1/66. Hypotonia, congenital nystagmus, ataxia, and abnormal auditory brainstem responses: a report on the first white patient.A white Italian boy, aged 5 years and 8 months, is reported with failure to thrive, hypotonia, truncal ataxia, psychomotor retardation, and congenital horizontal pendular nystagmus with only waves I and II on auditory brainstem responses. Our patient's clinical picture resembles that previously reported in 10 male Oriental patients. He did not manifest spastic diplegia by the age of 2 years, as did the subjects reported in the literature, but knee-jerk hyperreflexia was evident at the most recent clinical reevaluation. Serial brain MRI studies revealed a cystic brain lesion and peritrigonal hyperintensities with no brainstem abnormalities. To date, no other child with a similar syndrome has been described either in europe or in America. The clinical features of this condition are consistent and characteristic. A definitive diagnosis is achieved by demonstrating the absence of all waves following wave I or wave II on auditory brainstem responses as early as 3 months of age. Due to the predominance of males, the occurrence in siblings, the early age at onset, the non-progressive course, and the characteristic auditory brainstem response findings, the syndrome may have a genetic origin and be attributable to a dysgenetic brainstem lesion.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/66. A patient with cerebral palsy whose mother had a traffic accident during pregnancy: a diffuse axonal injury?A 16-year-old girl had spastic cerebral palsy (CP) with triplegia and focal epilepsy. The patient's past history included her mother's lower abdominal trauma caused by a traffic accident at the 7th month of gestation. Brain examination with magnetic resonance imaging (MRI) revealed encephalomalacia at the bilateral parieto-temporal lobes and the left caudate nucleus, segmental narrowing of the splenium of the corpus callosum, dilatation of the left lateral ventricle and an abnormally high intensity at the right posterior portion of the internal capsule. These findings might indicate a diffuse axonal injury (DAI), but not an asphyxic brain damage. In this patient, CP might be caused by an intrauterine DAI when her mother was involved in the accident.- - - - - - - - - - ranking = 0.090909090909091keywords = brain (Clic here for more details about this article) |
3/66. magnetic resonance imaging findings in cerebral palsy.OBJECTIVE: To review all cases of cerebral palsy (CP) that had magnetic resonance imaging (MRI) over a defined period of time. METHODOLOGY: The MRI brain scans of 42 children (12 premature, 30 full-term) with CP were studied. The scans were performed at the Royal Children's Hospital, Melbourne, between January 1995 and June 1996. RESULTS: Abnormalities were found in 39 of the 42 scans. Five children had cortical malformations and three children had white matter hypoplasia, indicating insults during the second trimester of pregnancy. Twenty-one children had hypoxic-ischaemic lesions (eight premature, 13 full-term) with patterns of periventricular leucomalacia, subcortical lesions or cortical infarction indicating insults perinatally or in the third trimester. Only 10 children had scans that could not be categorized into these groups. CONCLUSIONS: In this study sample of children with CP, MRI was useful in revealing underlying brain abnormalities, most of which were due to events in the third trimester or the perinatal period.- - - - - - - - - - ranking = 0.18181818181818keywords = brain (Clic here for more details about this article) |
4/66. Thalamic stimulation for choreiform movement disorders in children. Report of two cases.Surgery for movement disorders is most commonly performed in patients with dyskinesia and tremor associated with Parkinson's disease or in those with essential tremor. The role of ablative surgery or deep brain stimulation in patients with choreiform movements is poorly defined. The authors placed thalamic stimulation systems in two children with disabling choreiform disorders due to intracerebral hemorrhage or cerebral palsy. Each patient displayed choreiform movements in the upper extremities both at rest and with intention, which interfered with daily activities and socialization. Both children obtained significant improvement in their choreiform movements, and their upper extremity function improved with no incidence of morbidity. Thalamic stimulation appears to be a promising and nonablative approach for children with choreiform movement disorders.- - - - - - - - - - ranking = 0.090909090909091keywords = brain (Clic here for more details about this article) |
5/66. Postoperative stroke in a child with cerebral palsy heterozygous for factor v Leiden.A 5-year-old with spastic quadraparetic cerebral palsy suffered multiple strokes after extensive orthopedic surgery. Coagulation testing was undertaken to determine whether a familial thrombophilia was present. The patient was found to be heterozygous for factor v Leiden. factor v Leiden may be a risk factor for central nervous system events in special-needs children, particularly when common medical conditions create additional procoagulant risks.- - - - - - - - - - ranking = 0.037590544876178keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/66. Neonatal periventricular leukomalacia preceded by fetal periventricular echodensity.OBJECTIVE: The purpose of this prospective study is to verify whether fetal periventricular echodensity (PVE) precedes neonatal periventricular leukomalacia (PVL). methods: Fetal brains were studied with transvaginal scan in 63 high-risk fetuses from 17 to 32 weeks of pregnancy, PVE echogenicity was quantified with ultrasonic histogram, and neonatal brains and clinical courses were studied after birth. RESULTS: No fetal cystic PVL was found, instead, fetal PVE was detected in 42 fetuses. The quantified echogenicity value was higher in PVE than in normal brain. Four cases developed neonatal PVL among 28 preterm and 1 among 14 term births. Neonatal PVL developed in the 23 cases of persistent fetal PVE, whereas no neonatal PVL was found when fetal PVE was negative or disappeared. Cord compression signs were common in PVL cases. CONCLUSION: Neonatal PVL was preceded by antepartum persistent fetal PVE in the present study.- - - - - - - - - - ranking = 0.27272727272727keywords = brain (Clic here for more details about this article) |
