Pediatric neurology
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Pediatric neurology · Apr 1999
Review Case ReportsEarly diagnosis of pediatric Wernicke's encephalopathy.
Wernicke's encephalopathy may be fatal if untreated. Because Wernicke's encephalopathy is suspected to be underdiagnosed in children, the authors wished to assess the frequency of overlooked diagnosis and to establish pertinent findings that could lead to early identification of pediatric Wernicke's encephalopathy. The authors performed multiple literature searches seeking pediatric patients with Wernicke's encephalopathy (age = 20 years or younger). ⋯ Only six presented with the Wernicke's encephalopathy clinical triad (mental status changes, ocular signs, and ataxia) at neurologic onset; nine eventually demonstrated this triad. The high rate of patients diagnosed only at postmortem examination (41.9%) confirms that Wernicke's encephalopathy is underdiagnosed in children. Thiamine therapy is warranted if any component of the Wernicke's encephalopathy triad is present in an appropriate clinical setting.
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This study describes the clinical spectrum of patients with Klinefelter's syndrome and seizures. Klinefelter's syndrome is a sex chromosomal abnormality and the most common cause of male hypogonadism. It is characterized by cognitive dysfunction, hypogonadism, and abnormalities of physical maturation. ⋯ The seizures of six of 11 patients with epilepsy were well controlled with antiepileptic drugs. One patient had a single seizure and was not treated with medication. In patients with Klinefelter's syndrome and recurrent seizures, the electroclinical spectrum is heterogenous and outcome with antiepileptic drug treatment is favorable.
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Pediatric neurology · May 1998
Review Case ReportsPseudotumor cerebri in Lyme disease: a case report and literature review.
Pseudotumor cerebri is an unusual presentation of Lyme disease. The case of an 8-year-old girl with pseudotumor cerebri secondary to acute neuroborreliosis is reported. She presented with acute onset of headache, papilledema, sixth nerve palsy, increased intracranial pressure, and cerebrospinal fluid pleocytosis. ⋯ Twelve reported cases that mostly presented with systemic findings and signs of Lyme disease before development of pseudotumor cerebri were reviewed. We conclude that acute neuroborreliosis can present with pseudotumor cerebri as an initial manifestation. It is important to include Lyme disease in the differential diagnosis of pseudotumor cerebri in an area endemic for Lyme disease.
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Pediatric neurology · Apr 1998
ReviewOutcome of children with cerebral edema caused by fulminant hepatic failure.
Mortality is high in patients with fulminant hepatic failure (FHF). Neurologic complications of encephalopathy and cerebral edema are major contributors to mortality. Orthotopic liver transplantation has improved survival in these patients. ⋯ Termination of care is a reasonable option. Comprehensive monitoring of cerebral function and intracranial pressure is required in children with FHF. Orthotopic liver transplantation should be performed in children with severe and worsening encephalopathy before the development of radiographically apparent cerebral edema.
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Pediatric neurology · Oct 1996
Review Case ReportsRhabdomyolysis and anesthesia: a report of two cases and review of the literature.
Rhabdomyolysis occurred in two apparently healthy boys aged 9.5 and 5.5 years after general anesthesia with suxamethonium. Mild hyperkalemia and renal failure were observed in the first patient, who was subsequently diagnosed with Becker dystrophy. In the second patient, the clinical presentation was not classic for malignant hyperthermia and a muscle biopsy failed to disclose any pathological finding. ⋯ Forty-nine (74%) cases were caused by an underlying, mostly unrecognized congenital muscle disease, and 14 (21%) cases were caused by malignant hyperthermia susceptibility. Hyperkalemia (23 patients), cardiac arrhythmias (38 patients), renal failure (4 patients), and death (11 patients) were the most serious complications of anesthesia-associated rhabdomyolysis. The neuromuscular blocking agent suxamethonium had been used in at least 43 of the patients reported in the literature.