Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026838 (spasticity)
 6,471 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Baclofen, a gamma-aminobutyric acid agonist, acts at the spinal cord level to impede the release of excitatory neurotransmitters that cause spasticity. Oral baclofen improves cerebral spasticity mildly, but its activity is limited because of its poor lipid solubility. Cerebrospinal fluid baclofen levels after intrathecal administration are many times higher than those achieved after oral administration. Continuous intrathecal baclofen infusion has been used to treat cerebral spasticity in two patient groups: in older ambulatory children with inadequate underlying leg strength, and in patients with severe spasticity in both the upper and lower extremities. Responsiveness to intrathecal baclofen is confirmed by test injections before insertion of a programmable subcutaneous pump. Continuous intrathecal baclofen infusion dosages vary from 27 to 800 micrograms/day. Continuous intrathecal baclofen infusion reduces spasticity in the upper and lower extremities, and improves upper extremity function and activities of daily living but has no effect on athetosis in the dosages used to treat spasticity. Complications related to the intrathecal catheter occur in approximately 20% of patients, and infection requiring pump removal occurs in approximately 5%. Preliminary studies indicate that continuous intrathecal baclofen infusion alleviates some forms of generalized dystonia associated with cerebral palsy.
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PMID:Baclofen in the treatment of cerebral palsy. 888 81

Asphyxia may play an important role in the pathogenesis of cerebral palsy (CP) in a subpopulation of patients, although this has recently been questioned by some investigators. Here I describe the results of our analyses into the involuntary movements of children with CP, which resulted from perinatal hypoxic brain damages. Based on cranial CT or MRI findings, the patients were divided into five groups: A, those showing high density on CT in the basal nuclei (bilateral thalami in particular); B, those showing destruction of bilateral putamina; C, those with diffuse low-density areas in the cerebral white matter on CT during the neonatal period that evolved later into polycystic leukomalacia; D, those with similar low-density areas which subsequently resolved; and E, those without any pathological findings. Using video records, I demonstrated the motor development of six cases with CP. Patients of Group A showed pure athetosis with hypotonia. A patient of Group B had severe athetoid CP with spasticity, being unable to right his trunk and neck. A case of Group C developed severe spastic quadriplegia with athetosis. His mental ability was retained to some extent. In Group D, there was moderate spasticity and mild athetosis. A patient with transient anxia had disturbance in the coordinated finger movements. Cases with choreic movements had no particular CT or MRI findings. Even in the severest of these cases, distinction from normal infants was difficult in the very early infancy. After four to five months, locomotive prognoses were well predicted by the patients' ability to control their trunk. To know more about the pathogenesis and CP and to elucidate the significance of involuntary movements, further data should be accumulated by clinical observations on motor development, and by imaging studies.
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PMID:[Involuntary movements and birth injuries to brain]. 914 27

Denervation, provosed at the turn of the century and abandoned because of inconstant results, has again been taken up in principle, but with a notable change in technique. It is necessary to do a careful examination of the muscles that are to be denervated (of the median and ulnar nerves) and to determine the nature of the spasticity. The incision, longer than in the original technique, allows a better view of the median and ulnar nerves. The nerve branches to each muscle are thus easily identified, and by electric stimulation during the operation one judges how much denervation to subject the muscles to while taking into account the phenomenon of the take-over of the denervated muscles by the remaining nerve fibers. Part of the nerve branches are then sectioned under microscopic control near the muscle, taking care to cauterize the proximal stump. It may be necessary to do associated tenotomies or capsulotomies. This method gives good results if the technique and the contraindications, (athetosis, chorea and cerebral deficits) are respected. It must be emphasized that the results depend on the degree of denervation, which is hardly quantifiable, and is subject to the operator's experience.
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PMID:[Selective microsurgical denervation in spastic paralysis]. 938 48

