Pediatric neurology
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Pediatric neurology · Oct 2012
Case ReportsInternal carotid artery stenosis as the sequela of a pseudoaneurysm after methicillin-resistant Staphylococcus aureus infection.
Pseudoaneurysm of the internal carotid artery is an extremely uncommon complication of pediatric deep neck space infections in the postantibiotic era, and poses a diagnostic challenge to clinicians. Early recognition and aggressive management are essential to prevent mortality and serious morbidity. We describe a pseudoaneurysm of the left internal carotid artery after a retropharyngeal abscess in an infant. ⋯ During 7-year follow-up, she demonstrated no neurologic sequelae and normal development. Despite the left internal carotid artery stenosis, blood supply to the left cerebral hemisphere is well maintained through the circle of Willis. We highlight the importance of early recognition and aggressive management to avoid serious outcomes.
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Pediatric neurology · Sep 2012
Case ReportsDecompressive craniectomy in childhood posterior circulation stroke: a case series and review of the literature.
Few reports address the role of decompressive craniectomy in children with space-occupying cerebral edema attributable to severe ischemic infarction of the posterior cerebral arterial circulation. We describe four children with posterior circulation arterial ischemic stroke who required decompressive craniectomy. These children accounted for 11% of all cases of posterior circulation ischemic stroke at our institution from 2002-2010. ⋯ Deterioration occurred within 72 hours of stroke onset. Two patients demonstrated minimal functional deficits at follow-up, and two demonstrated moderate deficits with functional limitations. Because decompressive craniectomy can be lifesaving in children with severe posterior circulation arterial ischemic stroke, early neurosurgical referral should be considered.
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Guillain-Barré syndrome is characterized by acute progressive weakness, areflexia, and maximal motor disability that occur within 4 weeks of onset. Various clinical subtypes have been described since the original description of the syndrome. This study aimed to identify characteristics of clinical variants of Guillain-Barré syndrome through retrospective review of cases in Chang Gung Children's Hospital from 2000-2010. ⋯ Follow-up revealed that 35 recovered satisfactorily, eight were persistently disabled, and none died during hospitalization. At the earliest stage, differentiating clinical variants from typical Guillain-Barré syndrome was difficult. Children with clinical variants of Guillain-Barré syndrome are more likely to manifest rapid onset from disease onset to nadir, increasing the severity of disability, cranial nerve involvement, urine incontinence, respiratory impairment, and need for ventilator support than in typical Guillain-Barré syndrome.
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Hyperargininemia is an autosomal recessive metabolic disorder caused by a deficiency of enzyme arginase I. It is a rare pan-ethnic disease with a clinical presentation distinct from that of other urea cycle disorders, and hyperammonemic encephalopathy is not usually observed. Hyperargininemia is one of the few treatable causes of pediatric spastic paraparesis, and can be confused with cerebral palsy. ⋯ Progressive spastic diplegia constituted the key clinical abnormality in this group, but variability in clinical presentation and progression were evident in our series. Seizures in hyperargininemia may be more common than reported in previous studies. Features distinguishing hyperargininemia from cerebral palsy and hereditary spastic paraplegia are emphasized in this large series of patients.
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Pediatric neurology · Jun 2012
Management of patients with status epilepticus treated at a pediatric intensive care unit in Turkey.
We investigated the etiology, treatment, and prognosis of patients treated for status epilepticus at a pediatric intensive care unit. Medical records of 89 patients admitted to a pediatric intensive care unit with status epilepticus were reviewed retrospectively. Patients ranged in age from 2 months to 18 years (mean age ± S. ⋯ The total mortality rate was 3.4%. This lower mortality rate may be considered evidence of the effectiveness and reliability of the status epilepticus treatment protocol in our pediatric intensive care unit. Prehospital rectal diazepam administration and the treatment of brain edema in the intensive care unit may be useful in the management of patients with status epilepticus.