Major causalgia is usually caused by an open wound involving a mixed peripheral nerve. Pain develops that typically demonstrates two characteristics: (1) it is made worse by light touch, and (2) there is an element of constant pain, often burning in character. Minor causalgia is a poorly defined group of syndromes, and usually indicates psychopathology, often premorbid and not just reactive to the pain. The treatment for major causalgia is prompt sympathetic nerve block and/or surgical sympathectomy. The treatment for minor causalgia is not surgical, but psychotherapy, often best administered within a group setting, and occasionally needing a multidisciplinary pain team approach, especially for cases with reflex sympathetic dystrophy. Deafferentiation means loss of peripheral afferent input, believed to lead under many circumstances to central hyperirritability or excitatory states.
LETM is defined as an inflammation affecting the spinal cord and extending over three or more vertebral segments [1]. Seemingly, this definition is fully clear in terms of radiological characteristics, but far more ambiguous in terms of clinical presentation. Even though most of the related cases in the literature presented with symptoms of long tract dysfunction, more insidious presentations with causalgia were also conducted.
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Reflex sympathetic dystrophy syndrome RSD is a clinical syndrome with three cardinal symptoms and signs; those are pain, edema and vasomotor dysfunction. The typical triggers are peripheral trauma, surgery and, to a lesser degree, central triggers like stroke and spinal cord injury, tumors and myelitis. In these cases, causalgia develops insidiously, months to years following the inducing pathology [2]. Interestingly, in 2011, Michael J. Regan and Jeetandera Rathi published a case report of cervical spinal cord tumor presented with causalgia [3]. Similarly, Asha Das conducted a case of a 53 year old female with bilateral optic neuritis presented with symptoms of left upper limb causalgia simultaneously with cervical spinal cord demyelination plaque which progressed later into syrinx [4].
Overwhelming pain is usually the mainstay feature of RSD. However, the pain in this patient was mild and limited to the acute phase of presentation and the other features accumulated during the following period. Although is not inherent in the presumed pathology of causalgia, superficial sensation is typically affected in most of the cases [6]. Hence, intact superficial sensation in the affected limb may represent another caveat in this case and may had a role in the absence of pain as a cardinal manifestation of RSD in this patient.
In cases of incomplete presentation of arthritis, causalgia should be suspected and serial physical monitoring is recommended. In spite of its scarcity, autonomic dysreflexia may complicate highly located spinal lesions. Hints of autonomic disturbance may have an alarming role in this context and it may be beneficial to be checked early in the course.
In this review we will focus on the condition of pediatric chronic peripheral pain, more commonly referred to as pediatric complex regional pain syndrome (CRPS). Various nomenclature are used to describe this condition, including complex regional pain syndrome (CRPS), reflex sympathetic dystrophy, reflex neurovascular dystrophy, causalgia, and localized idiopathic pain. Matles was the first to describe a case of reflex sympathetic dystrophy in a child [1].
CRPS is a condition of chronic peripheral pain, usually of a distal extremity. This syndrome is characterized by spontaneous or stimuli-induced pain, which is amplified to a very high visual analog scale (VAS) score, disproportionate to the actual incident trauma/stimulus, in the presence of a wide variety of autonomic and motor disturbances. CPRS is divided into two types. CRPS type I, previously known as reflex sympathetic dystrophy, usually develops after a preliminary event; usually minor a trauma or fracture, without any significant nerve injury. In contrast, CRPS type II, which is very rare in childhood, previously named causalgia, is induced by partial injury of a nerve or one of its major branches [2]. This review summarizes the current information regarding the epidemiology, etiology, diagnosis, treatment and prognosis of pediatric CRPS.
Complex regional pain syndrome (CRPS), formerly known as "reflex sympathetic dystrophy," is a chronic neurological disorder characterized by disabling pain, swelling, vasomotor instability, sudomotor abnormality, and impairment of motor function. The disorder usually develops after minor trauma or surgery. No specific diagnostic test is available and, hence, diagnosis is based mainly on history, clinical examination, and supportive laboratory findings. This review gives a synopsis of CRPS and discusses the principles of management based on the limited available literature in the area. A literature search was conducted using electronic bibliographic databases (Medline, Embase, Pubmed, CENTRAL) from 1970 to 2006. Keywords complex regional pain syndrome, reflex sympathetic dystrophy, neuropathic pain, and causalgia were used for the search. Relevant articles from the reference lists in retrieved articles were also studied. There were 3,771 articles published in the area. Seventy-six randomized controlled trials were identified. Most studies were on the role of sympathetic blockade in the treatment of CRPS (n = 13). The role of sympathectomy is unclear, with some studies showing transient benefit and others showing no beneficial effects, with most studies containing only a small number of patients. Nine studies were on bisphosphonates or calcitonin. Studies involving bisphosphonates showed benefit, but studies involving calcitonin showed no definite benefit. Four studies were on cognitive behavioral therapy, physiotherapy, or occupational therapy, all of which demonstrated a potential beneficial effect. Three studies on spinal cord stimulation and two studies each on acupuncture, vitamin C, and steroid all showed a potential beneficial effect in pain reduction. The remaining studies were on miscellanous therapy or combination therapy, making it difficult to draw any conclusions on the effect of treatment. There is very little good evidence in the literature to guide treatment of CRPS. Early recognition and a multidisciplinary approach to management seems important in obtaining a good outcome. Treatments aimed at pain reduction and rehabilitation of limb function form the mainstay of therapy. Comorbidities, such as depression and anxiety, should be treated concurrently.
Complex regional pain syndrome is separated into two categories. Type 1, previously known as reflex sympathetic dystrophy (RSD), most often occurs in the setting of illness or injury without direct nerve damage and is the more commonly diagnosed type of CRPS. Type 2, otherwise known as causalgia, is characterized by the presence of known nerve damage related to a specific nerve injury, often being seen after surgery or trauma and typically exhibiting more neuropathic pain symptoms including allodynia, dysesthesia, and hyperalgesia [1]. As mentioned above, CRPS is a clinical diagnosis given the often overlapping features of nociceptive and neuropathic pain symptoms. The Budapest criteria contain four diagnostic components to help aid in making a diagnosis of CRPS: sensory, vasomotor, pseudomotor/edema, and motor/trophic [5]. If a patient has all four of these diagnostic components, then the diagnosis of CRPS becomes more certain (sensitivity 0.95, specificity 0.81) [5]. 2ff7e9595c
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