Complex regional pain syndrome (Reflex sympathetic dystrophy)

Dr Nilesh Nolkha Senior Resident, Department of Rheumatology KGMC, Lucknow

Introduction & history Epidemiology Pathophysiology Signs, symptoms and stages of RSD Complications, is it a systemic disease?, RSD spread Diagnosis Investigations Management Take home points

Introduction
Case 1 - A 20 year old female with history of right sided radiculopathy secondary to PID around a year back presented with history of pain and swelling right ankle - Subsequent course, pain in right shoulder and elbow - Injection pamidronate - Right temporomandibular joint pain and tinnitus Case 2 - Middle age adult male with history of minor shoulder trauma complaining of pain and dystonica episodes involving right shoulder.

History

SILAS WEIR MITCHELL 1864

long after the efects of wound have gone..neuralgic symptoms lingermany carry final reminder of battlefield "its favorite site is the foot or hand...the palm of the hand or palmar face of the fingers, and on the dorsum of the foot; scarcely ever on the sole of the foot or the back of the hand When it first existed in the whole foot or hand, it always remained last in the parts referred to...if it lasted long it was finally referred to the skin alone. The part itself is not alone subject to an intense burning sensation, but becomes exquisitely hyperaesthetic, so that a touch or a tap of the finger increases the pain."

SILAS WEIR MITCHELL 1864

"As the pain increases, the general sympathy becomes more marked. The temper changes and grows irritable, the face becomes anxious, and has a look of weariness and suffering. The sleep is restless, and the constitutional condition, reacting on the wounded limb, exasperates the hyperaesthetic state, so that the rattling of a newspaper, a breath of air...the vibrations caused by a military band, or the shock of the feet in walking, gives rise to increase of pain.

More than 150 years since first descriptionstill

RSD remains one of the most misunderstood, underdiagnosed and overdiagnosed conditions

CRPS is one of most painful disease Mcgill pain scale

WHATS IN A NAME?
Algodystrophy Major causalgia Mimo causalgia Reflex neurovascular dystrophy Morbus Sudeck Sudecks atrophy Minor causalgia

Post-traumatic Postdystrophy traumatic algodystrophy Pourfour du Petit Syndrome Peripheral trophoneuros is

Neurovascular dystrophy

The big names in RSD

1. Dr H Hooshmand 2. Dr Robert Schwartzmann 3. Dr Stephen Bruehl

4. Dr Veldman
5. Dr Ralph Baron

Epidemiology
Common in younger adults - Mean age 41.8 years - Mean age of injury 37.7 years Females > males 2-3:1 Usually involves a single limb in early stage Duration from injury to onset can vary usually days to months Genetics Children

Pathophysiology
What is sympathetic system? What is its role in normal physiology? Sympathetically maintained pain (SMP) is not equal to RSD, pain is later sympathetically independent pain (SIP) SMP eg. chronic neuropathic pains Watershed zones- why peripheries are involved more? Mild trauma No RSD a misconception Why RSD can be aggravated by ice, inactivity and use of assisted devices?

Autonomic dysfunction
Pathological sympatho-afferent coupling: Peripheral nociceptors develop adrenergic sensitivity (mainly alpha-2 receptors) such that tonic sympathetic efferent activity leads to their activation Painful impulses via these nociceptors maintain the central nervous system in a sensitized state Painful and non-painful stimuli to the affected limb result in hyperalgesia and allodynia, respectively Catecholamine levels, however, are actually lower in the affected extremity, thus, it is not a problem of excessive sympathetic nerve output

Neuroplastic changes within the CNS

Studies using functional brain imaging in patients with CRPS have found a significant degree of cortical reorganization in the central sensory and motor cortices The amount of reorganization positively correlates with the extent of pain intensity The areas of reorganization were found to be reversible in adequately treated patients

CRPS hence a better term than RSD ?

Clinical features, types and stages of CRPS

RSD is a syndrome with multiple manifestations, it is not necessary a diagnosis of exclusion

1. Pain: constant, burning pain, and in some forms at times during the course of the disease, stabbing type of pain. The pain is relentless and is invariably accompanied by allodynia (even simple touch or breeze aggravating the pain) and hyperpathia (marked painful response to even a simple stimulation). 2. Spasms in the blood vessels of the skin- cold extremity. The muscle spasms result in tremor, movement disorders such as dystonia, flexion spasm, weakness and clumsiness of the extremities, and tendency to fall.