Feeding difficulty and malnutrition are common in disabled children. Intake may be reduced because of anorexia, chewing and swallowing difficulties, or vomiting. Feeding is often time consuming, unpleasant, and may result in aspiration. Malnutrition may result in impaired growth and neurodevelopment, and impaired cardiorespiratory, gastrointestinal, and immune functions. Multidisciplinary assessment is recommended and should include a feeding history, oral-motor examination, and nutritional assessment. The energy requirements of most disabled children are less than those for a normal child of the same age but may be increased by spasticity, athetosis, convulsions, and recurrent infections. Micronutrient deficiencies may occur even in children receiving nutritionally complete feeds if the volume is reduced because of low energy requirements. Oral intake may be improved by a change of posture, special seating, feeding equipment, oral desensitization, mashing or pureeing of lumpy food, thickening of liquids, use of calorie supplements, and treatment of reflux/esophagitis. Non-oral feeding should be considered when oral feeding is unsafe, not enjoyable, inadequate, or very time consuming. Long-term support requires a gastrostomy. This is less obtrusive than a nasogastric tube, less likely to become displaced, less traumatic, and is associated with improved quality of life, but is also associated with significant morbidity. If there is symptomatic reflux a fundoplication may be required, but this is associated with significant mortality and substantial morbidity.
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PMID:Feeding the disabled child. 978 66

We reviewed 90 consecutive patients with various neuromuscular diseases and a progressive spine deformity treated with a prefabricated Boston-type underarm corrective brace. Of these, 38 patients had spastic tetraplegia; seven, syndrome-related muscular hypertonia; 24, muscular hypotonia; and 21, myelomeningocele. The mean age at the treatment start was 9.2 years (range, 1.4-17.7 years). Twenty-four were ambulating and 66 wheelchair-bound. Hypotonia was the dominant type of muscle involvement in 49, spasticity in 28, and athetosis in 13 patients. The mean pretreatment Cobb angle was 47 degrees, with a range from 23 to 95 degrees. The mean brace-induced Cobb-angle correction was 60%, thus well comparable to that in idiopathic scoliosis. However, this did not predict favorable treatment results. At the follow-up, on average 3.1 years (range, 1-5.5 years) after weaning from the brace, the brace treatment was successful in 23 patients. Successful was defined as <10 degrees curve progression during the observation time and a good brace compliance. Forty-one patients discontinued the brace treatment, and 19 progressed despite adequate brace wear. Five patients are still in treatment, and two have died. Successful treatment was seen in ambulating patients with muscle hypotonia and short thoracolumbar/lumbar curves measuring <40 degrees as well as in nonambulating patients with spastic short lumbar curves. These types of neuromuscular scoliosis may be the only ones to respond to brace treatment. In other cases, the brace treatment cannot be expected to have a lasting corrective effect although it can be used as sitting support.
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PMID:Brace treatment in neuromuscular spine deformity. 1034 23

We present here 38 patients with athetotic cerebral palsy who has trained in our clinic in recent 5 years. The mean age was 4 years and 4 months. In preterm cases, their gestational ages ranged from 27 weeks to 36 weeks, less than 31 weeks in 74%. MRI showed periventricular leukomalacia in 75%. Their clinical picture was mixed quadriplegia with athetosis and spasticity. Half of the cases were profoundly handicapped in motor ability. Fifteen cases had been born in term. Sixty percent of them had a non-mixed type of cerebral palsy. Five cases had no remarkable risk factor during the perinatal period. Six cases were able to walk with or without support. Hypoxic-ischemic encephalopathy was the main cause of athetotic cerebral palsy in recent years. Twin pregnancy was also an important risk factor, especially in preterm infants. MRI could detect lesions in the thalamus or the basal ganglia in only 17% of the 36 cases examined.
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PMID:[Clinical features of children with athetotic cerebral palsy in relation to their gestational age at birth]. 1114 61

Dystonia is an interesting disorder characterized by involuntary movement of the body part or parts leading to abnormal deformed postures. The usual signs and symptoms are local pain, spasm and abnormal movements. Sensory trick is an important clinical phenomenon and is characteristic of dystonia. It is usually separated from other movement disorders such as chorea, athetosis, tics and myoclonus clinically. Various non-dystonic conditions simulate dystonia and need to be separated in view of different line of management. Improved understanding in molecular biology has helped in understanding of the disease. Confusing neuropathology and neurochemistry have deterred the finding of an effective drug, however empirical use of few drugs have improved the gloomy situation. Few conditions such as dopa-responsive dystonia have definite treatment. Recently use of botulinum toxin has provided beneficial response in hyper muscular contraction states such as dystonia and spasticity, Surgery and other non-medical therapies are effective in few situations.
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PMID:A spectrum of dystonias-clinical features and update on management. 1127 44

