1,721,098 research outputs found
Pharmacotherapy of spasticity in children with cerebral palsy
No full textSpasticity is one of the most common symptoms presented by neurologic patients. Apart from surgical management, drug therapy is an important treatment of children suffering from spasticity. In this review, recent advances in the pharmacologic armamentarium are reported in detail. In particular, there are oral medications (benzodiazepines, baclofen, dantrolene sodium, alpha 2 adrenergic agonists) and parenteral medications (botulinum toxin type A and B, alcohol). Moreover, there is also baclofen that can be administered intrathecally. There are some reports supporting the use of intramuscular alcohol (45% and/or 5-7% phenol) to reduce spasticity without the loss of voluntary movement or loss of sensation. Among these drugs, intrathecal baclofen is one of the most effective substances that can reduce spasticity significantly in the upper and lower extremities. Finally, the effectiveness of therapy with botulinum toxin type A in the management of spasticity is analyzed. Botulinum toxin type A reduces hypertonia in the injected muscles for a period of 2 to 4 months without important side effects. The purpose of this article is to provide an overview of available oral and parenteral drugs for treatment of spasticity in cerebral palsy and to outline indications and contraindications. © 2006 Elsevier Inc. All rights reserved
The possible use of the L-type calcium channel antagonist verapamil in drug-resistant epilepsy
No full textMultidrug transporters (MDTs) are likely to play a role in the pathogenesis of drug resistance in epilepsy, acting at the level of the blood-brain barrier by returning antiepileptic drugs to the blood vessels and lowering brain penetration and concentration (e.g. the so-called multidrug transporter hypothesis). In the last ten years experimental studies on both animal models and human brain tissues have highlighted a potential role of the P-glycoprotein-one of the multidrug transporters of the blood-brain barrier-in the pathophysiology of drug-resistant epilepsies. At the same time, verapamil has been administered to patients with drug-resistant epilepsy (e.g., Dravet syndrome, Lennox-Gastaut syndrome, focal epilepsies) or status epilepticus with promising results. In this drug profile paper the authors review current knowledge and main published studies regarding the role of the L-type calcium channel antagonist verapamil in drug-resistant epilepsy
Residual and Persistent Adie's Pupil After Pediatric Ophthalmoplegic Migraine
No full textWe report on a 9-year-old girl diagnosed with ophthalmoplegic migraines who had been previously diagnosed, at age 7 years, with typical migraines with aura. After resolution of the third ophthalmoplegic migraine attack, the only evident residual clinical sign was Adie's pupil. During 24-month follow-up, at age 11 years, a neurologic examination produced completely normal results. However, Adie's pupil persisted. Adie's tonic pupil can be associated with extraocular diseases, which were all excluded in this patient. The mechanisms underlying tonic pupil are not fully understood. This is the first report, to the best of our knowledge, of an ophthalmoplegic migraine followed by persistent Adie's pupil. Possible pathogenic mechanisms are discussed. © 2009 Elsevier Inc. All rights reserved
Editorial: Stress neurobiology in COVID-19: diagnosis, neuroimaging and therapeutic tools
Full text linkCoronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), is often asymptomatic in children. However, neurological involvement can occur, presenting with a broad range of manifestations. The neurological consequences of SARS-CoV-2 can vary from non-specific neurological symptoms to specific
central or peripheral nervous system (CNS and PNS) diseases (3). Furthermore, the
pandemic has disrupted the care for children with neurological conditions and has also resulted in significant psychological stress, which is still far from being fully quantified. In the Research Topic analysed, an extensive exploration of the wide-ranging pediatric clinical implications of various pathogenic mechanisms associated with SARS-CoV-2 on both the CNS and PNS, in acute and post-acute phases, is conducted. Possible
pathogenesis, laboratory and neuroimaging assessments, treatment responses, and prognosis are identified within each diagnostic framework. Additionally, the Research Topic seeks to shed light on the less explored, non-infection-related, neurological, and psychiatric consequences of the pandemic in the youth
Complex epileptic (Foix-Chavany-Marie like) syndrome in a child with neurofibromatosis type 1 (nf1) and bilateral (opercular and paracentral) polymicrogyria
No full textThe association of brain malformations and symptomatic epilepsy in the setting of neurofibromatosis type 1 (NF1) is rarely reported. When it occurs, patients can present clinically with infantile spasms, focal seizures, generalized tonic clonic seizures or atypical absences. We report on a 10-year-old (molecularly proven) NF1 girl manifesting a complex epileptic syndrome resembling the Foix-Chavany-Marie spectrum (also known as opercular syndrome) associated with bilateral (opercular and paracentral lobular) polymicrogyria (PMG). Anecdotal cases of unilateral PMG in the setting of NF1 have been described in association with other-than-opercular epileptic syndromes. The typical clinical opercular syndrome consisting in mild mental retardation, epilepsy and pseudobulbar palsy is usually associated to bilateral perisylvian PMG (BPP) CONCLUSION: To the best of our knowledge, the complex epileptic syndrome hereby reported has not been previously recorded in the setting of NF1. In addition, the present girl manifested all the clinical features of an opercular syndrome but had an asymmetrical PMG (not a BPP)
