Have you ever had a fracture? If you have, then you are probably aware of how difficult it can be to recover. Today, we have Hannah Pickering, a neuroscientist in Sydney, who provides us with an update on complex regional pain syndrome, a poorly understood neurological disorder that may occur with limb immobilisation. Over to you, Hannah.

Complex Regional Pain Syndrome (CRPS) was formally known as causalgia, from the Greek word, kausis, -which means fire. It was later termed reflex sympathetic Dystrophy. While there is debate over the first description of the condition, Mitchell et al. (1) described the presentation of severe burning pain after tissue injury, in soldiers who had fought in the civil war. In his description, he highlighted the exaggerated nature of the pain in relation to the injury, which to date remains a primary feature of the current CRPS classification.

CRPS is a chronic neuropathic pain disorder, which occurs primarily after limb trauma and can be classified as CRPS I – when there is no nerve lesion evident, or CRPS II when a definable nerve lesion is present. A history of injury, most commonly fracture or soft tissue injury, followed by subsequent immobilisation are the most consistently associated factors with a diagnosis of CRPS (2, 3). Recent observational studies suggest that 47% of patients identify physician-imposed immobilisation (4), however, the frequency is likely to be higher, as immobilisation due to disuse and protective guarding has not yet been investigated.

CRPS is characterised by autonomic motor and sensory abnormalities. Patients can suffer an array of symptoms including burning pain, inflammation, sensory abnormalities (5), autonomic (2), trophic, and motor disturbances (2, 3). Hyperalgesia (increased pain from a stimulus that normally provokes pain) and allodynia (pain due to a stimulus that does not normally provoke pain) are frequently reported in patients suffering CRPS (2, 3, 6). Some patients also demonstrate clinical signs of oedema (2, 5).

The aetiology of CRPS to date remains unclear with a variety of possible mechanisms being evaluated. However a recent review identified three major pathophysiological pathways, namely,  abnormal inflammatory mechanisms, vasomotor dysfunction and maladaptive neuroplasticity (7) which independently or in combination, can result in CRPS. Research from Drummond and Finch, published in JNNP, raised the additional possibility of a central nervous system contribution to the amplification of pain in CRPS, which may lead to further disability and explain the condition’s resistance to treatment (8).

Interestingly, disturbances in the way one perceived their own body are frequently reported in chronic pain conditions. Mosley and colleges demonstrated that sixty-three percent of patients with CRPS identified with an expanded image of their affected limb compared to only 7% of controls (9)  Additionally, studies using magnetoencephalography (MEG) revealed that the somatosensory representation (or a map of the body surface) of the limb affected by CRPS was significantly smaller than the healthy limb(10). Furthermore, reduction in CRPS pain through physiotherapy and pharmacological intervention, was correlated with recovery from cortical representation (11). This relationship raised the possibility that S1 changes mediated the increased perceived limb size experienced by CRPS patients (10), although the mechanisms that brought about this change remain unclear.

Recent evidence suggests that chronic pain distorts the processes of motor imagery.Recognition of whether a viewed image is of a left or right side is classified as a motor imagery task. Correctly identifying laterality, involves mentally moving one’s own body part to adopt the position of a pictured body part. It depends on an working intact body schema, brain-grounded maps of the body that are used for planning executing movements and are constructed, in-part, from real-time proprioceptive input (9). Studies have shown that CRPS patients take longer to recognise images corresponding with their affected limb (12).

In terms of treatment, the primary aims are first controlling the pain, followed by rehabilitation of the affected limb. An array of treatments options are utilised , often in combination and include; physical therapy (Graded motor imagery and mirror box therapy), pharmacologic therapies, anaesthesia techniques such as nerve blocks, and more recently neuromodulation. One focus of current research targets potential effects of high frequency spinal cord stimulation as a novel treatment approach in this condition.

References:

1)      Mitchell SW, Morehouse GR, Keen WW. Gunshot Wounds and Other Injuries of Nerves. Vol: 100. Philadelphia: J. Lippincott and Co.; 1864: 11.

2)      Birklein F, Riedl B, Sieweke N, Weber M, Neundorfer B (2000). Neurological findings in complex regional pain syndromes–analysis of 145 cases. ActaNeurolScand101,262-269.

3)      Birklein F (2005). Complex regional pain syndrome. J Neurol 252,131-138.

4)      Allen G, Galer BS, Schwartz L (1999). Epidemiology of complex regional pain syndrome: a retrospective chart review of 134 patients. Pain.  80,539-544.

5)      Huge V, Lauchart M, Forderreuther S, Kaufhold W, Valet M, Azad SC, et al. (2008). Interaction of hyperalgesia and sensory loss in complex regional pain syndrome type I (CRPS I). PLoS One. 3,2742.

