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2007| April-June | Volume 10 | Issue 5
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Evolution of abnormal postures in Duchenne muscular dystrophy
Maria Kinali, Marion Main, Eugenio Mercuri, Francesco Muntoni
April-June 2007, 10(5):44-54
Spinal deformities are common abnormalities of posture observed in Duchenne Muscular Dystrophy (DMD). Spinal alignment is the result of the integrity of different variables, ranging from normal vertebral development to balance and symmetry of tone and strength, normal control by central pathways and integrity of the sensory feedback. Any disturbance altering one or more of these variables increases the risk for developing abnormal postures. Scoliosis is a frequent complication (68-90%) of DMD. Although the risk of developing scoliosis is higher in non-ambulant DMD patients, scoliosis and kyphosis can be found occasionally in ambulant patients. In DMD patients the onset and the evolution of the abnormal postures are related to the onset and the progression of weakness. This review discusses the factors affecting posture in DMD, especially in relation to scoliosis and gives an overview of general guidelines on the diagnostic approach and some management issues related to DMD.
Cerebral palsy: A neonatal perspective
April-June 2007, 10(5):33-43
Cerebral palsy is the commonest form of motor disability in developed countries. Its prevalence has remained essentially unchanged, affecting two per 1000 live births, despite the advances in obstetric and neonatal care over the last three decades. This article reviews preterm and term infants most at risk of developing cerebral palsy and examines recent developments in the understanding of corticospinal tract development and plasticity.
Convulsive status epilepticus in children
Miquel Raspall-Chaure, Rod C Scott
April-June 2007, 10(5):7-18
Convulsive status epilepticus (CSE) is the most common neurological emergency in childhood. It differs from adult CSE in aspects of epidemiology, pathophysiology and clinical presentations, which justifies a separate analysis. The incidence is 18-20/100,000 children/year and distribution of aetiologies is markedly age-dependent: Febrile and acute symptomatic CSE are most common in children aged <2 years, whereas cryptogenic-idiopathic and remote symptomatic aetiologies are more common in older children. Mortality is 3-5% and morbidity directly attributable to CSE is less than 15%. When compared to brief seizures, the incidence of subsequent epilepsy is increased only in symptomatic goups. Aetiology is the main determinant of outcome, while the separate effect on outcome of duration, age and treatment remains controversial. The risk of sequelae in unprovoked and febrile CSE is low. There is some evidence that CSE, especially febrile CSE, might cause hippocampal injury, although its role in the development of mesial temporal sclerosis remains uncertain. Prolonged seizures lasting over 5-10 minutes are unlikely to stop spontaneously and should be treated as CSE. Prehospital administration of benzodiazepines is safe and simplifies subsequent management of CSE in the hospital setting. Treatment includes resuscitation measures, identification and treatment of causal factors and early antiepileptic treatment following local guidelines that may be based on national guidelines.
April-June 2007, 10(5):55-58
Sturge-Weber syndrome (SWS) is characterized by a facial capillary hemangioma (port wine stain) involving the periorbital area, forehead or scalp, a venous angioma of the leptomeninges and a choroidal angioma. Following normal early development, children with SWS often experience neurological and developmental deterioration in association with the onset of seizures and consequently suffer a high rate of disability. The classical imaging finding in these children is the evidence of calcification in the occipital area. The striking features of the EEG in SWS are the attenuation and the excess of slow activities. Recent advances in the diagnosis and treatment, particularly the surgical options are discussed.
April-June 2007, 10(5):3-6
Epileptic encephalopathy is applied to loss of skills in all domains of functioning including cognitive, behavioural, communication and motor that occurs with epilepsy and appears to be caused by epilepsy, particularly by subclinical seizure activity. It is particularly common in early onset epilepsies in which deterioration may be catastrophic but milder problem of cognition, attention and behaviour occur in up to half of school age children with epilepsy. Management involves early and vigorous treatment of the epilepsy and the identification and management of the additional impairments.
Arterial ischemic stroke in childhood
April-June 2007, 10(5):19-27
Arterial ischemic stroke is one of several childhood stroke syndromes, which has distinctive features according to age of onset. This article discusses clinical presentation, risk factors and outcomes of arterial ischemic stroke in newborns and older children. The majority of affected children have adverse outcomes; although recent research has identified important risk factors, facilitating a targeted clinical approach and research on treatment is still lacking. This will require multi-center international collaboration.
Epilepsy surgery in childhood
Judith Helen Cross
April-June 2007, 10(5):28-32
Surgery is now an established treatment for drug resistant symptomatic focal epilepsy in childhood. The aim is to resect the focus of epileptic seizures without functional compromise. A wide range of procedures may be considered from focal to multilobar resections and hemidisconnection procedures, the most common pathology being malformations of cortical development. Seizure freedom will be seen in 40-80% dependent on the underlying pathology and completeness of resection/disconnection of the epileptogenic zone. When likely long-term benefits are evaluated, surgery should be considered early in these children.
New frontiers in paediatric neurology: Challenges and opportunities
April-June 2007, 10(5):2-2
A supplement for the millions of children in India
Sanjeev V Thomas
April-June 2007, 10(5):1-1
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