Sudeck’s disease: what is CRPS?

Sudeck's disease: what is CRPS?

CRPS, often called Morbus Sudeck, is the abbreviation for the English term Chronic Regional Pain Syndrome. In German, it speaks of complex regional pain syndrome. After an arm or leg injury, people with CRPS experience pain that is unusually long and excessive for the cause. The movement and perception of the arms and legs are also restricted. What is this disease, and how is it treated? You can find out here.

CRPS: many names – one disease

Other names for CRPS are reflex dystrophy, Sudeck’s dystrophy, algodystrophy, or reflex sympathetic dystrophy. Dystrophy is a malformation, and CRPS is a nerve malformation.

The common term Sudeck’s disease (M. Sudeck) only describes CRPS type I. However, Sudeck’s disease is often equated with CRPS. This name also gives rise to designations such as Sudeck’s disease, Sudeck’s disease or Sudeck’s disease.

 

How does Sudeck’s disease develop?

The complex regional pain syndrome usually occurs on the arms or legs, i.e. the extremities. For example, it can affect the foot, hand or finger. Symptoms are always preceded by a trigger, for example:

  • Injuries, especially near the joint
  • nerve injuries
  • certain physical therapy exercises
  • broken bones
  • therapeutic or diagnostic interventions, for example, operations or general anaesthesia
  • constricting associations

CRPS is rare, occurring as a complication in only two to five per cent of all extremity injuries. It doesn’t matter how severe the injury was.

The causes of Sudeck’s disease are still unclear. However, it is suspected that parts of the nerves become inflamed. This may lead to sensitization and increased sensitivity to pain (hyperalgesia).

How is Sudeck’s disease diagnosed?

The most important symptom of CRPS is the already mentioned chronic, i.e. ongoing, pain, which cannot be explained by the original injury. In addition, the following symptoms may also occur:

  • Hypersensitivity to pain (hyperalgesia)
  • Hypersensitivity to touching the skin (hyperaesthesia)
  • an actually non-painful stimulus triggers pain (allodynia)
  • unterschiedlich warme Körperhälften und Färbung der Haut
  • Körperhälften schwitzen unterschiedlich viel
  • Schwellungen am Körper durch Wassereinlagerungen
  • Einschränkung von Beweglichkeit und Kraft, unwillkürliches Zittern der Hände (Tremor)
  • Störungen im Gewebswachstum (trophische Störung)
  • ungleichmäßiger Nagel- und Haarwachstum, wenn man die Körperhälften vergleicht
  • Ausdünnen der Haut an bestimmten Stellen (Hautatrophie), erkennbar durch hervortretende Venen und Verfärbungen der Haut

 

CRPS: Welche Typen gibt es?

A distinction is made between two possible types or types of CRPS:

  • On the one hand, CRPS type I, in which there is no evidence of nerve injury). This type used to be called Sudeck’s disease or algodystrophy. In the World Health Organization’s (WHO) International Classification of Diseases, abbreviated as ICD-10, the classification code is G90.5.
  • On the other hand, CRPS type II (formerly also causalgia) is spoken of if there is evidence of nerve injury (the ICD-10 code is G90.6-).

Verlauf: Einteilung in Stadien

CRPS wird in drei Stadien unterteilt, die aber nicht unbedingt nacheinander durchlaufen werden müssen1:

  • Stadium I, auch entzündliches Stadium genannt, äußert sich als brennender Ruheschmerz. Es treten entzündliche Schwellungen und Fehlregulierungen der Körpertemperatur auf.
  • Stadium II ist das dystrophe Stadium, das bedeutet, es kommt zu einer mangelhaften Nährstoffversorgung des Gewebes. Hier lassen die Schmerzen nach, die Gelenke beginnen, zu versteifen, Knochen entkalken, Muskeln bilden sich zurück (man bezeichnet das als Atrophieren) und die Haut wird kalt und blass.
  • Stadium III, auch atrophes Stadium, beginnt, sobald keine Schmerzen mehr empfunden werden. Die Gliedmaßen verlieren ihre Gebrauchsfähigkeit. Muskel-, Binde- und Fettgewebe beginnen, sich zurück zu bilden, die Gelenke versteifen schlussendlich komplett und die Haut wird dünn und glänzend.

Manche Autoren definieren ein viertes Stadium als Störung des Immunsystems, mit Wasseransammlungen und extremen Schwankungen der Körperkerntemperatur.

Wie erfolgt die Diagnose bei CRPS?

Für die CRPS-Diagnose liefert das Auftreten der typischen Symptome – vor allem die Schmerzen im betroffenen Bereich – entscheidende Hinweise. Zusätzlich können Röntgenaufnahmen Aufschluss über die Beschaffenheit der Knochen geben.

Eine andere Möglichkeit zur Diagnose ist die sogenannte Drei-Phasen-Skelettszintigraphie. Sie liefert nähere Informationen zum Blutfluss, zur Verteilung der Blutmenge und zum Knochenstoffwechsel. Hierbei wird eine radioaktive Flüssigkeit ins Blut gespritzt. Nach bestimmten Zeitabschnitten werden jeweils mithilfe eines Detektors Bilder vom Körper des Untersuchten gemacht:

  • Die ersten 60 Sekunden beobachtet man den Blutfluss zum Knochen hin (Einstromphase).
  • Nach fünf Minuten beobachtet man die Blutverteilung im Gewebe (Weichteilphase).
  • Anschließend, nach zwei bis fünf Stunden, wird die Aufnahme in den Knochen beobachtet (Knochenstoffwechselphase).2

Vor allem die letzte Phase der Drei-Phasen-Skelettszintigraphie kann den veränderten Stoffwechsel im Knochen beim CRPS darstellen. Hierbei ist zu beachten, dass die Szintigraphie das CRPS zwar bestätigen, aber nicht ausschließen kann. Liefert die Untersuchung also nicht die bei CRPS zu erwartenden Ergebnisse, kann die betroffene Person trotzdem CRPS haben.