6)      Terkelsen AJ, Bach FW, Jensen TS (2008). Experimental forearm immobilization in humans induces cold and mechanical hyperalgesia. Anesthesiology. 109,297-307.

7)      Marinus J, Moseley GL, Birklein F, Baron R, Maihöfner C, Kingery WS, van Hilten JJ. (2011) Clinical features and pathophysiology of complex regional pain syndrome.Lancet Neurology. 10(7):637-48.

8)      Drummond P and Finch PM. Persistence of pain induced by startle and forehead cooling after sympathetic blockade in patients with complex regional pain syndrome. J Neurol Neurosurg Psychiatry 2004;75:98-102

9)      Moseley GL (2005). Distorted body image in complex regional pain syndrome. Neurology. 65,773.

10)   Maihofner C, Handwerker HO, Neundorfer B, Birklein F (2003). Patterns of cortical reorganization in complex regional pain syndrome. Neurology. 61,1707-1715.

11)   Maihofner C, Handwerker HO, Neundorfer B, Birklein F (2004a). Cortical reorganization during recovery from complex regional pain syndrome. Neurology. 63,693-701.

12)   Moseley GL (2004). Why do people with complex regional pain syndrome take longer to recognize their affected hand? Neurology. 62,2182-2186.

The old paternalistic way of patient care is out the door as Robin Williams stars as Hunter ‘Patch’ Adams in this Oscar nominated film. Laughter is the best medicine is his mantra as he parades around the hospital in the best costumes you’ll ever see while breaking all health and safety regulations. Funny man Robin Williams portrays Patch as an awkward man whom everyone cannot help but love. The film leaves you crying, laughing and going through the complete spectrum of human emotions.

The plot

Patch commits himself into an inpatient psychiatric unit for depression and suicidal intentions. Whilst there he finds himself appalled by the false ‘how does this make you feel?’ treatment he gets from his psychiatrists. As the class clown on the wards, he inadvertently improves the quality of life of his co patients, by lifting their spirits and becoming a confidant. Patch realizes that he is at his best when helping others to find solutions to their problems and difficulties. He drastically discharges himself against medical advice and applies to medical school!

On getting an ‘I’m going to make doctors out of you’ speech by the dean of his medical school, he decides that he doesn’t want to be the old school sort of doctor. He refuses to conform to the ideals of doctors being better and more intelligent that their patients. As Patch simply put it, ‘the patient is the doctor too’ and they know what is best for them, defining autonomy with one easy phrase.

Patch took it on as his duty to attack the enemy which he felt was the indifference he felt was present in the treatment of patients, by his superiors. He paraded the hospital giving dying patients the lift they needed while also trying to fulfil their dying wishes whether it was a swimming pool full of spaghetti or a mock safari with animal balloons. Patch stuck to his belief that a happy patient is a healthy patient and was willing to give the evidence for it as change in catecholamine levels when you laugh.

While on his quest he realized that the only way to make his vision a reality was by opening a free clinic and recruited fellow medical students who helped him start it up, in an abandoned cottage. He named the clinic ‘Gesundheit Institute’ meaning the good health institute, and with time, he acquired a 320 acre property in West Virginia where the dream still lives.

But his journey through medical school was not as straight forward as one would expect for a regular student, he faced loss and had the struggles most medical students face. But his blatant disregard for authority and refusal to conform created a huge problem and was almost kicked out of medical school twice.  But Patch had a dream and ran with it, he wanted to be a doctor, but most importantly, he wanted to be a source of hope to people. He wanted the hospital to be a home, a place of comfort and safety rather than fear and grief.

Perhaps the film took the concept rather over the top, you really don’t need to wear squeaky shoes and a red nose to make children laugh, perhaps a little bit of peek-a –boo will be fine, but the message is still quite clear. Laughter and a bit of joy really do make people feel better.

The real Patch

Patch Adams describes himself as a clown doctor, but more than this, he has committed his life to studying what makes people happy. Patch encourages a holistic approach to the care of all patients. Today, the Gesundheit institute offers free medical care to all its patients, complimentary medicine and accepts students for electives as well. It would seem that one defiant medical student who chose to start a revolution is indeed changing the world one patient at a time.

Your Choice

In a mix of laughter and loss, Patch makes us think about what is really important, and asks the question we more often than not have a steadfast answer to. Why did you go to medical school? Do you really want to help people? Or do you just want to be another figure head?

Patch inspires us to go that extra mile, to really scratch the surface and find out what really makes our patients tick and maybe we will be able to find the root of their problems and the reason behind their complex presentations.

So the choice is yours, what will you be prescribing for your patients a good helping of tender loving care or will you stick to the BNF?

REFERENCE:

1. Patch Adams. Universal Studios 1998

By Dr Morounkeji Ogunrinde, Hull York Medical School, University of York