Complex Regional Pain Syndrome
According to the International
Association for the Study of Pain the general definition of the syndrome is that CRPS describes an array of painful conditions that are characterised by a continuing regional pain that is seemingly disproportionate to any known trauma.
It has the distinction of being described as the most painful long-term condition of any that have been tested scoring 42 out of a possible 50 on the McGill Pain Scale.
The condition that is now known as CRPS was originally described during the American Civil War by Silas Weir Mitchell. He noted that some people with injuries developed very significant pain out of proportion to the initial insult. In
the 1940s the term 'reflex sympathetic dystrophy' came into use to describe the condition. In 1959 Noordenbos observed causalgia in patients suggesting nerve damage. In 1993 a specialist consensus workshop held in Orlando, Florida, provided the
umbrella term Complex Regional Pain Syndrome with causalgia and reflex sympatheticity dystrophy and sub-types.
There are a number of criteria that have been published. Two criteria of the CRPS1 diagnosis are the Bruehl’s criteria and the Veldman’s criteria. Orthopaedic surgeons are more likely to use the Atkins'
The criteria accepted and devised by the International Association for the Study of Pain is referred to as the Budapest criteria. The modified clinical diagnostic
criteria for CRPS by the IASP are:-
continuing pain which is disproportionate to the inciting event;
must report at least one symptom in 3 of the 4 following categories:
of hyperaesthesia and/or allodynia;
vasomotor: reports of temperature asymmetry and/or skin colour changes and/or skin colour asymmetry;
pseudomotor/oedema: reports of oedema and/or sweating asymmetry;
motor/trophic: reports a decreased range of movements and/or motor dysfunction (weakness, tremor, dystonia (and/or trophic changes)), hair, nails, skin;
must display at least one sign at time of evaluation in 2 or more of the following categories:-
Sensory: evidence of hyperalgesia (pinprick) and/or
allodynia (to light touch and/or deep somatic pressure and/or joint movement);
Vasomotor: evidence of temperature asymmetry and/or skin colour changes;
Pseudomotor/oedema: evidence of oedema and/or sweating changes and/or sweating asymmetry;
Motor/trophic: evidence of decreased range of movement and/or motor dysfunction (weakness, tremor, dystonia (and/or trophic changes)), hair, skin, nails;
there is no other diagnosis that better explains the symptoms and signs.
The florid CRPS is spectacularly painful, but can be missed by some treating practitioners. The syndrome is regional by definition and therefore tends to affect one of the limbs although
it can spread to other limbs and can become widespread. The telltale signs that should set alarm bells ringing are difference in temperature between limbs, difference in colour, difference in hair/nail growth, exquisite tenderness, swelling, decreased
range of movement, clawing and dystonia.
There is no set test for CRPS and it diagnosed purely on a clinical basis.
CRPS can strike at any age. Statistically, it is three times more frequent
in females than males. The mean time appears to be age 42 but it can occur at any time. In my practice, I have seen cases involving septuagenarians and also children.
Although CRPS does commence regionally it is important to know that it can spread and therefore provisional damages should be claimed in the event that this occurs.
It is not really understood why some people develop CRPS. It appears to be associated
with dysregulation of the central nervous system and autonomic nervous system resulting in multiple functional loss impairment and disability.
CRPS Type 1 formerly known as reflex
sympathetic dystrophy tends to be without nerve lesions whereas Type 2, formerly known as causalgia, has a nerve involvement and tends to be more difficult to treat.
research demonstrates that CRPS is a systemic disease. Demonstrable changes in peripheral nerve fibres indicate central nervous system involvement. Potentially any organ could be affected including the bowels. Recent work has shown how CRPS can spread to the stomach.
Numerous types of treatment are put forward such as pharmacological, CBT, mirror boxtherapy, graded motor imagery, tactile
discrimination training, local anaesthetic/blocks, Botox injections, spinal chord stimulators and capsacin cream. In extreme cases, amputation has been tried. Anecdotal reports state that, on average,
half the patients will have a resolution of pain while half will develop phantom limb pain.
Prognosis is improved if treatment is begun early, ideally within three months of the first symptoms. If treatment is delayed the disorder can quickly spread to the entire limb and changes in bone, nerve and muscles may
become irreversible. The prognosis is not always good. Once CRPS has been diagnosed the likelihood of it resurfacing after going into remission is significant.
I have seen cases where there has been a development of CRPS after trauma and resolution. Further treatment by way of surgery has been suggested but the advice has been given to avoid it as it is possible to trigger the CRPS again. This is a very
relevant issue in terms of long-term treatment.
Running a CRPS Case
correct specialist for CRPS is a pain expert and/or a neurologist with a special interest in chronic pain cases. Inevitably, there will be a psychological component due to the experience of unremitting pain. In such cases it is prudent to obtain
a report from a psychologist/psychiatrist with a special interest in pain.
In many cases an OT/care report is necessary. In some cases an accommodation
expert is required to comment on housing needs.
In my practice I have seen many cases of CRPS. At best, they are manageable, at worst the Claimant suffers daily unremitting severe pain. It can lead to dystonia. On occasions my clients
have asked for amputations, as they are unable to cope with the pain. On one occasion the dystonia was so severe that an amputation was necessary to give any level of function. With this level of pain and lack of function, it is
inevitable that this leads to psychological problems which in turn feed into the pain.
There is no explanation as to why this condition develops, but it is now accepted
by the Courts that it is likely that it is triggered by an insult which includes trauma. This can be extremely modest. In one case that I had a lady who suffered mild bruising
to her foot which developed into CRPS and she effectively lost the use of her leg. Her claim was settled for £1m.
The Courts do recognise that this is an extremely
painful and debilitating condition and make awards to compensate the victim accordingly.