Darüber hinaus können auch Messungen der Hauttemperatur auftretende Unregelmäßigkeiten aufzeigen.

MRT images  (magnetic resonance imaging) allow observation of the bones and a statement about water retention ( oedema ), muscles or connective tissue. An MRI can be done on a case-by-case basis to rule out other disorders, but it is not usually necessary to diagnose CRPS.

 

How is CRPS treated?

The earlier the therapy for Sudeck’s disease begins, the better the prognosis. 

Therapy for CRPS consists of: 

  • Physiotherapy and occupational therapy
  • pain management
  • drug therapy

Physiotherapy and ergotherapy are intended to restore the extremities’ mobility or prevent the symptoms from worsening.

Pain management with physiotherapy

In the acute phase, pain and swelling are primarily treated: Lymphatic drainage ( massage of the lymph nodes ), cooling wraps, carbonic acid baths or elevation of the affected limbs are used here. To relieve pain, the physiotherapist moves neighbouring joints and the cervical or lumbar spine – referred to as mobilization. 

Electrotherapies such as ultrasound or TENS (transcutaneous electrical nerve stimulation) are also used.

As the disease progresses, the affected limbs are mobilized by manual therapy. The patient’s cooperation is crucial for the success of the therapy. Strength building, such as equipment training and walking school, are also used if the legs are affected.

Occupational therapy alleviates the symptoms.

Occupational therapy often works with so-called desensitization training. Here, the person concerned is “toughened” with targeted touches of materials (soft to scratchy) that they find uncomfortable. In contrast, awareness training aims to bring back the feeling in numb areas of the skin. 

Some CRPS sufferers have trouble identifying objects by touch (stereoagnosia). There are also specific training sessions for this. Swelling is joint in CRPS and can cause significant problems for those affected. On the other hand, warm and cold baths ly, mph tapes or compression bandages, can help.

In the later course of the disease, the incorrect posture of the limbs can occur. To treat or prevent this, the affected extremities can be splinted.

 Functional therapy is an integral part of occupational therapy. For example, with proprioceptive neuromuscular training (PNF), known movements are performed against resistance to relearn healthy movement patterns. Computer-assisted treatments such as Handtutor® or biofeedback have the same goal, but those treated can carry out the therapy more independently. For example, the movements are displayed with the Handtutor® or the temperature fluctuations with biofeedback and can thus be corrected and adjusted directly by the user.

The so-called ADL training  (daily life activities) helps the patient through targeted exercises to relearn movement sequences that are important for everyday life and thus give those affected more self-confidence and security.

 

Drug therapy for CRPS

In the context of pain treatment, in addition to the painkillers Ibuprofen or Novalgin®, so-called anticonvulsants (against muscle tension), antidepressants and opioids can be prescribed for pain treatment. Pain plasters with capsaicin (the substance that causes heat in the chilli) are also used. 

In addition, in the early stages of CRPS, bisphosphonates can be used for bone formation and pain relief or glucocorticoids for severe inflammation

Psychotherapy in Sundeck’s disease

It is also possible to apply psychotherapeutic measures. CRPS is not only physically but also mentally very stressful. Pain psychotherapy is used to process experiences and to reduce stress and the associated pain.

Some CRPS sufferers can even develop neglect-like syndrome. This syndrome is a disturbance in the perception of a specific body part. Those affected then perceive this body part as less or as non-existent. Mirror therapy can be helpful for treatment.

In mirror therapy, the diseased limb is hidden behind a mirror. The healthy limb performs specific movements visible in the mirror. Meanwhile, the diseased limb is moved in parallel behind the mirror. The treated observes the mirror image of the healthy part of the body. In this way, the brain stores the movements of the reflected healthy body part as the movement of the diseased body part.

Invasive treatments for CRPS

There is also the possibility of treating CRPS using invasive methods, i.e. methods that penetrate the body: 

  • In the Sympathetic blockade, nerves are specifically anaesthetized using, among other things, a syringe filled with alcohol. This is usually done with local anaesthesia, i.e. without anaesthesia. This effect can last several weeks or months and must then be repeated.
  • Spinal cord stimulation (SCS = Spinal Cord Stimulation) is used for the targeted stimulation of nerves to achieve the “tingling feeling” created in this way. Masks are the actual pain. An electrode and a pulse generator are implanted into the spinal cord and then activated in the event of pain. This also includes dorsal root ganglion stimulation, which can achieve effects in regions such as the feet or lower legs by influencing the transmission of stimuli to specific nerve nodes, the dorsal root ganglia.

 

How long does CRPS last?

The healing time for CRPS can vary greatly. In principle, CRPS is curable, but only a tiny proportion of those affected are cured. However, only about the same few reach stage III. Most of those affected are affected by the disease to varying degrees for the rest of their lives. The pain can remain constant or recur.

It is essential for those affected to choose the right therapy. Ideally, specialists should be consulted, usually in neurology, orthopaedics or pain medicine, as they usually have experience dealing with this complex disease.

Who is at the most significant risk of developing CRPS?

Women are most commonly affected, mainly between 40 and 60. Arms and hands are most commonly affected. There is a particular risk of a fracture of the radius, one of the forearm bones.

Sudeck’s disease: the reason for disability?

Some courses of the disease are very severe, can severely limit the affected person’s professional life, and sometimes even lead to disability. Since the cause of Sudeck’s disease is still unclear, those affected can apply for a disability pension and severe disability, but whether this is successful depends on whether the report adequately takes into account the pain that occurs.

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