Etymologically, migraine originally is described as typical hemicranial severe headache (Greek – hēmíkraira  =  half the head). Women suffer from migraine about three times as often as men. A similar gender distribution is found in adolescents.

So far, two migraine genes have been identified which are responsible for the rare familial hemiplegic migraine. There are many hints that genetic factors and hormonal factors – e.g. during the female cycle –are jointly responsible for triggering a migraine attack. In adolescent girls, starting with the menarche, and women, migraine without aura is frequently associated with menstruation. Therefore, the International Headache Society (IHS) included both menstrual migraine and menstrually related migraine in the IHS classification.

Unfortunately, the term “migraine” has developed in common language into a term for any type of severe headache. On closer examination, a headache termed “migraine” often does not comply with the criteria of the IHS. According to the IHS, migraine is defined as a sudden periodic headache, usually with a throbbing quality. This may be accompanied by symptoms such as nausea, vomiting, or increased sensitivity to light (photophobia) or auditory stimuli (phonophobia). Very often, symptoms increase in severity with physical activity. The patient withdraws, avoiding physical activity. Especially in younger children who are not able to verbally describe their photophobia or phonophobia due to their developmental age, their behavior delivers important diagnostic clues. If the migraine attack leads to transient neurological deficits preceding the pain, this is called an “aura.” Manifestations of an aura may be visual or sensory disturbances of perception or motoric disturbances like paresis or expressive speech disturbances. The most common type of migraine is without aura, which has a higher attack frequency than migraine with aura. The diagnosis of migraine as a primary headache should not be given unless other neurological diseases can be excluded. The IHS defines the following diagnostic criteria for migraine (http://ihs-classification.org/en/):

Sometimes, it is quite difficult to differentiate between migraine without aura and episodic tension-type headache (see below). In order to help children, parents, and professionals to differentiate tension-type headache from migraine in childhood, Table 2.1 lists the typical symptoms pinpointing the differences.

In case the patient falls asleep during a migraine attack and wakes up free of pain, the duration of the attack is calculated from the time of the first symptom until ­symptom-free awakening. Please note that especially in younger children, migraine ­headache is often described as double sided. The typical adult-type one-sided headache develops in adolescence or in young adulthood. Most migraine headache is localized frontotemporal. Especially in children, occipital pain (single or double sided) is seldom reported. Doing diagnostics in such cases, one should be careful not to miss space-occupying cerebral processes or structural lesions. During the migraine attack, cerebral blood flow does not show primary changes that would be characteristic of cortical spreading depression moving across the cortex in waves. However, perfusional changes in the brain stem may well exist as may secondary cortical changes as a consequence of pain activation. In contrast, in migraine with aura, there is a pathognomonic decreased perfusion underlying the neurological deficits. Thus, the “spreading depression” in migraine without aura is seen as pathophysiologically irrelevant. With certainty, the vasodilating substances nitrous oxide (NO) and calcitonin gene-related peptide (CGRP) are part of this process. For a long time, the vascular changes and the consecutive changes in perfusion were held primarily responsible for migraine. More recently, the sensitization of perivascular nerve endings is seen as a possible pain trigger. Therefore, the possibility of migraine attacks being generated within the CNS is being discussed.

Meanwhile, we know about the relationship between migraine pain and neurotransmission. Data being gathered since the implementation of triptans into therapy are of special importance in clarifying these interrelationships. Triptans turned out to be very effective in the treatment of an acute migraine attack. Nowadays, it is undisputed that migraine is a complex neurobiological disease and not just the result of primarily vascular changes.

In spite of the severity of migrainous pain, there is no underlying destructive cerebral process. The only risk with migraine is not to treat it the right way, i.e., using analgesics at the very beginning of an attack. Treated with delay (i.e., not taking the medication until the patient can’t stand the pain anymore), insufficiently (i.e., using a low drug dose), or in the wrong way (taking a nap instead of taking medication, using relaxation techniques during a migraine attack) make children suffer severe headaches more frequently. As time goes by, it becomes more probable that pain accompanied by fear of the upcoming pain attack is learned, establishing a pain memory and chronic headache.

