Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013421 (dystonia)
 8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

We report on 26 patients with a distinct phenotype of complex regional pain syndrome that progressed toward a multifocal or generalized tonic dystonia. The dystonia initiated distally, involved mainly flexor muscles, and was associated with sensory and autonomic symptoms. Dryness of the eyes or mouth and bladder and bowel disturbances were frequently reported. There was no increase in the familial prevalence of autoimmune-mediated diseases. Compared with controls, a significant elevation of HLA-DR13 was found in the patients. Thus, HLA-DR13 may be a factor indicating susceptibility to this distinct phenotype of complex regional pain syndrome.
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PMID:Multifocal or generalized tonic dystonia of complex regional pain syndrome: a distinct clinical entity associated with HLA-DR13. 1089 25

Dysfunction of the autonomic nervous system is an under-recognised but important aspect of the aetiological and clinical manifestation of primary degenerative dysautonomias such as multiple system atrophy (MSA) and Parkinson's disease (PD). Although the clinical presentation of dysautonomia in these two disorders may overlap, yet pathological and in vivo imaging studies suggest considerable differences. Functional imaging studies suggest that selective cardiac sympathetic denervation may occur early in PD but not in other parkinsonian syndromes. The clinical implication of this apparently disease specific peripheral dysautonomia is unknown and would be the subject of much interest in future years. Dysautonomia in degenerative disorders also affect respiration, genitourinary function and sleep. Sleep related disorders such as rapid eye movement behaviour disorder and urinary voiding dysfunction appear to precede the development of PD related symptoms while patients with sporadic ataxia have been shown to progress to develop MSA. Dysautonomia has also been recognised in other movement disorders, examples being the combination of dystonia and complex regional pain syndrome with elevated HLA-DR13 and late onset Huntington's disease presenting with dominant parkinsonism and minimal chorea. These studies have helped progress in various diagnostic and management parameters in relation to autonomic dysfunction and movement disorders.
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PMID:Autonomic dysfunction in movement disorders. 1147 Sep 68

An association between HLA-DR13 and patients with complex regional pain syndrome (CRPS) who progressed towards multifocal or generalized tonic dystonia was recently reported. We now report on a new locus, centromeric in HLA-class I, which was significantly associated with a spontaneous development of CRPS, suggesting an interaction between trauma severity and genetic factors conferring CRPS susceptibility. Additionally, an association with the D6S1014 locus was found, supporting the previous finding of an association with HLA-DR13.
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PMID:Susceptibility loci for complex regional pain syndrome. 1274 63

The efficacy of botulinum toxin (BTX) without systemic effects has led to the rapid development of applications in neuromuscular disorders, hyperactivity of sudomotor cholinergic-mediated glandular function, and pain syndromes. The successful use of BTX in conditions with muscle overactivity, such as dystonia and spasticity, has been established and new areas in the field of movement disorders such as tics, tremor, myoclonic jerks, and stuttering has been explored with satisfactory results. Strategies to temporarily inactivate muscle function after orthopaedic or neurosurgery have also been developed. BTX treatment of hyperhidrosis was followed by its application in other hypersecretory conditions (hyperlacrimation and nasal hypersecretion) and in excessive drooling. Studies are in progress, aimed at optimising the technique and protocol of administration. Other applications for BTX have been proposed in gastroenterological and urogenital practice; it appears to be effective in replacing standard surgical procedures. Trials of BTX in painful conditions are ongoing mainly on refractory tension headache, migraine, and backache as well as dystonia-complex regional pain syndrome and myofascial pain with promising results. Recently, the fastest growing use for BTX toxin has been in the cosmetic applications. Clearly, the indications for the use of BTX are expanding, but further clinical trials will be needed in many different areas.
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PMID:New therapeutic indications for botulinum toxins. 1502 69