3. Inflammation It may be in the form of swelling (edema), skin rash (neurodermatitis), inflammatory changes of the skin color (mottled or purplish, bluish or reddish or pale discolorations), tendency for bleeding in the skin, skin becoming easily bruised, inflammation and swelling around the joints as well as in the joints (such as wrists, shoulders, knee, etc.) which can be identified on MRI in later stages, and secondary freezing of the joints. 4. Insomnia and emotional disturbance Sympathetic sensory nerve fibers terminate in"limbic system".

RSD and psyche Facts and fallacies

RSD is a psychiatric illness RSD causes psychiatric illness Psychiatric illness and personality disorders are predisposing

factors for RSD

Psychological factors modify the course of RSD Adjustment and function in RSD are worsened by maladaptive behavior

CRPS types
Type 1 occurs after an illness or injury that did not directly damage a nerve in the affected area Type 2 follows a distinct nerve injury

Stages of CRPS
Stage I (acute 3months): A sympathetic dysfunction with thermatomal distribution of the pain. Redness and warmth. In stage one, the pain is usually SMP in nature. Dependent edema and hyperhydrosis.

Stage II (subacute 3-12months): The dysfunction changes to dystrophy manifested by edema (more constant), neurovascular instability with fluctuation of livedo reticularis and cyanosis causing change of temperature and color of the skin in matter of minutes.

 Stage III (atrophic->12 months): The dystrophic changes include bouts of hair loss, ridging, dystrophic, brittle and discolored nails, skin rash, subcutaneous bleeding, neurodermatitis, and ulcerative lesions. Stage IV: Identifies the final stage of RSD / CRPS manifested by: Failure of the immune system, reduction of helper T-cell lymphocytes and elevation of killer T-cell lymphocytes. Orthostatic complaints. Intractable generalized extremity and visceral edema involving the abdomen, pelvis, lungs, and extremities .The same organs may eventually be subject to multiple abscesses due to failure of immune function. Ulcerative skin lesions. High risks of cancer and suicide.

Complications Is it a systemic disease? RSD spread

Is it a systemic disease Yes it is

Constitutional complaints Cardiac Gastrointestinal Urological Neurogenic edema Dermatology Neuropshychological What is pathophysiology Cytokines?

Spread of RSD

Patterns of spread - Contiguous - Independent spread - Mirror image spread Whole body RSD

Red flush & Jump sign

Diagnosis & Investigations (Diagnosis is clinical, No single test confirmatory, Investigations at best supportive)

Thermography

Xray & Triple phase bone scan

QSART (quantitative sudomotor axon reflex test) MRI NCV / EMG Dexa scan

Management
- A multidisciplinary approach utilizing pharmacotherapy, physical therapy and psychological therapy is most appropriate - No one doctor can treat RSD completely - Treat aggressively - No patient is alike - Do no harm

Individualize!

No protocol works for all patients with CRPS. It is dependent on current pain level, symptoms, and tolerance

Non pharmacological therapy

Physiotherapy Hydrotherapy Propriotherapy Biofeedback and mirror box

Diet

Pharmacological
Topical- DMSO (dimethylsulfoxide), topical steroids, epsom (magnesium) salt dressings Oral corticosteroids Calcitonin Antidepressants (SSRI) Anticonvulsants Opiods Bisphosphonates Mannitol

 Clonidine and yohimibine Baclofen, botulinum, clonazepam Ketamine (most recent development)

Interventional
Sympathetic blocks Epidural blocks (infusion pumps) Intrathecal baclofen Sympathectomy (surgical and chemical) Spinal cord stimulators Disadvantages

Avoid
Surgery or procedures during active CRPS Alcohol Excessive assistive devices, ice or immobilization

 Pregnancy and CRPS Surgery and recurrence Preventive measures ?

Take home points.Do Nots

miss RSD
say trivial injury cannot cause RSD say its all in your head ignore systemic RSD complaints ignore physical therapy use ice, strong narcotics ad assistive devices excessive surgical procedures attribute RSD to carpel tunnel syndrome, consider other way around

Thank you