Tension-type headache is said to be the most frequent primary headache. Although its etiology and pathomechanisms are still unknown, the IHS defines this type of headache as a disease entity assigned to the primary headaches. It might well be that TTH comprises several different types of headache of still unknown origin. Meanwhile, many studies suggest that at least TTH with a severe course has a neurobiological origin.

It is helpful to distinguish between chronic TTH and episodic TTH. Chronic TTH leads to an impaired quality of life and has the potential to severely affect daily routine. Episodic TTH comprises two subtypes: the sporadic subtype, exhibiting pain less than once per month, and the subtype with more frequent attacks. The impact of the sporadic subtype on the patient’s life is mild, while the subtype with more frequent attacks may result in life impairment similar to chronic TTH, leading to frequent usage of analgesics and frequent contact with health-care professionals, which may become a true financial burden to the family. Not only are medical ­doctors contacted due to persistent or recurrent headache; alternative practitioners and other health professionals are also visited. Some of them release spinal blockages; others blame the teeth or their position for the pain.

The first release of the IHS classification arbitrarily discriminated between patients with and without increased pain sensitivity of the pericranial muscles, a discrimination which turned out to be beneficial. The result of palpation of the neck muscles was helpful for discrimination. As mentioned earlier, pathophysiology of TTH is still unknown. Peripheral mechanisms seem to be of importance in sporadic and frequent episodic TTH, while the central mechanism seems to play a role in chronic TTH.

The term “rheumatic disease” has its origin in the French doctor Guillaume de Baillou (1538–1616) who comprehensively described complaints of the musculoskeletal system. While the underlying theory of humoral pathology is long outdated, common language still uses the term, subsuming diseases of most different etiologies into the rheumatic spectrum disorder. The more specific immunologically mediated rheumatoid diseases are as follows:

Many readers will miss the diagnosis of juvenile fibromyalgia syndrome (JFMS). According to the latest guidelines, that diagnosis is subsumed under “chronic pain disease with somatic and psychic factors.” The relationship between the symptoms of JFMS and psychic disorders like depression or posttraumatic distress syndrome were too obvious, which is in accordance with our clinical impression.

In paediatrics, the most frequent rheumatic diseases are reactive arthritis of different origin, frequently due to a recent infection, and juvenile idiopathic arthritis (JIA). The etiology of the latter is still obscure. It may well be that JIA comprises different yet unknown diseases. It goes without saying that causal treatment of JIA is not yet available. An important prerequisite for successful therapy is the early diagnosis and transfer of the patient to physicians with experience in the treatment of JIA. Only by doing so can early and effective treatment of both the inflammatory reaction and the pain be given. Effective control of any underlying disease and – if necessary – induction of remission, avoidance of joint contractures, or destruction leading to persistent physical disabilities as well as avoidance of impaired growth resulting in axial malposition are the main goals of quality rheumatologic treatment. Next to drug therapy, it is of utmost importance that the patient keeps moving and under no circumstances submits to passive pain control. Otherwise there is a high risk that the acute pain will become chronic, developing further into chronic pain disorder with somatic and psychic elements (called pain amplification syndrome by rheumatologists; see also Sect. 3.1).

Successful therapy should allow children a somatic and psychic development free of major disturbances. On an eleven-point scale, JIA patients rate their disease-related quality of life worse than their general quality of life (Feldman et al. 2000). Paediatric JIA patients score their quality of life lower than their healthy peers (Manschwetus 2003). Based on several JIA studies and the JIA national guidelines of the German scientific medical societies (AWMF), early diagnosis (within 1–2 months) and the assessment of disease activity are essential to rheumatologic therapy. To this end, there are various validated scales (i.e., PED ACR) available. Drug therapy should comply with the latest version of national or international guidelines.