7/66. Hypotensive hemorrhagic necrosis in basal ganglia and brainstem.Hypotensive hemorrhagic necrosis of the basal ganglia and brainstem has only occasionally been described. Three such cases are reported. Cardiac arrest had occurred in all cases, and it took at least 1 hour to restore adequate circulation. The patients remained comatose for 2 days to 2 weeks until death. Persistent hypotension causing ischemia in the distribution of deep perforating arteries is considered to have been the key underlying mechanism. hemorrhage is thought to have been caused by extravasation of red blood cells through damaged blood vessels.- - - - - - - - - - ranking = 0.45454545454545keywords = brain (Clic here for more details about this article) |
8/66. Bielschowsky bodies (Lafora bodies of Bielschowsky type): report of a case associated with Rosenthal fibers in the brain stem.Bielschowsky bodies are an uncommon type of polyglucosan body. Similar to Lafora bodies, they are characteristically identified within neuronal perikarya and neurites. However, they lack the diffuse distribution of Lafora bodies, and instead are typically restricted to the external pallidum, often in association with status marmoratus or atrophy of the putamen. Fewer numbers of Bielschowsky bodies have also been identified in other areas such as the substantia nigra, putamen and inner globus pallidus. We report an additional case with Bielschowsky bodies in an 18-year old female with cerebral palsy. This case demonstrated multifocal Bielschowsky bodies and abundant Rosenthal fibers in the midbrain and pons. To our knowledge the association of Bielschowsky bodies with this peculiar distribution of Rosenthal fibers has not previously been reported.- - - - - - - - - - ranking = 0.45454545454545keywords = brain (Clic here for more details about this article) |
9/66. magnetic resonance imaging in three children with kernicterus.The incidence of kernicterus has been greatly reduced by effective monitoring and treatment for hyperbilirubinemia. Findings on magnetic resonance imaging (MRI) in patients with kernicterus are characteristic. This study presents three cases of possible kernicterus without typical symptoms but with MRI features consistent with kernicterus. These cases suggest that kernicterus can develop, especially in preterm infants, in the presence of relatively low levels of bilirubin and the absence of obvious acute symptoms. Therefore assessing the risk of kernicterus may be difficult in the neonatal period. In addition, MRI findings at the posteromedial border of the globus pallidus in patients with athetotic cerebral palsy are strong evidence of brain damage caused by kernicterus.- - - - - - - - - - ranking = 0.090909090909091keywords = brain (Clic here for more details about this article) |
10/66. Management of the upper limb with botulinum toxin type A in children with spastic type cerebral palsy and acquired brain injury: clinical implications.The aim of this article is to describe our clinical experience in treating muscle imbalance in 49 children with spastic upper extremity involvement. We discuss four cohorts of children treated with botulinum toxin type A (BTX-A), each with different treatment objectives. In the first group, 27 children were treated for functional improvement and, of these, 23 had a positive effect, while four had no objective benefit. In the second group, eight children were treated for purposes of presurgical planning; of these, four were referred for surgery, three continued with serial treatment and one child did not benefit from injection. The third group comprised six children who were treated to improve posture and care: in this group, four children demonstrated clear benefit and two children lost some function subsequent to injection. Finally, a fourth group of seven children were treated after acquired brain injury (three with severe tetraplegia, four with hemiplegia). In this group, all children experienced spasticity relaxation and two children with hemiplegia also gained functional benefit. In terms of adverse events, deterioration of upper extremity function was poorly tolerated but limited to the first 1--3 weeks postinjection. Grip strength or thumb grip were diminished if too high doses were used. overall, our results with BTX-A were rewarding in children with no fixed contracture, good motor learning capacity and high motivation to train. Additionally, BTX-A treatment has proven valuable for counteracting spasticity in children with acquired brain injury. This treatment modality may not, however, be an appropriate treatment option for all children with severe upper extremity spasticity, due to the shorter duration of effect and the potential reduction in functional abilities seen in this cohort. In all cases, the selection of muscles to be treated needs careful clinical assessment. Dynamic EMG analysis should be performed whenever required to aid muscle selection, especially in children with spasticity combined with dystonia. Evaluation of M-responses suggests that for the forearm muscles, doses of BTX-A above 1.5 U/kg/muscle should not be used.- - - - - - - - - - ranking = 0.54545454545455keywords = brain (Clic here for more details about this article) |
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