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Query: UMLS:C0018991 (hemiplegia)
 3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Recent discoveries about the central nervous system's response to injury and how patients reacquire behavioral capabilities by training have yielded promising new therapies for neurorehabilitation. This family of interventions is termed constraint-induced (CI) therapy and is essentially behavioral in nature. Constraining movement of the arm which is less affected by the stroke and training (by shaping) the more affected arm for many hours a day for two consecutive weeks proved effective in the treatment of hemiplegia in many studies. Successful applications other than for stroke have been for traumatic brain injury, cerebral palsy, spinal cord injury, fractured hip, and focal hand dystonia. Extending the principles to other consequences of stroke such as aphasia is examined. Constraint-induced therapy is shown to produce large changes in the organization and function of the brain,which emphasizes the significance of cortical reorganization and learning for neurorehabilitation.
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PMID:[New developments in stroke rehabilitation based on behavioral and neuroscientific principles: constraint-induced therapy]. 1270 2

Tourette syndrome (TS) is a neuropsychiatric disorder with onset in early childhood. It is characterized by tics and often accompanied by disturbances in behavior, such as obsessive-compulsive disorder (OCD). In most cases, the disorder is self-limited or can be treated by medication or behavioral therapy. In a small percentage, however, symptoms are intractable to any conservative treatment. Since 1955, various attempts have been made to treat these patients through neurosurgical procedures. The target sites have been diverse and include the frontal lobe (prefrontal lobotomy and bimedial frontal leucotomy), the limbic system (limbic leucotomy and anterior cingulotomy), the thalamus, and the cerebellum. Combined approaches have also been tried such as anterior cingulotomies plus infrathalamic lesions. The results have often been unsatisfactory or major side effects have occurred, such as hemiplegia or dystonia. Our review of the literature from 1960 until 2003 revealed 21 reports and 3 descriptions in textbooks covering about 65 patients in total who had undergone ablative procedures for intractable TS, the first being reported in 1962. In 1999, deep brain stimulation (DBS) was introduced as a new approach for intractable TS. To date, 3 patients have been reported who underwent bilateral thalamic stimulation, with promising results on tics and obsessive-compulsive symptoms.
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PMID:Surgery in Tourette syndrome. 1474 54

Alternating hemiplegia of childhood is a rare progressive disorder usually diagnosed in early childhood. It consists of repeated attacks of dystonia, hemiplegia or quadriplegia followed by fixed neurological deficits and developmental delay. A case is reported of a 33-year-old woman with alternating hemiplegia of childhood who presented at 34 weeks' gestation for urgent lower segment caesarean section due to repeated antepartum haemorrhages and following failed induction of labour.
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PMID:Caesarean section under general anaesthetic in a woman with alternating hemiplegia of childhood. 1532 38

The aim of this study was to determine whether botulinum toxin A (BTX-A) injected into the parotid and submandibular salivary glands of children aged 6 to 16 years with cerebral palsy (CP) and relative sialorrhoea significantly decreases their drooling and improves their quality of life. Twenty children (10 males, 10 females; mean age 10y 1mo [SD 3y 8mo]; range 6y 1mo-16y 7mo) with CP, identified as having significant daily drooling (scoring at least 6 on drooling frequency/severity scales) were recruited. BTX-A (2U/kg; maximum 70U) was injected under sedation, using ultrasound guidance (1.4U/kg and 0.6U/kg divided between parotid and submandibular glands respectively). Nineteen children completed the study: 15 with spastic quadriplegia (Gross Motor Function Classification System [GMFCS] Level V); one with dystonia (GMFCS Level V); one with hemiplegia (GMFCS Level II); and two with a mixed pattern of CP (GMFCS Levels III and V). Drooling was assessed by five methods at baseline, and 4 and 12 weeks after injection. Qualitative assessment of drooling frequency and severity scores showed statistically significant reductions at 4 weeks (p<0.001) and 12 weeks (p=0.006). Qualitative assessment of quality of life scores (rated by parents and teachers separately) also significantly improved (p<0.001 and p=0.023 respectively). Quantitative assessments showed that the number of bibs/scarves changed per day was significantly reduced at 4 weeks (p<0.001). There was no side effect from the injections themselves; 89% of parents and children wished for further intrasalivary BTX-A injections in the future. We conclude that percutaneous intrasalivary BTX-A injections into the parotid and submandibular salivary glands can reduce drooling in children with CP and relative sialorrhoea, leading to an improvement in their quality of life.
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PMID:Parotid and submandibular botulinum toxin A injections for sialorrhoea in children with cerebral palsy. 1704 54

