with L-Dopa and dopamine agonists in children. In addition to iron and vitamin C supplementation, positive effects are achieved with vitamin B complexes and magnesium (53,54).
Osteopathic Treatment Contexts
In osteopathic literature, RLS currently does not appear, with one exception (55). The only published osteopathic study on RLS by Peters is of a prospective-observational nature (6). It investigated the effectiveness of positional release points (Strain-Counterstrain according to Jones). Other forms of treatment were not applied. To do justice to the complex clinical picture, RLS should be treated with all forms of osteopathic “art.” Reducing accompanying symptoms and thereby the severity of RLS is a problem-oriented approach in osteopathy. The multifactorial symptom complex can be effectively addressed with various osteopathic techniques in the following areas. In children, the following functions and organs should be examined in particular: • The abdominal organs, especially the duodenum and jejunum (mucosa), which are responsible for active iron absorption, as well as the iron storage organs liver and spleen; not to forget the important early imprinting of the small intestinal flora in newborns and nutritional factors (a stool examination is also recommended). To optimally absorb iron, sufficient vitamin C must be present, which is absorbed in the ileum. The treatment here aims to improve organ functions. The small intestine, as well as its fascial, vascular, and neural relationships and the mesentery, must be treated. In addition to specific osteopathic spleen and liver treatment, the application of a liver pump technique also seems useful. The stomach – in addition to other nutritional tips and a general so-called detoxification/deacidification of the patient – could also be treated osteopathically to promote iron absorption. (The acidity in the stomach is important here for iron absorption). • The closely coupled structures with the autonomic nervous system, which provides insights into the state of the autonomic nervous system (nociceptive system, organs). Treatment approaches to harmonize the autonomic nervous system could include: general techniques such as CV-4, CV-3, Midline, dural tube rocking, and inhibition of the lower thoracic and lumbar spine. • The surrounding structures of central hormone control or the brainstem, i.e., the craniosacral system (dopamine, ferritin, and transferrin levels in the CSF, nociceptive system). • The endocrine organs in general. Cerebral techniques such as those for the striatum and substantia nigra are also possible as supplementary treatments. • Jones (or Longden) finally recommends a Strain-Counterstrain treatment of a tender point on the surface of the os pubis, very close to the symphysis (55). In this, the homolateral leg – hanging from the treatment couch – is stretched into hyperextension. By Matthias Schiess and Torsten Liem
