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Query: UMLS:C0026838 (spasticity)
 6,471 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The physician needs to integrate the results of the assessment of overall patterns of functional use (Table 1), static contractures, dynamic deformities of motor imbalance for multiple levels of involvement (shoulder, elbow, wrist, and hand), and sensory deficiencies. This information is combined with a general assessment of the child's mentation, motivation, and generalized medical condition. Certain patients benefit most from surgical intervention in cerebral palsy. Patients with spastic deformities or flaccid control of specific movements can be helped significantly. In patients with flaccid control of certain movements, such as absent ability to extend the wrist or abduct the thumb, surgery is centered on tendon transfers to augment the patient's ability to perform that movement. In patients with excessive spasticity or musculotendinous contracture, surgery is centered on muscle lengthening. In general, patients with athetosis are not treated surgically; the only surgical treatment considered for the athetotic patient is fusion, as this helps preposition the limb in a desired position and "simplifies" the system for the patient to control. Sensibility deficiencies do not preclude effective tendon transfer but do limit the overall use of the limb; patients with sensibility deficiencies need to be coached to use visual input as their afferent information. Motor deficiencies can be assessed by observation, examination, functional testing, and motion laboratory analysis. Combining an assessment of shoulder, elbow, forearm, wrist, thumb, and finger abilities and disabilities helps provide the physician with an overall plan of upper limb reconstruction using soft tissue releases, tendon transfers, and joint stabilization procedures to address the upper limb functional deficiencies. The ideal candidate for tendon transfer surgery is 7 years of age or older so they can be cooperative with postoperative rehabilitation and motivated to improve the use of their limb. Children with passive use of their limb (Functional Use Classification levels 1-3) can be improved most, on average 2.7 levels to active use of their limb (Functional Use Classification levels 4-6). An overall treatment plan is synthesized, taking into account the child's capabilities, disabilities, and potential, in the context of the child's age and expectations. The assessment techniques discussed in this article are the first step to appropriate treatment.
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PMID:Functional assessment aided by motion laboratory studies. 1459 48

Neurological findings, motor symptoms, mental abnormality and dysarthria were examined in 28 children with lesions in the thalamus, putamen, and/or peri-Rolandic area. The thalamus and putamen were involved in eight, and only the thalamus in ten of the children. Most of these 18 children had mild disabilities; they did not have severe mental retardation and could walk alone, speak words, and grasp an object. Dominant flexion of the hips was observed in many of the children who could walk. Two-thirds of these children had athetotic involuntary movement and the remaining had gross or fine motor abnormalities although they had no involuntary movement. In most of these children, reaching patterns were abnormal and were affected by shoulder retraction. Their abnormal movements were thought to be inappropriate muscle activity brought about by voluntary movements. In the remaining ten children, the thalamus, putamen, and peri-Rolandic area were all involved. Many had severe disabilities such as severe mental retardation and the inability to sit, speak words, or grasp an object. All had athetotic involuntary movements. Three children had spasticity of the lower extremities. Five children with severe disabilities and no spasticity were thought to have apparent weakness with athetosis.
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PMID:Clinical profiles of children with cerebral palsy having lesions of the thalamus, putamen and/or peri-Rolandic area. 1513 Jun 88

Since its introduction in the late 1980s, intrathecal baclofen (ITB) therapy has become the standard treatment for severe generalized spasticity and dystonia in children. Treatment with ITB decreases spasticity in the upper and lower extremities and has been associated with improved function and decreased musculoskeletal contractures. In addition, ITB decreases generalized secondary dystonia and has been associated with improved comfort and ease of care in approximately 85% and with improved function in approximately 33% of patients. Continued effectiveness of ITB in treating spasticity has been observed for up to 17 years, and its effectiveness in treating dystonia has been observed for up to 10 years. Although ITB therapy is frequently associated with complications such as infections, catheter malfunctions, and cerebrospinal fluid leaks, the benefits of therapy appear to outweigh the risks. Additional investigation is needed to determine the effects of ITB on other movement disorders such as athetosis and chorea.
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PMID:Intrathecal baclofen therapy in children. 1691 24