We herein report the findings of a 2-year-6-month-old boy, who had been experiencing monocular pendular nystagmus, strabismus, and episodic eye deviation nystagmus, intractable dystonia and apneic attack which all began when he was 2 days of age. He underwent a complete blood count test, blood chemistry test, analysis of amino acids in the blood and urine, analysis of pyruvate/lactate in blood and cerebrospinal fluid, head computed tomography and magnetic resonance imaging and no abnormal results were identified. His attacks were resistant to multiple antiepileptic and dopaminergic drugs. He showed transient left and/or right hemiplegia after nystagmus, dystonia and/or apneic attacks at 8-months of age with retardation in intelligence. We diagnosed him to have alternating hemiplegia of childhood (AHC). We were unsure how to deal with his attacks after he was discharged from the hospital, however, resuscitation with the ambu bag by his mother at home and the intravenous infusion of diazepam or thiamylal at the hospital together was proven to be an effective method for treating his severe apneic attacks. The effect of diazepam and amantadine on these attacks was transient, however, the administration of flunarizine with amantadine resulted in an improvement in his attacks. We therefore consider the administration of flunarizine to be essential for the effective treatment of AHC in this case.
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PMID:[A boy with nystagmus, refractory dystonia and apneic attack due to alternating hemiplegia of childhood]. 1763 86

The aim of this study was to describe the motor function of a population of children at age 5 years enrolled on the South Australian Cerebral Palsy Register. Among children born between 1993 and 1998, there were 333 with confirmed cerebral palsy (prevalence rate 2.2 per 1000 live births), in whom 247 assessments (56.7% males, 43.3% females) were completed. The distribution by Gross Motor Function Classification System (GMFCS) level was: level I, 50.6%; level II, 18.2%; level III, 9.3%; level IV, 9.7%; level V, 12.1%. The most common topographical classification was spastic diplegia (38.5%), followed by spastic hemiplegia (34.8%) and spastic quadriplegia (14.6%). Abnormal movements occurred at rest or with intention in 19.4% of children. A high proportion of the population with relatively mild gross motor impairments have difficulty with everyday bimanual tasks, reinforcing the need to assess upper limb function independently of gross motor function. The use of ankle-foot orthoses was common, particularly across GMFCS levels II to IV. Further refinement is indicated for this population's motor dataset, to include more recently described classification measures as well as future novel measures to better describe the presence of both spasticity and dystonia.
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PMID:Motor function in 5-year-old children with cerebral palsy in the South Australian population. 1953 22

Botulinum toxin has become an important component of standard practice in the management of children with hypertonia (spasticity or dystonia). Following intramuscular injection, the neurotoxin causes a reversible neuromuscular blockade, creating both muscle weakness and a reduction in tone. Frequent goals of botulinum toxin injection include improving motor function, promoting longitudinal muscle growth and decreasing painful muscle spasms. The neuromuscular blockade lasts for three to six months on average. The clinical effect may last longer and the safety profile is excellent. Strong evidence, including randomized trials, exists for the efficacy of botulinum toxin in the management of lower and upper extremity tone in children. Common clinical indications include spastic equinus (toe walking), hip subluxation, upper extremity spasticity associated with hemiplegia or quadriplegia, and multilevel leg muscle injections in children with spastic diplegia.
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PMID:The use of botulinum toxin in paediatric hypertonia. 1966 43