Diffuse onset of ictal electroencephalography in a typical case of panayiotopoulos syndrome and review of the literature
No full textPanayiotopoulos syndrome is a common and benign childhood autonomic epilepsy of debated localization. Although officially considered as occipital epilepsy, this is most likely of multifocal origin. Ictal electroencephalography is the gold standard of seizure localization, but in Panayiotopoulos syndrome, because patients have single or rare seizures, only 7 cases with ictal electroencephalography have been reported. Ictal onsets show variable anterior and more often posterior locations. We describe an 8-year-old girl with 5 nocturnal autonomic seizures typical of Panayiotopoulos syndrome from age 4. The last seizure was captured with electroencephalography and showed a diffuse onset of the ictal discharge, whereas various interictal electroencephalography had infrequent multifocal spikes. This case contributes to the understanding of the pathophysiology of Panayiotopoulos syndrome in favor of a diffuse and multifocal cortical epileptogenicity that triggers an unstable central autonomic nervous system solely or prior to the focal cortical symptoms. © 2009 Sage Publications
Myoclonic status and central fever in Angelman syndrome due to paternal uniparental disomy
No full textEfficacy of verapamil as an adjunctive treatment in children with drug-resistant epilepsy. A pilot study
No full textPurpose: Verapamil, a voltage-gated calcium channel blocker, has been occasionally reported to have some effect on reducing seizure frequency in drug-resistant epilepsy or status epilepticus. We aimed to investigate the efficacy of verapamil as add-on treatment in children with drug-resistant epilepsy. Methods: Seven children with drug-resistant structural-metabolic, unknown or genetic (e.g., Dravet syndrome [DS]) epilepsy received verapamil as an add-on drug to baseline antiepileptic therapy. Verapamil was slowly introduced at the dosage of 1 mg/kg/day and titrated up to 1.5 mg/kg/day. After completing the titration period, patients entered a 14-month maintenance period and were followed up at 3, 8, and 14 months. Heart monitoring was performed at baseline and at each follow-up. The primary outcome measure was the response of seizures to verapamil. Results: Three subjects with genetically determined DS showed a partial (reduction of 50-99%) response for all types of seizures. A patient with DS without known mutation showed a partial control of all types of seizures in the first 13 months; then seizures worsened and verapamil was suspended. Two patients with structural epilepsy and one with Lennox-Gastaut syndrome showed no improvement. Any side effects were recorded. Conclusions: Add-on treatment with verapamil seems to have some effect in controlling seizures in patients with genetically determined DS. Our observations justify further research on the relationship between calcium channels, calcium channel blockers, and channelopathies
New trends in neuronal migration disorders
No full textNeuronal migration disorders are an heterogeneous group of disorders of nervous system development and they are considered to be one of the most significant causes of neurological and developmental disabilities and epileptic seizures in childhood. In the last ten years, molecular biologic and genetic investigations have widely increased our knowledge about the regulation of neuronal migration during development. One of the most frequent disorders is lissencephaly. It is characterized by a paucity of normal gyri and sulci resulting in a "smooth brain". There are two pathologic subtypes: classical and cobblestone. Classical lissencephaly is caused by an arrest of neuronal migration whereas cobblestone lissencephaly caused by overmigration. Heterotopia is another important neuronal migration disorder. It is characterized by a cluster of disorganized neurons in abnormal locations and it is divided into three main groups: periventricular nodular heterotopia, subcortical heterotopia and marginal glioneural heterotopia. Polymicrogyria develops at the final stages of neuronal migration, in the earliest phases of cortical organization; bilateral frontoparietal form is characterized by bilateral, symmetric polymicrogyria in the frontoparietal regions. Bilateral perisylvian polymicrogyria causes a clinical syndrome which manifests itself in the form of mild mental retardation, epilepsy and pseudobulbar palsy. Schizencephaly is another important neuronal migration disorder whose clinical characteristics are extremely variable. This review reports the main clinical and pathophysiological aspects of these disorders paying particular attention to the recent advances in molecular genetics. © 2009 European Paediatric Neurology Society
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