Lesch-Nyhan syndrome is a very rare X-linked recessive disorder characterized by mental retardation, spasticity resembling cerebral palsy, choreo-athetosis, self-mutilation and hyperuricemia. Self-mutilative behavior is a hallmark of the disease. Hyperuricemia leads to hyperuricuria and uric acid nephrolithiasis. The underlying defect is a deficiency of hypoxanthine-guanine-phosphoribosyl transferase. We report on a 7-year-old boy with Lesch-Nyhan syndrome, lacking self-mutilative behavior, who was erroneously diagnosed as having athetotic cerebral palsy. He also had no renal stones; hyperechoic renal medullary pyramids were the only renal abnormality detected and were sonographically indistinguishable from medullary nephrocalcinosis.
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PMID:Rare variant of Lesch-Nyhan syndrome without self-mutilation or nephrolithiasis. 1768 Feb 74

The American Academy of Neurology now recommends that all cases of cerebral palsy of unknown origin undergo neuroimaging. Controversy surrounds this recommendation because of concerns about the adequacy of the supporting evidence. This article reviews the evidence provided by magnetic resonance imaging (MRI) and computed tomography (CT) imaging studies in cerebral palsy and discusses the potential benefits of imaging, techniques in current use, and future directions, with a focus on improving etiologic understanding. Most (83%) children with cerebral palsy have abnormal neuroradiological findings, with white matter damage the most common abnormality. Combined gray and white matter abnormalities are more common among children with hemiplegia; isolated white matter abnormalities are more common with bilateral spasticity or athetosis, and with ataxia; isolated gray matter damage is the least common finding. About 10% of cerebral palsy is attributable to brain malformations, and 17% of cerebral palsy cases have no abnormality detectable by conventional MR or CT imaging. Although neuroimaging studies have increased our understanding of the abnormalities in brain development in cerebral palsy, they are less informative than they might be because of 4 common problems: (1) inappropriate assignment of etiology to morphologic findings, (2) inconsistent descriptions of radiologic findings, (3) uncertain relationship of pathologic findings to brain insult timing estimates, and (4) study designs that are not based on generalizable samples. Neuroimaging is not necessarily required for diagnosis of cerebral palsy because the disorder is based on clinical findings. The principal contribution of imaging is to the understanding of etiology and pathogenesis, including ruling in or out conditions that may have implications for genetic counseling, such as malformations. In the future, as more sophisticated imaging procedures are applied to cerebral palsy, specific morphologic findings may be linked to etiologic events or exposures, thus leading to potential pathways for prevention.
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PMID:A systematic review of neuroimaging for cerebral palsy. 1826 59

Cerebral palsy is the most prevalent cause of persisting motor function impairment with a frequency of about 1/500 births. In developed countries, the prevalence rose after introduction of neonatal intensive care, but in the past decade, this trend has reversed. A recent international workshop defined cerebral palsy as "a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain." In a majority of cases, the predominant motor abnormality is spasticity; other forms of cerebral palsy include dyskinetic (dystonia or choreo-athetosis) and ataxic cerebral palsy. In preterm infants, about one-half of the cases have neuroimaging abnormalities, such as echolucency in the periventricular white matter or ventricular enlargement on cranial ultrasound. Among children born at or near term, about two-thirds have neuroimaging abnormalities, including focal infarction, brain malformations, and periventricular leukomalacia. In addition to the motor impairment, individuals with cerebral palsy may have sensory impairments, cognitive impairment, and epilepsy. Ambulation status, intelligence quotient, quality of speech, and hand function together are predictive of employment status. Mortality risk increases incrementally with increasing number of impairments, including intellectual, limb function, hearing, and vision. The care of individuals with cerebral palsy should include the provision of a primary care medical home for care coordination and support; diagnostic evaluations to identify brain abnormalities, severity of neurologic and functional abnormalities, and associated impairments; management of spasticity; and care for associated problems such as nutritional deficiencies, pain, dental care, bowel and bladder continence, and orthopedic complications. Current strategies to decrease the risk of cerebral palsy include interventions to prolong pregnancy (eg, 17alpha-progesterone), limiting the number of multiple gestations related to assisted reproductive technology, antenatal steroids for mothers expected to deliver prematurely, caffeine for extremely low birth weight neonates, and induced hypothermia for a subgroup of neonates diagnosed with hypoxic-ischemic encephalopathy.
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PMID:Diagnosis, treatment, and prevention of cerebral palsy. 1898 5

