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)

Disturbance of posture may occur in a variety of neurological disorders and occasionally is the presenting or even the only sign. In the majority of cases, the head or the trunk or both are bent forward (bent spine syndrome, dropped head syndrome). A feature of these primary neurogenic or myogenic postural disturbances that is in contrast to antalgic contraction or ankylosis is that they are not fixed, but the trunk or head are easily erected by the examiner and show a characteristic sagging. Neuromuscular disorders are a frequent cause. They may be confined to the paraspinal muscles. Axial computed tomography of the spine, electromyography of the involved muscles, and muscle biopsy help to make the diagnosis. However, also central movement disorders may lead to a sagging of the head or trunk or of both due to a lessened tone of the head and trunk extensors. This is frequently seen in the various parkinsonian syndromes which may, however, occur in association with a focal myopathy of the paraspinal muscles. Occasionally, sagging of the trunk is seen as a side effect of neuropharmacologic medication. Sagging of the trunk or head should be differentiated from a pathologically increased innervation of the ventral muscles in dystonic movement disorders such as antecollis or camptocormia. Pathologic reclination of the head or trunk or both is a rare disturbance of posture. It may occur in dystonia (retrocollis) or, occasionally, as a consequence of musculotendinous contractures secondary to certain neuromuscular disorders such as the rigid spine syndrome.
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PMID:[Primary neurogenic and myogenic disorders of posture]. 1511 20

Cerebral palsy (CP) has always been considered a static condition in the neurological sense. Secondary and associated conditions that occur in the patient with CPcan progress over time and cause unwanted sequelae. This paper discusses four musculoskeletal conditions that present across the lifetime and can lead to progressive loss of function in the patient with CP. Patella alta can be particularly painful in the early adult years, limiting mobility particularly when associated with crouch gait. Adults with lower-extremity weight-bearing status having hip dysplasia, progressive over time, often develop pain and severe degenerative arthritis, with or without arthrodesis. Spondylolysis, particularly at the L5 S1 level, is fairly common in the ambulatory adult with diplegia and may, if not diagnosed early, progress to spondylolisthesis. Cervical stenosis appears to be more prevalent in adults with spastic quadriparesis and dystonia and is often associated with myelomalacia and/or radiculopathy. All four of these conditions may be lessened, or even prevented, with intervention and diagnosis in the younger years. Possible interventions and outcomes over time are discussed in the context of multidisciplinary team management of the individual with CP.
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PMID:Cerebral palsy lifetime care - four musculoskeletal conditions. 1974 Feb 8

Mechanism of hypertonia in cerebral palsy children is dual: a neural component due to spasticity (velocity dependent) and a biomechanical component linked to soft tissue changes. Their differentiation-which might be clinically difficult-is however crucial, as only the first component will respond to anti-spastic treatments, the second to physiotherapy. Furthermore, spasticity is frequently associated with dystonia, which is a sustained hypertonic state induced by attempts at voluntary motion. Spasticity and dystonia have to be carefully distinguished as dorsal rhizotomy will not significantly influence the dystonic component. Spasticity, which by definition opposes to muscle stretching and lengthening, has two important consequences. First, the muscles tend to remain in a shortened position, which in turn results in soft tissue changes and contracture. The second is that movements are restricted. Thus, both hypertonia and lack of mobilization create a vicious circle leading to severe locomotor disability linked to irreducible musculotendinous retraction and joint ankylosis/bone deformities. These evolving consequences should be highly considered during the child's assessment for decision-making. The hypotonic effects of lumbosacral dorsal rhizotomy, which are not only segmental on the lower limbs but also supra-segmental through the reticular formation, are finally discussed.
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PMID:Neurophysiological mechanisms of hypertonia and hypotonia in children with spastic cerebral palsy: surgical implications. 3254 70