European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society
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Eur. J. Paediatr. Neurol. · Mar 2016
Ketamine-based procedural sedation and analgesia for botulinum toxin A injections in children with cerebral palsy.
Adequate procedural sedation and analgesia (PSA) is essential to reduce pain and distress for children undergoing intramuscular botulinum toxin (BoNT-A) injections. This study describes our institution's experience with ketamine-based PSA in terms of safety and efficacy in children with cerebral palsy receiving BoNT-A injections. ⋯ Administration of ketamine-based PSA for intramuscular BoNT-A procedures in children can be both safe and efficacious.
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Eur. J. Paediatr. Neurol. · Jan 2016
Review Case ReportsCMV-associated axonal sensory-motor Guillain-Barré syndrome in a child: Case report and review of the literature.
Guillain-Barré syndrome is the most frequent cause of flaccid paresis in Western countries. Moreover, CMV infection is the most common antecedent viral infection in adult patients and the presence of specific IGM antiganglioside antibodies is often identified. Instead, Guillain-Barré syndrome following CMV infections is rarely reported in childhood and often presents severe symptoms at onset and longer recovery times. ⋯ The sensory-motor axonal form of Guillain-Barré syndrome following CMV infection may present a good prognosis and a prompt full recovery also in children, if adequate treatment is started in time.
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Eur. J. Paediatr. Neurol. · Jan 2016
Case Reports"Acquired" Dandy-Walker malformation and cerebellar hemorrhage: Usefulness of serial MRI.
Neuroimaging plays a fundamental role in the diagnosis of Dandy Walker malformation (DWM), a posterior fossa anomaly that is usually associated with genetic abnormalities, but may rarely be ascribed to acquired causes. Here, we report the clinical history and neuroimaging studies of a child with a complex cardiac malformation, developmental delay, and oculomotor anomalies whose neuroimaging findings were consistent with an acquired form of DWM. ⋯ This case strengthens the opinion that DWM is a heterogeneous condition, and may support the hypothesis that acquired meningeal abnormalities in the form of cortico-pial hemosiderosis may play a role in the development of DWM. This case also demonstrates that serial neuroimaging plays a key role in the correct diagnosis of posterior fossa malformations, whose prognosis is difficult to establish on second trimester fetal MRI and requires longer clinical follow-up.
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Eur. J. Paediatr. Neurol. · Nov 2015
Case ReportsDevastating recurrent brain ischemic infarctions and retinal disease in pediatric patients with CD59 deficiency.
Identification of CD59 p. Cys89Tyr mutation in 5 patients from North-African Jewish origin presenting with chronic inflammatory demyelinating polyradiculoneuropathy like disease and chronic hemolysis, led us to reinvestigate an unsolved disease in 2 siblings from the same origin who died 17 years ago. The two patients carried the same CD59 gene mutation previously described by our group. ⋯ Revising the brain autopsy of one of these patients confirmed the finding of multiple brain infarctions of different ages. CD59 protein expression was missing on brain endothelial cells by immunohistochemical staining. This new data expands the clinical spectrum of CD59 mutations and further emphasizes the need for its early detection and treatment.
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Eur. J. Paediatr. Neurol. · Nov 2015
Case ReportsA rare cause of status epilepticus; alpha lipoic acid intoxication, case report and review of the literature.
Alpha lipoic acid is a powerful antioxidant widely used for the supplementary treatment of diabetic neuropathy. Intoxication with alpha lipoic acid is very rare. There is no reported dose of safety in children. ⋯ Alpha lipoic acid and derivatives cause side effects in children like refractory convulsions. They are frequently rendered as vitamins by diabetic patients and are left at places where children can easily access them. Therefore, when faced with refractory convulsions in children who have had no disease before, intoxication by medicaments with alpha lipoic acid should be taken into consideration.