We studied a case series of 9 patients with posttraumatic cervical dystonia, in whom involuntary muscle spasms and abnormal head postures occurred within 7 days after cervical injury. Patients were examined, treated with botulinum toxin as necessary, and were followed up to 5 years. Based on our observations of these cases, we propose that complex regional pain syndrome (CRPS) could represent a variant of posttraumatic cervical dystonia that may develop over time after the initiation of dystonia.
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PMID:Natural history of posttraumatic cervical dystonia. 1539 63

The origin of myoclonus in patients with complex regional pain syndrome (CRPS) is unknown. Eight patients with CRPS related myoclonus were clinically evaluated and studied with intermuscular and corticomuscular coherence analysis. Jerks were present at rest, aggravated during action and were frequently associated with tremulousness or dystonia. Electromyography demonstrated a burst duration ranging from 25 to 240 ms with burst frequencies varying from <1 jerk/s during rest to 20 Hz during action. Coherence studies showed increased intermuscular coherence in 4 patients in the 6 to 12 Hz band, as reported in patients with enhanced physiological tremor. In 2 patients side-to-side coherence was observed, pointing to a central oscillatory drive. Significant coherence entrainment was detected in 5 patients. We conclude that the characteristics of myoclonus in CRPS are different from other forms of myoclonus.
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PMID:Clinical and neurophysiological characterization of myoclonus in complex regional pain syndrome. 1897 59

A form of fixed dystonia (FD) without evidence of basal ganglia lesions or neurodegeneration has been recently characterized (Schrag et al., Brain 2004;127:2360-2372), which may overlap the clinical spectrum of either complex regional pain syndrome or psychogenic dystonia. Transcranial magnetic stimulation studies in typically mobile dystonia revealed abnormal motor cortical excitability and sensori-motor integration. We compared 12 patients with limb FD to 10 patients with primary adult-onset typically mobile dystonia and 11 age-matched healthy volunteers. Measurements at the first digital interosseus representation area on both hemispheres included: short intracortical inhibition (SICI), contralateral silent period (cSP), and short and long afferent inhibition (SAI and LAI). Repeated measure ANOVA and post-hoc t-tests were used for statistical analysis. SICI was significantly reduced in both hemispheres of patients with "typical" and FD, compared to healthy subjects. For both hemispheres, cSP duration was shorter in both fixed and "typical" dystonia patients. SAI and LAI did not significantly differ between the three groups. The abnormal cortical excitability observed in FD might represent an underlying trait predisposing to different clinical forms of dystonia.
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PMID:Cortical excitability is abnormal in patients with the "fixed dystonia" syndrome. 1817 41

The question whether peripheral trauma can cause movement disorders has since long been a subject of debate. In this review we present the pro's and con's of arguments that have been presented in the literature and discuss their plausibility. Additionally, recent developments on the potential mechanisms that underlie dystonia in complex regional pain syndrome are used to illustrate how the relation between peripheral trauma and movement disorders may evolve.
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PMID:Peripheral trauma and movement disorders. 1826 71

A significant proportion of chronic pain is of musculoskeletal origin. Botulinum toxin (BTX) has been successfully used in the treatment of spasmodic torticollis, limb dystonia, and spasticity. Investigators have, thus, become interested in its potential use in treating many chronic pain conditions. Practitioners have used BTX, outside the product license, in the treatment of refractory myofascial pain syndrome and neck and low back pain (LBP). This article reviews the current evidence relating to chronic pain practice. There is evidence supporting the use of both BTX type A and type B in the treatment of cervical dystonias. The weight of evidence is in favor of BTX type A as a treatment in: pelvic pain, plantar fasciitis, temporomandibular joint dysfunction associated facial pain, chronic LBP, carpal tunnel syndrome, joint pain, and in complex regional pain syndrome and selected neuropathic pain syndromes. The weight of evidence is also in favor of BTX type A and type B in piriformis syndrome. There is conflicting evidence relating to the use of BTX in the treatment whiplash, myofascial pain, and myogenous jaw pain. It does appear that BTX is useful in selected patients, and its duration of action may exceed that of conventional treatments. This seems a promising treatment that must be further evaluated.
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PMID:Evidence for the use of botulinum toxin in the chronic pain setting--a review of the literature. 1850 28


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