It has recently been suggested that lycra garments are helpful for children with cerebral palsy (CP). Twelve children, with athetosis, ataxia, and spasticity, were fitted with lycra garments (Kendall-Camp UK Ltd). Scores on the Paediatric Evaluation of Disability Inventory (PEDI) scales were determined before and after wearing the garment for at least 6 hours a day for 6 weeks. Five children with motor problems representative of the whole group were investigated during a reach-and-grasp task by kinematic motion analysis; reflective markers were used with and without the garment. Carers were given a questionnaire concerning the practicalities of using the garments. All 12 children made improvements in at least one of the functional scales of the PEDI, and scores for the whole group showed significant gains (Wilcoxon chi2 test, self-help p<0.01; mobility p<0.5; social p<0.1). These changes were usually slight, although noticed by carers. Six children made gains of at least one scale of the caregiver assistance scores, two of the children showed losses (due to difficulties removing the garment for toileting), and four showed no change. Motion analysis indicated that (1) two children with athetosis had improved proximal stability in sitting and in smoothness of arm movements, (2) one child with ataxia had improved in proximal and distal stability, and (3) two children with spasticity had more jerky movements, although one improved in proximal stability. All children had problems in wearing the garments, including problems with toileting and incontinence of urine; the parents of only one child wanted to continue using it. Results suggest that the functional benefit of lycra garments for children with CP is mainly due to improvements in proximal stability but this should be weighed against the inconvenience and loss of independence.
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PMID:Assessment of upper-limb function and movement in children with cerebral palsy wearing lycra garments. 1140 27

Within the cerebral palsy syndromes, athetosis is most commonly causally associated with serious perinatal complications. Genetic factors are thought to play a lesser role, although the risk of recurrence in siblings has been suggested to be as high as 10%. We have conducted a clinical study of 22 subjects with a diagnosis of athetoid cerebral palsy and a review of the literature aiming to identify instances of familial recurrence of athetoid cerebral palsy. The birth history, family history, and previous investigations of subjects with athetoid cerebral palsy were studied and subjects were clinically examined for evidence of an underlying genetic etiology. Factors suggesting a genetic cause were specifically sought, such as advanced paternal age, progression of symptoms, and associated congenital abnormalities. No subjects in the study group had similarly affected relatives, and additional features suggesting a genetic cause were not observed. A literature search identified 16 instances of familial recurrence of athetoid cerebral palsy. Familial cases were typically associated with significant spasticity, microcephaly, intellectual disability, seizures, and a lack of history of birth asphyxia, and most could be explained by either autosomal-recessive or X-linked-recessive inheritance. The genetic contribution to athetoid cerebral palsy is small, with an overall risk of recurrence in siblings of about 1%. This risk is lower than previously suggested in the literature.
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PMID:Genetic factors in athetoid cerebral palsy. 1173 63

For children whose spasticity and movement disorders are inadequately treated by oral medications and botulinum toxins, neurosurgical procedures are now available to effectively treat spasticity, tremor, and many cases of dystonia. Spastic diplegia can be treated with selective lumbar rhizotomies, which significantly decrease spasticity, increase range of motion, and improve Gross Motor Function Measure scores. Children with spastic quadriparesis and those with secondary dystonia can be treated with intrathecal baclofen, which diminishes both spasticity and dystonia and is associated with improved function and quality of life. Children with primary dystonia and those with tremor can be treated with deep brain stimulation of the internal globus pallidus and thalamus, respectively. Some children with chorea respond to deep brain stimulation. There are no effective neurosurgical treatments for athetosis or ataxia. The effectiveness of neurosurgical treatments of pediatric movement disorders has increased significantly in the past 15 years.
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PMID:Neurosurgical treatment of spasticity and other pediatric movement disorders. 1367 72


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