Gene/Protein Disease Symptom Drug Enzyme Compound
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This prospective, consecutive series describes peripheral nerve stimulation (PNS) for treatment of severe reflex sympathetic dystrophy (RSD) or complex regional pain syndrome, in patients with symptoms entirely or mainly in the distribution of one major peripheral nerve. Plate-type electrodes were placed surgically on affected nerves and tested for 2 to 4 days. Programmable generators were implanted if 50% or more pain reduction and objective improvement in physical changes were achieved. Patients were followed for 2 to 4 years and a disinterested third-party interviewer performed final patient evaluations. Of 32 patients tested, 30 (94%) underwent permanent PNS placement. Long-term good or fair relief was experienced in 19 (63%) of 30 patients. In successfully treated patients, allodynic and spontaneous pain was reduced on a scale of 10 from 8.3 +/- 0.3 preimplantation to 3.5 +/- 0.4 (mean +/- standard error of the mean) at latest follow up (p<0.001). Changes in vasomotor tone and patient activity levels were markedly improved but motor weakness and trophic changes showed less improvement. Six (20%) of the 30 patients undergoing PNS placement returned to part-time or full-time work after being unemployed prestimulator implantation. Initial involvement of more than one major peripheral nerve correlated with a poor or no relief rating (p<0.01). Operative modifications that minimize technical complications are described. This study indicates that PNS can provide good relief for RSD that is limited to the distribution of one major nerve.
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PMID:Long-term results of peripheral nerve stimulation for reflex sympathetic dystrophy. 860 52

The purpose of the present study was to investigate the extent and quality of sensory impairment and their relation to pain characteristics and movement disorders in patients suffering from complex regional pain syndrome (CRPS) type I. Neurological testing was performed independently by two examiners in 24 patients with CRPS type I. In eight patients (33%), a hemisensory impairment with decreased temperature and pinprick sensation ipsilateral to the limb affected by CRPS could be observed. In four patients (17%), a sensory deficit in the upper quadrant of the body could be demonstrated and in eight patients (33%), sensory impairment was limited to the limb affected by CRPS. Mechanical allodynia and mechanical hyperalgesia could be observed in a higher percentage of patients with hemisensory deficit or sensory impairment in the upper quadrant (92%), than in those patients with sensory impairment limited to the affected limb (17%) (P < 0.005). In patients with left-sided CRPS, sensory abnormalities in the upper quadrant or hemisensory impairment were more frequently demonstrated (77%) than in patients with right-sided CRPS (18%) (P < 0.005). There was a high correlation (92%) for the sensory findings between the two examiners, and hemisensory abnormalities were stable over a period of 3-6 months in all six patients with repeated examinations. Motor impairment (contractures, weakness, tremor or difficulties in initiating movement) could be observed in a higher percentage in patients with sensory abnormalities in the upper quadrant or hemisensory impairment (83%) than in patients with sensory impairment limited to the affected limb (42%) (P < 0.05) and was significantly correlated with allodynia/hyperalgesia (P < 0.005). The results demonstrated that sensory deficits in patients with CRPS, frequently extend past the painful area of the affected limb. The increased frequency of mechanical allodynia and movement disorders in patients with hemisensory impairment or sensory deficits in the upper quadrant, might indicate that central mechanisms are involved in the pathogenesis of CRPS in these patients.
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PMID:Hemisensory impairment in patients with complex regional pain syndrome. 1068 Dec 42

Reflex sympathetic dystrophy (RSD), recently reclassified as a complex regional pain syndrome, type I (CRPS-I), is best known for its disabling sensory symptoms, including pain, allodynia, and abnormal skin temperature. Yet, motor dysfunction is common in CRPS and can result in major disability. In addition to weakness of the involved limb, CRPS patients may develop symptoms akin to a neurological neglect-like syndrome, whereby the limb may feel foreign ("cognitive neglect") and directed mental and visual attention is needed to move the limb ("motor neglect"). Members of the patient support group, the Reflex Sympathetic Dystrophy Syndrome Association (RSDSA), were mailed a questionnaire inserted in their newsletter which inquired about the presence of these neglect-like symptoms; in addition, a separate medical history questionnaire was included to assess adequate documentation for the diagnosis of CRPS. A total of 242 patients returned the questionnaire but only 224 of the questionnaires were analyzed; 15 were excluded due to inadequate documentation of CRPS and 3 were excluded due to non-limb involvement. Eighty-four percent (84%) of these respondents endorsed the presence of at least one neglect symptom and 47% indicated they had both "cognitive" and "motor" neglect symptoms. Of interest, approximately 33% of respondents spontaneously wrote comments regarding the significant disability due to these neglect symptoms and the difficulty explaining these unusual symptoms to their health care providers and family. This patient survey confirms the presence of neglect-like symptoms in a subset of CRPS patients. Neglect-like symptoms need to be addressed and validated by health care providers.
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PMID:Neglect-like symptoms in complex regional pain syndrome: results of a self-administered survey. 1051 43