Alternating hemiplegia of childhood is a neurological disorder characterized by episodes of hemiplegia, various non-epileptic paroxysmal events and global neurological impairment. Characterization of the evolution and outcome into adulthood has not been sufficiently investigated. The goal of this study was to elucidate the natural history of alternating hemiplegia within a large cohort of 157 patients, as part of the European Network for Research on Alternating Hemiplegia project. A questionnaire was formulated to determine the severity of both paroxysmal and global neurological impairment and address progression of the disorder by allocating data to specific age epochs up to and over 24 years of age. Patients in early age groups were consistently present in subsequent later age groups and for each patient, data were collected for each corresponding age epoch. The study was based on predominantly retrospective and, for a period of 2 years, prospective data. At inclusion, patients were aged from 9 months to 52 years. The median age at diagnosis was 20 months. All patients experienced hemiplegic attacks; 86.5% reported episodes of bilateral weakness, 88% dystonic attacks, 53% epileptic seizures, 72% developed chorea and/or dystonia and 92% mental retardation. When data over the course of the illness were examined for the whole cohort, the severity of symptoms did not appear to change, with the exception of abnormal ocular movements and hypotonia that regressed, but did not disappear into adulthood (from 86 to 36% and 76 to 36%, respectively). No statistically significant correlation between a history of severe paroxysmal hemiplegic/dystonic episodes and a worse neurological outcome was identified. Seven patients died, some of whom experienced severe plegic attacks or epileptic seizures at the time of death. History of severe plegic/dystonic attacks was not found to be an aggravating factor for deceased patients. Our results provide evidence that the natural history of alternating hemiplegia is highly variable and unpredictable for individual patients. However, we did not find evidence to support a steadily progressive and degenerative course of the disorder when patients were analysed as a group. For a minority of patients, a risk of sudden death was associated with more severe neurological impairment. The European Network for Research on Alternating Hemiplegia Registry, validated by our study, includes all major neurological signs and symptoms of alternating hemiplegia and may thus be used as a precedent for the progressive inclusion and follow-up of patients as well as a reference for genetic studies and treatment trials.
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PMID:Evidence of a non-progressive course of alternating hemiplegia of childhood: study of a large cohort of children and adults. 2097 17

Glucose transporter type 1 deficiency syndrome is characterized by infantile onset seizures, development delay, movement disorders, and acquired microcephaly. The phenotype includes allelic variants such as intermittent ataxia, choreoathetosis, dystonia, and alternating hemiplegia of childhood with or without epilepsy. Dystonias involve allelic variants of glucose transporter type 1 deficiency syndrome. Three Chinese patients presented with paroxysmal behavioral disturbance, weakness, ataxia (especially after fasting), and exercise intolerance. Electroencephalogram findings did not correlate with clinical manifestations. Cranial magnetic resonance imaging produced normal results or mild hypomyelination. Hypoglycorrhachia was evident in all cases. Cerebrospinal fluid glucose ranged from 1.63-2.45 mmol/L. Erythrocyte 3-O-methyl-d-glucose uptake was decreased to 58% in patient 1. Three SLC2A1 disease-causing mutations (761delA, P383H, and R400C) were observed. No patient tolerated ketogenic diets. Two patients responded to frequent meals with snacks. Cerebrospinal fluid evaluation constitutes the diagnostic testing permitting early treatment of glucose transporter type 1 deficiency syndrome. Early diagnosis and treatment improve prognoses.
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PMID:Allelic variations of glut-1 deficiency syndrome: the chinese experience. 2270 13

Alternating hemiplegia of childhood is a rare, predominantly sporadic disorder. Diagnosis is clinical, and little is known about genetics. Glucose transporter 1 deficiency syndrome shares with alternating hemiplegia of childhood paroxysmal and nonparoxysmal symptoms. The aim of the study was to investigate glucose transporter 1 mutations in 30 Italian patients. Genetic material was analyzed by DNA amplification and glucose transporter 1 region sequencing. Mutational analysis findings of the SLC2A1 gene were negative in all patients. The pattern of movement disorders was reviewed. Interictal dystonia and multiple paroxysmal events were typical of alternating hemiplegia of childhood. In conclusion, alternating hemiplegia of childhood is a heterogeneous clinical condition, and although glucose transporter 1 deficiency can represent an undiagnosed cause of this disorder, mutational analysis is not routinely recommended. Alternatively, a careful clinical analysis and the 3-O-methyl-D-glucose uptake test can allow prompt identification of a subgroup of patients with alternating hemiplegia of childhood treatable with a ketogenic diet.
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PMID:Lack of SLC2A1 (glucose transporter 1) mutations in 30 Italian patients with alternating hemiplegia of childhood. 2289 93


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