Of special importance is physiotherapy and ergotherapy given by experienced and specifically trained therapists with the aims of maintenance or recovery of normal joint mobility, avoidance of contractures, stretching and activation of muscles, strengthening of muscles, and facilitation of physiological movements in order to avoid the development of relieving postures or false posture. In case of good disease control, participation in sports at school and other sporting activities is allowed and should be encouraged(!), since graded physical exposure has a positive effect on development and coping with the illness, and the risk of social isolation is minimized. As soon as acute inflammation has receded, training should be resumed, but only after the type of sports and training intensity are individually determined, since in inflamed joints sports that stress the joints may provoke an acceleration of destructive processes and lead to irreversible cartilaginous defects.

Blunt joint trauma and bruises primarily provoke an inflammation response similar to that seen in other types of arthritis. Both the vasoactive substances released by tissue damage and pain lead to locally increased blood flow and local edema. An injured joint may accumulate an increased amount of fluid (effusion). Local cooling of the joint will depress pain, inflammatory response, and edema. Nonsteroidal ­antirheumatics will also depress the inflammation. Skeletal or ligamental injuries need to be diagnosed as soon as possible to allow for targeted therapy. Any necessary immobilization should be as short as possible. If impairment is observed under immobilization, one should critically reevaluate therapy. One should be aware of thrombosis as well as nerve compression. When the acute injury is healed, the aim is to mobilize the joint as soon as possible in order to avoid the development of relieving posture or inappropriate straining of a joint.

CRPS may develop after all types of trauma to the distal parts of the extremities. By definition, symptoms do not follow the course of peripheral nerves or spinal roots. In extremely rare cases, the symptoms may spread to other extremities. The diagnosis is made according to the clinical picture since there are no other suitable diagnostic methods available. The diagnosis is by exclusion. Nuclear magnetic resonance (NMR) tomography, quantitative sensory testing (QST), or skeletal scintigraphy may be helpful. Complex regional pain syndrome develops with variable latency after injury of an extremity, i.e., trauma, or even diagnostic or therapeutic procedures, independent of type or grade of injury. Even a minor trauma may induce CRPS.

If the injury causes damage to a peripheral nerve and CRPS develops, the disease is named CPRS type II. If there is no nerve lesion present, it is CPRS type I. The International Association for the Study of Pain (IASP) defined sensitive and specific diagnostic criteria (Baron 2004; Harden et al. 2007).

Generally, any findings assessed by a doctor are more important than the subjective symptoms as described by the patient. A sentinel characteristic of CRPS is that symptoms are not confined to the area of the injured nerve but tend to generalize, affecting the whole extremity.

The “Budapest” criteria of CRPS (Harden et al. 2007) are given below:

General definition of the syndrome:

CRPS describes an array of painful conditions that are characterized by a continuing (spontaneous and/or evoked) regional pain that is seemingly disproportionate in time or degree to the usual course of any known trauma or other lesion. The pain is regional (not in a specific nerve territory or dermatome) and usually has a distal predominance of abnormal sensory, motor, sudomotor, vasomotor, and/or trophic findings. The syndrome shows variable progression over time.

To make the clinical diagnosis, the following criteria must be met:

For research purposes, diagnosis should be applied when there is at least one symptom in all four symptom categories and at least one sign (observed at evaluation) in two or more sign categories.

Skin temperature is measured with suitable tools. All other symptoms are judged clinically. In order to answer point 4, especially the presence of diseases that can imitate CRPS must be excluded: rheumatic diseases, inflammation (i.e., infectious arthritis of any kind, postsurgical infections, polyneuritis, or radiculitis), thrombotic affections, compartment syndrome, or nerve compression syndrome. To this end, the patient should undergo biochemical investigations. It is impossible to diagnose CRPS exclusively by means of laboratory investigations like CRP or erythrocyte sedimentation rate.