Early diagnosis is a prerequisite for a successful treatment of complex regional pain syndrome (CRPS). In order to describe neurological symptoms which characterize CRPS, we evaluated 145 patients prospectively. Two-thirds of these were women, the mean age at time of investigation was 50.4 years. CRPS followed limb trauma, surgery and nerve lesion. Employing the current IASP criteria 122 patients were classified as CRPS I and 23 as CRPS II. All patients were assessed clinically pain was quantified using the McGill pain questionnaire, skin temperature was measured by an infrared thermometer and a subgroup of 57 patients was retested in order to determine thermal thresholds (QST). Of our patients 42% reported stressful life events in a close relationship to the onset of CRPS and 41% had a history of chronic pain before CRPS. The latter group of patients gave a higher rating of CRPS pain (P<0.05). The major symptoms were pain at rest in 77% and hyperalgesia in 94%. Typical pain was deep in the limb having a tearing character. Patients getting physical therapy had significantly less pain than those without (P<0.04). Autonomic signs were frequent (98%) and often changed with the duration of CRPS. Skin temperature was warmer in acute and colder in chronic stages (P<0.001). Likewise edema had a higher incidence in acute stages (P<0.001). We found no correlation between pain and autonomic dysfunction. Motor dysfunction (present in 97%) included weakness, tremor, exaggerated tendon reflexes, dystonia or myoclonic jerks. QST revealed increased warm perception thresholds (P<0.02) and decreased cold pain thresholds (P<0.03) of the affected limb. The detailed knowledge of clinical features of CRPS could help physicians early to recognize the disease and thus to improve therapy outcome.
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PMID:Neurological findings in complex regional pain syndromes--analysis of 145 cases. 1077 May 24

Four hundred eighty five patients whose chief complaints were work related pain and other symptoms received a comprehensive upper-body clinical evaluation to determine the extent of their illness. The group had a mean age of 38.5 years. Sixty-three percent of patients were females. Seventy percent were computer users, 28% were musicians, and 2% were others engaged in repetitive work. The time between the onset of symptoms and our initial visit ranged from 2 weeks to over 17 years. A majority sought care within 30 months with the greatest number of them seeking care before 12 months. Fifty nine percent of subjects were still working when seen despite increasing pain and symptoms such as weakness, numbness, tingling, and stiffness. Following a history, a physical assessment utilizing commonly employed clinical tests were performed including evaluation of joint range of motion, hyperlaxity, muscle tenderness, pain, strength, and imbalance. Neurologic tests included Tinel's sign performed in wrist, elbow, tricipital sulcus, and neck and tests for thoracic out syndrome (TOS). Specific tests such as Finkelstein's test for deQuervain's tenosynovitis, Phalen's test for carpal tunnel syndrome and grip strengths were included in the examination protocol. Significant findings included postural misalignment with protracted shoulders (78%), head forward position (71%), neurogenic TOS (70%), cervical radiculopathy (0.03%), evidence of sympathetic dysfunction (20%), and complex regional pain syndrome (RSD) (0.6%). Hyperlaxity of fingers and elbows was found in over 50%, carpal tunnel syndrome in 8%, radial tunnel syndrome in 7%, cubital tunnel in 64%, shoulder impingement in 13%, medial epicondylitis in 60%, lateral epicondylitis in 33%, and peripheral muscle weakness in 70%. We conclude that despite initial presentation distally, work-related upper-extremity disorders are a diffuse neuromuscular illness with significant proximal upper-body findings that affect distal function. While neurogenic TOS remains a controversial diagnosis, the substantial number of patients with positive clinical findings in this study lends weight to the concept that posture related neurogenic TOS is a key factor in the cascading series of physical events that characterize this illness. A comprehensive upper-body examination produces findings that cannot be obtained through laboratory tests and surveys alone and lays the ground work for generating hypotheses about the etiology of work related upper-extremity disorders that can be tested in controlled investigations.
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PMID:Understanding work-related upper extremity disorders: clinical findings in 485 computer users, musicians, and others. 1170 73

A 67-year-old man complained of a burning pain and weakness of bilateral feet after contusion of the left lumbar region. Skin as well as bone dystrophy and disturbance of bladder function were not seen, but low skin temperature was observed in the left lower leg. Glove anesthesia was seen on bilateral feet. Patellar tendon reflex was accentuated but Achilles tendon reflex was diminished, and bilateral Babinski sign was positive. Compression of the spinal cord or spinal root nerve was not noticed by MRI, myelography and myelo-CT (from cervical to lumbar level). We suspected the complex regional pain syndrome type I, and performed sympathetic blockade, but burning pain was not relieved. We looked for spinal tumor, myelitis, collagen disease, vitamin deficiency and malignancy but could not find out any disorder. However, the patient had neuropathic sign in electromyogram, and high anti-HTLV-I antibody titers in blood serum (8192x) and cerebrospinal fluid (256x). We diagnosed this case as HTLV I-associated myelopathy (HAM). He developed, so called, HTLV I-associated pneumonia at 74 years of age. We suggest that HAM may rarely accompany a burning pain and neuropathy (not myelopathy) as main symptoms. The present case suggests that a patient with HAM may develop HTLV I-associated pneumonia during its process; indicating a new concept of this very rare disease.
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PMID:[A patient with HTLV I-associated myelopathy (HAM) complaining of burning pain on the bilateral feet: a case report]. 1459 76