Cerebral folate deficiency (CFD) is associated with low levels of 5-methyltetrahydrofolate in the cerebrospinal fluid (CSF) with normal folate levels in the plasma and red blood cells. The onset of symptoms caused by the deficiency of folates in the brain is at around 4 to 6 months of age. This is followed by delayed development, with deceleration of head growth, hypotonia, and ataxia, followed in one-third of children by dyskinesias (choreo-athetosis, hemiballismus), spasticity, speech difficulties, and epilepsy. The low level of 5-methyltetrahydrofolate in the CSF can result from decreased transport across the blood-brain barrier, which is most probably because of the blocking of folate transport into the CSF by the binding of folate receptor antibodies to the folate receptors in the choroid plexus. Treatment of the condition with folinic acid for prolonged periods can result in significant improvement of clinical symptoms and a return of 5-methyltetrahydrofolate levels in the CSF to normal. In view of this response to treatment in CFD and allied conditions, a case can be made for screening the CSF of patients with neurological disorders of unknown origin.
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PMID:Cerebral folate deficiency. 1926 Sep 31

Motor disturbances are very common in paediatric neurology. Often families can be reassured that these are just variants of normal development. However, abnormal movements can also be the hallmark of severe brain dysfunction of different and complex origins. This review concentrates on motor disturbances as frequent and important symptoms of inborn errors of metabolism. A structured diagnostic approach is developed taking into account age-dependent physiological developments and pathophysiological responses of gross and fine motor functions. A series of investigations are presented with the primary aim of early diagnosis of treatable conditions. The correct recognition and differentiation of movement disorders (ataxia, rigid akinetic syndrome (Fparkinsonism_), dystonia, athetosis, tremor,and others), spasticity, and neuromuscular disorders, requires profound neurological expertise. A high level of suspicion and close interaction between paediatric neurologists and specialists in inborn errors of metabolism are indispensable to effectively and timely identify patients in whom motor disturbances are the presenting and/or main symptom of an inborn error.
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PMID:Inborn errors of metabolism and motor disturbances in children. 1973 Oct 74

Crigler-Najjar Syndrome is an uncommon genetic disorder characterized by the elevation of unconjugated plasmatic bilirubin secondary to deficiency of the enzyme uridine diphosphate glucuronyltransferase (UDP-GT). We report a 19-years-old woman with the syndrome diagnosed during the neonatal period, when she developed a severe jaundice in the first 10 days of life, reaching unconjugated bilirubin levels of 29 mg/dl, with normal liver function tests. After transient response to phototherapy, the patient was referred to a tertiary medical center in which an extensive work up ruled out other etiologies and the diagnosis of type I Crigler-Najjar syndrome was established. Currently, the patient has a mild mental retardation. She is receiving homemade phototherapy 18 h per day with acceptable control of bilirubin levels. Many mutations have been associated with UDP-GT dysfunction resulting in a broad spectrum of the disease. When bilirubin rises above physiological limits, it permeates the hematoencephalic barrier, inducing bilirubin impregnation of basal ganglia with secondary neuronal damage and necrosis. The worst outcome, kernicterus, is characterized by mental retardation, central deafness, ophthalmoplegia, ataxia, athetosis, spasticity, seizures and death. First line therapy includes phototherapy, but definitive therapy is liver transplantation before the occurrence of neurological damage.
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PMID:[Crigler-Najjar syndrome. Report of one case with a long term follow up]. 2486 Nov 23

Spasticity represents a common and very often incapacitating neurologic condition, for which a limited number of treatment options are available. Stereotactic ablation of the dentate cerebellar nuclei (dentatotomy) was widely used in the past with variable results. In the present study, we reviewed 12 consecutive cases operated on at the Midland Centre for Neurosurgery and Neurology at Birmingham University (Smetwick, UK) in the 1980s. The following clinical aspects were analyzed: severity of spasticity, occurrence of abnormal movements (dystonia/athetosis), language fluency, gait, and overall clinical condition. Follow-up ranged from 0.5 to 94 (mean: 31.6) months. Immediate improvement was noted in 10 patients, and five of them had sustained this improvement at the time of the last assessment. A more pronounced improvement was observed for gait, relative to speech and abnormal movements. No significant morbidity related to the procedure was observed. We conclude that dentatotomy is a safe procedure that should still be considered in specific cases. Here, we review the cases reported in the literature, and present a mechanistic hypothesis about how dentatotomy influences motor tonus, in light of the current knowledge about cerebellar physiology. We believe that this issue is critical for the development of alternative surgical approaches targeting the cerebellum, such as chronic electric stimulation.
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PMID:Evaluating cerebellar dentatotomy for the treatment of spasticity with or without dystonia. 2582 25


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