The clinical characteristics of complex regional pain syndrome (CRPS)--spontaneous and stimulus-evoked pain, autonomic abnormalities, motor dysfunction, and trophic changes in the affected limb--are well known. However, its pathogenesis is unclear, and the diagnosis is often delayed, in part due to lack of objective laboratory tests. Endothelin-1 (ET-1) is a potent vasoconstrictor that has recently been shown to produce pain, allodynia, edema, and muscle weakness, as well as to exert a direct excitatory effect on nociceptive afferents. Furthermore, new evidence indicates that ET-1 is involved in various cancer- and non-cancer-related painful conditions. The aim of the present explorative study was to determine the ET-1 plasma levels in patients with CRPS in an attempt to identify a 'laboratory marker' for CRPS and to search for evidence suggesting that ET-1 may be involved in the pathogenesis of CRPS. ET-1 plasma levels were determined in 20 severely affected CRPS patients, in eight patients with non-CRPS chronic painful conditions, and in 10 healthy volunteers. The results showed that there were no significant differences in ET-1 plasma levels between the three groups. We conclude that the plasma level of ET-1 cannot be regarded as a 'marker' for CRPS. Yet, the possibility that ET-1 is involved in the pathophysiology of CRPS has not been excluded and deserves further investigation.
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PMID:Plasma endothelin-1 levels in patients with complex regional pain syndrome. 1553 Dec 21

Viral invasion of the motoneurons and the subsequent inflammation in the anterior horn cells by the varicella zoster virus results in a weakness in the area of the cutaneous eruption. The exact mechanism of zoster paresis is uncertain. The occurrence of symptoms resembling complex regional pain syndrome (CRPS) is common in subjects where the herpes zoster (HZ) outbreak affects an extremity, particularly if it is the distal extremity that is involved. We report the case of a 54-year-old man with monoparesis, hyperalgesia, allodynia, edema, and both color and skin-temperature changes in his left arm after a skin eruption. Electrophysiologic examination revealed the partial degeneration of the superior, middle, and inferior truncus in the brachial plexus, with evidence of HZ infection. Magnetic resonance imaging of the cervical spine and brachial plexus showed degenerative changes without any evidence of nerve root compression. Brachial plexopathy may be the direct cause of the reversible upper-limb paresis resulting from HZ with CRPS-like symptoms.
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PMID:Monoparesis with complex regional pain syndrome-like symptoms due to brachial plexopathy caused by the varicella zoster virus: a case report. 1714 48

Lower-extremity trauma is an uncommon but reported cause of sciatic nerve injury in children and adolescents. Failure to identify sciatic neuropathy after traumatic injury to the lower extremity may lead to the delayed institution of neuropathic pharmacotherapy, electrodiagnostic testing, physical therapy, and increased risk for the development of complex regional pain syndrome. This article presents a case of an adolescent male with neuropathic pain and weakness in the right lower extremity after traumatic injury. Spontaneous recovery of the injured nerve occurred with early institution of pharmacologic and physical therapies. Operative exploration and neurolysis were considered but were not ultimately necessary.
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PMID:Sciatic neuropathy after lower-extremity trauma: successful treatment of an uncommon pain and disability syndrome in an adolescent. 1758 Dec 94

We report a case of type 1 complex regional pain syndrome (CRPS I) of the left leg following the implantation of an artificial disc type in the L4/5 segment of the lumbar spine using a midline left-sided retroperitoneal approach. This approach included the mobilisation of the sympathetic trunk with incision and resection of the intervertebral disc. The perioperative and immediate postoperative periods were uneventful, but on the second postoperative day the patient complained of a progressive allodynia of the whole left leg in combination with weakness of the limb. Neurological examination did not reveal any radicular deficit or paresis. A sympathetic reaction following the mobilisation of the sympathetic trunk during the ventral preparation of the spine was suspected and investigated further. A diagnosis of CRPS I was proposed, and the patient was treated with analgesia, co-analgesics for pain alienation, and systemic corticosteroid therapy. A computed tomography-guided sympathetic block and lymphatic drainage were performed. Following conservative orthopaedic rehabilitation therapy, the degree of pain, allodynia, weakness, and swelling were reduced and the condition of the patient was ameliorated. The cost-benefit ratio of spinal arthroplasty is still controversial. The utility of this paper is to debate a possible cause of a painful complication, which can invalidate the results of a successful operation.
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PMID:CRPS I following artificial disc surgery: case report and review of the literature. 2127 30


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